Radiology Review Manual
6th Edition

Heart and Great Vessels
Differential Diagnosis of Cardiovascular Disorders
Congenital Heart Disease
Approach to congenital Heart Disease
  Acyanotic
√ enlarged main pulmonary artery
Cyanotic
√ concave main pulmonary artery
Increased Pulmonary BF
+ increased C/T ratio
L-R shunts
VSD
ASD
PDA
ECD
PAPVR
Admixture lesions = bidirectional shunts = T-lesions
Transposition
Truncus arteriosus
TAPVR
Tricuspid atresia (without RVOT obstruction)
“Tingles” (single ventricle/atrium)
Normal Pulmonary Blood Flow
+ normal C/T ratio
+ pulmonary venous HT
LV outflow obstruction
AS
Coarctation
Interrupted aortic arch
Hypoplastic left heart
LV inflow obstruction
Obstructed TAPVR
Cor triatriatum
Pulmonary vein atresia
Congenital MV stenosis
Muscle disease
Cardiomyopathy
Myocarditis
Anomalous LCA
 
Decreased Pulmonary BF
+ normal C/T ratio
+ increased C/T ratio
  R-to-L shunts
+ nonrestrictive intracardiac shunt
Tetralogy of Fallot
Tricuspid atresia (with PS + nonrestrictive ASD)
Pulmonary atresia + nonrestrictive VSD
+ restrictive intracardiac shunt
Pulmonary atresia + ASD without VSD
PS with ASD/patent foramen ovale
Tricuspid atresia + PS + restrictive ASD
Trilogy of Fallot
Ebstein anomaly
Congenital tricuspid insufficiency
Incidence of CHD in Liveborn Infants
Overall incidence: 8–9:1000 livebirths
  • Most common CHD: mitral valve prolapse (5–20%), bicuspid aortic valve (2%) [usually not recognized before late infancy/childhood]
  • ASD + VSD + PDA account for 45% of all CHD
  • 12 lesions account for 89% of all CHD:
    Ventricular septal defect 30.3%
    Patent ductus arteriosus 8.6%
    Pulmonary stenosis 7.4%
    Septum secundum defect 6.7%
    Coarctation of aorta 5.7%
    Aortic stenosis 5.2%
    Tetralogy of Fallot 5.1%
    Transposition 4.7%
    Endocardial cushion defect 3.2%
    Hypoplastic right ventricle 2.2%
    Hypoplastic left heart 1.3%
    TAPVR 1.1%
    Truncus arteriosus 1.0%
    Single ventricle 0.3%
    Double outlet right ventricle 0.2%
P.580

High-risk pregnancy: (1) Previous sibling with CHD: 2–5%
(2) Previous 2 siblings with CHD: 10–15% (3) One parent with CHD: 2–10%
Most common causes for CHF + PVH in neonate:
  • Left ventricular failure due to outflow obstruction
  • Obstruction of pulmonary venous return
CHD Presenting in 1st Year of Life
  • VSD
  • d-transposition of great vessels
  • Tetralogy of Fallot
  • Isolated coarctation
  • Patent ductus arteriosus
  • Hypoplastic left heart syndrome
CHD Compatible with Relatively Long Life
  • Mild tetralogy: mild pulmonic stenosis + small VSD
  • Valvular pulmonic stenosis: with relatively normal pulmonary circulation
  • Transposition of great vessels: some degree of pulmonic stenosis + large VSD
  • Truncus arteriosus: delicate balance between systemic + pulmonary circulation
  • Truncus arteriosus type IV: large systemic collaterals
  • Tricuspid atresia + transposition + pulmonic stenosis
  • Eisenmenger complex
  • Ebstein anomaly
  • Corrected transposition without intracardiac shunt
Juxtaposition of Atrial Appendages
  • Tricuspid atresia with transposition
  • Complete transposition
  • Corrected transposition of great arteries
  • DORV
Continuous Heart Murmur
  • PDA
  • AP window
  • Ruptured sinus of Valsalva aneurysm
  • Hemitruncus
  • Coronary arteriovenous fistula
Presenting Age in CHD
Age Severe PVH PVH Shunt Vascularity
0-2 d Hypoplastic left heart Hypoplastic left heart
  Aortic atresia TAPVR above diaphragm
  TAPVR below diaphragm Complete transposition
  Myocardiopathy in IDM
3-7 d PDA in preterm infant
7-14 d CoA + VSD/PDA Coarctation of aorta
  Aortic valve stenosis Peripheral AVM
  Peripheral AVM
  Endocardial fibroelastosis
  Anomalous LCA
Syndromes with CHD
5 p–(Cri-du-chat) Syndrome
Incidence of CHD: 20%
DiGeorge Syndrome
  • = congenital absence of thymus + parathyroid glands
  • 1. Conotruncal malformation
  • 2. Interrupted aortic arch
Down Syndrome = MONGOLISM = TRISOMY 21
  • Endocardial cushion defect (25%)
  • Membranous VSD
  • Ostium primum ASD
  • AV communis
  • Cleft mitral valve
  • PDA
  • 11 rib pairs (25%)
  • Hypersegmented manubrium (90%)
Ellis-van Creveld Syndrome
Incidence of CHD: 50%
  • • polydactyly
  • single atrium
Holt-Oram Syndrome
  • = UPPER LIMB-CARDIAC SYNDROME
Incidence of CHD: 50%
  • ASD
  • VSD
  • Valvular pulmonary stenosis
  • Radial dysplasia
Hurler Syndrome
  • Cardiomyopathy
Ivemark Syndrome
Incidence of CHD: 100%
  • •asplenia
  • complex cardiac anomalies
Klippel-Feil Syndrome
Incidence of CHD: 5%
  • Atrial septal defect
  • Coarctation
Marfan Syndrome = ARACHNODACTYLY
  • Aortic sinus dilatation
  • Aortic aneurysm
  • Aortic insufficiency
  • Pulmonary aneurysm
Noonan Syndrome
  • Pulmonary stenosis
  • ASD
  • Hypertrophic cardiomyopathy
Osteogenesis Imperfecta
  • Aortic valve insufficiency
  • Mitral valve insufficiency
  • Pulmonic valve insufficiency
Postrubella Syndrome
  • • low birth weight
  • • deafness
  • P.581

  • • cataracts
  • •mental retardation
  • 1. Peripheral pulmonic stenosis
  • 2. Valvular pulmonic stenosis
  • 3. Supravalvular aortic stenosis
  • 4. PDA
Trisomy 13–15
  • VSD, tetralogy of Fallot, DORV
Trisomy 16–18
  • VSD, PDA, DORV
Turner Syndrome (XO) = OVARIAN DYSGENESIS
Incidence of CHD: 35%
  • Coarctation of the aorta (in 15%)
  • Bicuspid aortic valve
  • Dissecting aneurysm of aorta
Williams Syndrome = IDIOPATHIC HYPERCALCEMIA
  • • peculiar elfinlike facies
  • •mental + physical retardation
  • •hypercalcemia (not in all patients)
  • 1. Supravalvular aortic stenosis (33%)
  • 2. ASD, VSD
  • 3. Valvular + peripheral pulmonary artery stenosis
  • 4. Aortic hypoplasia, stenoses of more peripheral arteries
Shunt Evaluation
Evaluation of L-to-R Shunts
  • AGE
    • Infants:
      • Isolated VSD
      • VSD with CoA/PDA/AV canal
      • PDA
      • Ostium primum
    • Children/adults:
      • ASD
      • Partial AV canal with competent mitral valve
      • VSD/PDA with high pulmonary resistance
      • PDA without murmur
  • SEX
    • 99% chance for ASD/PDA in female patient
  • CHEST WALL ANALYSIS
    • 11 pair of ribs + hypersegmented manubrium:
    • Down syndrome
    • pectus excavatum + straight back syndrome + funnel chest:
    • prolapsing mitral valve
    • rib notching
  • CARDIAC SILHOUETTE
    • absent pulmonary trunk:
      • corrected transposition with VSD; pink tetralogy
    • left-sided ascending aorta:
      • corrected transposition with VSD
    • tortuous descending aorta:
      • aortic valve incompetence + ASD
    • huge heart:
      • persistent complete AV canal (PCAVC); VSD + PDA; VSD + mitral valve incompetence
    • enlarged left atrium:
      • intact atrial septum; mitral regurgitation (endocardial cushion defect, prolapsing mitral valve + ASD)
Differential Diagnosis of L-R Shunts
  RA RV PA LA LV Prox. Ao
ASD
VSD
PDA often ↑
Shunt with Normal Left Atrium
  • Precardiac shunt
    • Anomalous pulmonary venous connection
  • Intracardiac shunt
    • ASD (8%)
    • VSD (25%)
  • Postcardiac shunt
    • PDA (12%)
Aortic Size in Shunts
  • Extracardiac shunts
    • aorta enlarged + hyperpulsatile
    • 1. PDA
  • Pre- and intracardiac shunts
    • aorta small but not hypoplastic
    • 1. Anomalous pulmonary venous return
    • 2. ASD
    • 3. VSD
    • 4. Common AV canal
Cyanotic heart disease
Chemical cyanosis = PaO2 ≤94%
Clinical cyanosis = PaO2≤85%
  • Decrease in hemoglobin delays detectability!
  • Most common cause of cyanosis
— in newborn: transposition of great vessels
— in child: tetralogy of Fallot!
N.B.: tricuspid atresia = the great mimicker
Increased Pulmonary Blood Flow with Cyanosis
  • = ADMIXTURE LESIONS
  • = bidirectional shunt with 2 components:
    • mixing of saturated blood (L-R shunt) and unsaturated blood (R-L shunt)
    • NO obstruction to pulmonary blood flow
Evaluation process:
  • cardiomegaly
  • increased pulmonary blood flow
  • concave main pulmonary artery:
    • PA segment absent = transposition
    • PA segment present:
      • L atrium normal (= extracardiac shunt) = TAPVR
      • L atrium enlarged (= intracardiac shunt) = truncus arteriosus
N.B.: Overcirculation + cyanosis = complete transposition until proven otherwise!
Admixture Lesions = T-lesions
mnemonic: 5 T’s + CAD
  • Transposition of great vessels = complete TGV ± VSD
    • Most common cause for cyanosis in neonate
  • P.582

  • Tricuspid atresia with or without transposition + VSD
    • 2nd most common cause for cyanosis in neonate
  • Truncus arteriosus
  • Total anomalous pulmonary venous return (TAPVR) above diaphragm:
    • supracardiac
    • cardiac (coronary sinus/right atrium)
  • Tingle” = single ventricle
  • Common atrium
  • Aortic atresia
  • Double-outlet right ventricle (DORV type I)/Taussig-Bing anomaly (DORV type II)
Clues:
  • skeletal anomalies: Ellis-van Creveld syndrome (truncus/common atrium)
  • polysplenia: common atrium
  • R aortic arch: persistent truncus arteriosus
  • ductus infundibulum: aortic atresia
  • pulmonary trunk seen: supracardiac TAPVR; DORV; tricuspid atresia; common atrium
  • ascending aorta with leftward convexity: single ventricle
  • dilated azygos vein: common atrium + polysplenia + interrupted IVC; TAPVR to azygos vein
  • left-sided SVC: vertical vein of TAPVR
  • “waterfall” right hilum: single ventricle + transposition
  • large left atrium (rules out TAPVR)
  • prominent L heart border: single ventricle with inverted rudimentary R ventricle; levoposition of right atrial appendage (tricuspid atresia + transposition)
  • age of onset ≤2 days: aortic atresia
Decreased Pulmonary Blood Flow with Cyanosis
  • = two components of
    • impedance of blood flow through right heart due to obstruction/atresia at pulmonary valve/infundibulum
    • R-to-L shunt
  • • pulmonary circulation maintained through systemic arteries/PDA
  • normal/decreased pulmonary blood flow
  • concave main pulmonary artery
  • cardiomegaly
  • restrictive intracardiac R-to-L shunt
mnemonic: P2 TETT
  • Pulmonic stenosis with ASD
  • Pulmonic atresia
  • Tetralogy of Fallot
  • Ebstein anomaly
  • Tricuspid atresia with pulmonic stenosis
  • Transposition of great vessels with pulmonic stenosis
  • SHUNT AT VENTRICULAR LEVEL
    • 1. Tetralogy of Fallot
    • 2. Tetralogy physiology (associated with pulmonary obstruction):
      • — complete/corrected transposition
      • — single ventricle
      • — DORV
      • — tricuspid atresia (PS in 75%)
      • — asplenia syndrome
    • prominent aorta with L/R aortic arch; inapparent pulmonary trunk
    • NORMAL R atrium (without tricuspid regurgitation)
    • NORMAL-sized heart (secondary to escape mechanism into aorta)
    Clues:
    • Skeletal anomaly (eg, scoliosis): tetralogy (90%)
    • Hepatic symmetry: asplenia
    • Right aortic arch: tetralogy, complete transposition, tricuspid atresia
    • Aberrant right subclavian artery: tetralogy
    • Leftward convexity of ascending aorta: single ventricle with inverted right rudimentary ventricle, corrected transposition, asplenia, JAA (tricuspid valve atresia)
  • SHUNT AT ATRIAL LEVEL
    mnemonic: PET
    • Pulmonary stenosis/atresia with intact ventricular septum
    • Ebstein malformation + Uhl anomaly
    • Tricuspid atresia (ASD in 100%)
    • moderate to severe cardiomegaly
    • R atrial dilatation
    • R ventricular enlargement (secondary to massive tricuspid incompetence)
    • inapparent aorta
    • left aortic arch
Pulmonary Venous Hypertension with Cyanosis
  • during 1st week of life
    • 1. Hypoplastic left heart syndrome
      • marked cardiomegaly
    • 2. TAPVR below diaphragm
      • normal cardiac size
  • during 2nd week of life
    • 3. Aortic coarctation
    • 4. Aortic atresia
  • during 4–6th week of life
    • 5. Critical aortic stenosis
    • 6. Endocardial fibroelastosis
    • 7. Anomalous origin of LCA
    • 8. Atresia of common pulmonary vein
Acyanotic heart disease
Increased Pulmonary Blood Flow without Cyanosis
  • = indicates L-R shunt with increased pulmonary blood flow (shunt volume >40%)
  • WITH LEFT ATRIAL ENLARGEMENT
    • indicates shunt distal to mitral valve = increased volume without escape defect
    • 1. VSD (25%): small aorta in intracardiac shunt
    • 2. PDA (12%): aorta + pulmonary artery of equal size in extracardiac shunt
    • 3. Ruptured sinus of Valsalva aneurysm (rare)
    • 4. Coronary arteriovenous fistula (very rare)
    • 5. Aortopulmonary window (extremely rare)
  • WITH NORMAL LEFT ATRIUM
    • indicates shunt proximal to mitral valve = volume increased with escape mechanism through defect
    • P.583

    • 1. ASD (8%)
    • 2. Partial anomalous pulmonary venous return (PAPVR) + sinus venosus ASD
    • 3. Endocardial cushion defect (ECD) (4%)
Normal Pulmonary Blood Flow without Cyanosis
  • OBSTRUCTIVE LESION
    • Right ventricular outflow obstruction
      • at level of pulmonary valve: subvalvular/valvular/supravalvular pulmonic stenosis
      • at level of peripheral pulmonary arteries:
        • peripheral pulmonary stenosis
    • Left ventricular inflow obstruction
      • at level of peripheral pulmonary veins:
        • pulmonary vein stenosis/atresia
      • at level of left atrium: cor triatriatum
      • at level of mitral valve:
        • supravalvular mitral stenosis, congenital mitral stenosis/atresia, “parachute” mitral valve
    • Left ventricular outflow obstruction
      • at level of aortic valve:
        • anatomic subaortic stenosis, functional subaortic stenosis (IHSS), valvular aortic stenosis, hypoplastic left heart, supravalvular aortic stenosis
      • at level of aorta:
        • interruption of aortic arch, coarctation of aorta
  • CARDIOMYOPATHY
    • Endocardial fibroelastosis
    • Hypertrophic cardiomyopathy
    • Glycogen storage disease
  • hyperdynamic STATE
    • Noncardiac AVM (cerebral AVM, vein of Galen aneurysm, large pulmonary AVM, hemangioendothelioma of liver)
    • Thyrotoxicosis
    • Anemia
    • Pregnancy
  • MYOCARDIAL ISCHEMIA
    • Anomalous left coronary artery
    • Coronary artery disease (CAD)
Pulmonary vascularity
Normal Pulmonary Vasculature
  • Vascular Distribution
    • pulmonary vessels within upper perihilum approximate 1/3 of total vascularity
    • pulmonary vessels within lower perihilum approximate 2/3 of total vascularity
  • Vascular Tapering
    • pulmonary vessels taper near transition of middle 1/3 to outer 1/3 of lung
  • Vascular Caliber
    • straight/slightly concave main pulmonary artery contour (mild convexity is normal in young females)
    • CT:
      • upper limits of normal for main pulmonary artery = 3 cm
      • pulmonary trunk measures <4.5 cm (leftward distance from vertical line at carina to most lateral aspect of main pulmonary artery contour)
      • right interlobar/intermediate pulmonary artery measures 10–15 mm in males and 9–14 mm in females on PA radiographs
      • pulmonary vessel size <1–2 mm in extreme lung periphery
      • arteries within 1st anterior intercostal space measure ≤3 mm
Normal Pulmonary Vascularity & Normal-sized Heart
mnemonic: MAN
  • Myocardial ischemia
  • Afterload (= pressure overload problems)
  • Normal
Increased Pulmonary Vasculature
  • OVERCIRCULATION
    • = shunt vascularity = arterial + venous overcirculation
    • (a) congenital heart disease (most common)
      • L-R shunts
      • Admixture cyanotic lesions
    • (b) high-flow syndromes
      • Thyrotoxicosis
      • Anemia
      • Pregnancy
      • Peripheral arteriovenous fistula
    • diameter of right descending pulmonary artery larger than trachea just above aortic knob
    • increased size of veins + arteries with size larger than accompanying bronchus (= “kissing cousin” sign), best seen just above hila on AP view
    • enlarged hilar vessels (lateral view)
    • visualization of vessels below 10th posterior rib
  • PULMONARY VENOUS HYPERTENSION
    • redistribution of flow (not seen in younger children)
    • indistinctness of vessels with Kerley lines (= interstitial edema)
    • fine reticulated pattern
    • alveolar edema
  • PRECAPILLARY HYPERTENSION
    • enlarged main + right and left pulmonary arteries
    • abrupt tapering of pulmonary arteries
  • PROMINENT SYSTEMIC/AORTOPULMONARY COLLATERALS
    • Tetralogy of Fallot with pulmonary atresia (= pseudotruncus)
    • VSD + pulmonary atresia (single ventricle, complete transposition, corrected transposition)
    • Pulmonary-systemic collaterals
    • coarse vascular pattern with irregular branching arteries (from aorta/subclavian arteries)
    • small central vessels despite apparent increase in vascularity
Decreased Pulmonary Vascularity
  • = obstruction to pulmonary flow
  • vessels reduced in size and number
  • hyperlucent lungs
  • small pulmonary artery segment + hilar vessels
P.584

Pulmonary artery
Invisible Main Pulmonary Artery
  • Underdeveloped = RVOT obstruction
    • Tetralogy of Fallot
    • Hypoplastic right heart syndrome (tricuspid/pulmonary atresia)
  • Misplaced pulmonary artery
    • Complete transposition of great vessels
    • Persistent truncus arteriosus
Decreased Diameter of Pulmonary Artery
  • Lung cancer
    • Tumor extending >180° of arterial circumference of main pulmonary artery indicates unresectability!
  • Mediastinal fibrosis
  • Takayasu arteritis
  • Chronic thromboembolic disease
Unequal Pulmonary Blood Flow
  • Tetralogy of Fallot
    • diminished flow on left side (hypoplastic/stenotic pulmonary artery in 40%)
  • Persistent truncus arteriosus (esp. type IV)
    • diminished/increased blood flow to either lung
  • Pulmonary valvular stenosis
    • increased flow to left lung secondary to jet phenomenon
Dilatation of Pulmonary Artery
  • Idiopathic dilatation of pulmonary trunk
  • Pulmonic valve stenosis
    • poststenotic dilatation of trunk + left pulmonary a.
  • Pulmonary regurgitation
    • severe pulmonic valve insufficiency
    • absence of pulmonic valve (may be associated with tetralogy)
  • Congenital L-to-R shunts
  • Pulmonary arterial hypertension
  • Aneurysm: mycotic/traumatic
  • Intravascular pulmonary metastases
Filling Defect in Pulmonary Artery
  • Thromboembolism (99%)
  • Nonthrombotic embolus:
    • fat droplets, bubbles of air/nitrogen, tumor, foreign bodies (talc, polymethylmethacrylate particles)
    • Particles mostly too small to be visualized
  • Primary sarcoma
Evaluation of Pulmonary vasculature on Erect chest film
  Normal PAH PVH Overload Overload
Distribution (UL:LL) 1:2 1:2 1:1 or 1:2 1:1
Vessel tapering middle:outer third variably pruned outer third outer third
Vascular caliber   increased increased increased
Artery-to-bronchus ratio
   upper lung zone 0.85 ± 0.15   1.50 ± 0.25 1.62 ± 0.31
   lower lung zone 1.34 ± 0.25   0.87 ± 0.20 1.56 ± 0.28
Vessel margins sharp sharp obscured obscured obscured
Pulmaonary Hypertension
  • = sustained pulmonary arterial pressure in systole >25 mm Hg at rest/ >30 mm Hg during exercise (normal levels, 10 and 15 mm Hg) determined by right heart catheterization secondary to reduction in cross-sectional area of the pulmonary vascular bed with concomitant increase in pulmonary vascular resistance
Definition: central pulmonary pressure >30 mm Hg
Cause: cardiac/pulmonary/hepatic disease > primary pulmonary hypertension
  • often not clinically recognized until advanced stage
CT:
  • vascular signs:
    • main pulmonary artery diameter >29 mm (87% sensitive, 89% specific) measured in the plane of PA bifurcation just lateral to ascending aorta
    • segmental artery-to-bronchus ratio >1 in three lobes
    • diameter ratio of main pulmonary artery to aorta >1 (strong correlation in patients <50 years)
    • diameter of left + right pulmonary artery >16 mm (poor indicator of pulmonary arterial hypertension)
    • “pruning” of peripheral pulmonary arteries
      • = disproportionate increase in caliber of central fibrous arteries (from sustained increase in flow by a factor of >2) + decrease in caliber of smaller muscular arteries (from vasoconstriction)
    • pulmonary veins
      • small: secondary to precapillary pulmonary HTN
      • enlarged: secondary to left-sided heart disease
    • enlargement of bronchial systemic arteries to >1.5 mm (more common in chronic thromboembolic pulmonary HTN than primary pulmonary HTN)
    • vascular complications:
      • subpleural pulmonary infarcts (with elevated pulmonary venous pressure/underlying malignancy)
      • calcified plaques of central pulmonary arteries (PATHOGNOMONIC)
      • dissection/massive thrombosis of central pulmonary arteries
  • lung parenchymal signs:
    • mosaic perfusion without dilatation of bronchi (= increase in vessel diameter in areas of hyperattenuation + tapering of peripheral vessels in areas of hypoattenuation); most common in chronic pulmonary thromboembolism
  • mediastinal & cardiac signs:
    • right heart enlargement + hypertrophy:
      • right heart dilatation = ratio of RV:LV > 1:1
      • P.585

      • bowing of interventricular septum toward LV
      • RV myocardial thickness >4 mm
    • dilatation of IVC + coronary sinus
    • reflux of contrast medium into IVC + hepatic veins (usefulness diminishes with injection rate >3 mL/sec)
    • mild pericardial thickening/small pleural effusion
    • adenopathy + septal lines + ground-glass opacities suggestive of pulmonary veno-occlusive disease
    • NO increase of pulsations in middle third of lung
MR:
  • RV hypertrophy (in almost 100% if RV systolic pressure exceeds 70 mm Hg):
    • mean thickness of RV wall = 6 mm
  • RV dilatation:
    • mean RV end-diastolic volume = 80 mL
    • tricuspid regurgitation (from dilatation of tricuspid valve annulus)
  • enlargement of RA + IVC + SVC
  • septum straight/bowed toward LV
  • diminished RV ejection fraction (mean of 45%)
  • reduced distensibility of main pulmonary artery (mean of 8%)
  • systolic intraluminal signals in pulmonary arteries (due to slow flow) correlating with severity of PAH
Cx: central arterial thrombosis, premature atherosclerosis of central elastic + muscular pulmonary arteries, aneurysmal dissection of pulmonary arteries, hypertrophy + dilatation of right side of heart
Dx: clinical assessment of hemodynamic parameters, medical history, histologic findings
Pulmonary Arterial Hypertension (PAH)
Classification by anatomic level:
  • Precapillary cause
    • = changes limited to pulmonary arterial circulation @ level of muscular arteries
    • 1. Chronic thromboembolic disease
    • 2. In-situ pulmonary arterial thrombotic disease (polycythemia, sickle-cell disease)
    • 3. Widespread pulmonary embolism from intra-vascular malignant cells/parasites/foreign material
    • 4. Pulmonary vasculitis (eg, collagen vascular disease, especially scleroderma, CREST, HIV)
    • 5. Long-standing L-to-R shunt = Eisenmenger syndrome with reversal of L-to-R shunt
    • 6. Alveolar hypoventilation syndromes
    • 7. Primary pulmonary hypertension (idiopathic)
  • Pulmonary parenchymal disease
    • = mechanical interference with small pulmonary arteries (classified under precapillary cause)
    • Hypoxic lung disease:
      • Chronic obstructive pulmonary disease
      • Interstitial pulmonary fibrosis
      • Ventilatory failure from primary chest wall dysfunction
    • Connective tissue disorder:
      • Scleroderma
      • CREST (calcinosis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, telangiectasia)
    • Infection:
      • HIV (6–12 × compared to general population)
      • Sarcoidosis
      • Tuberculosis
  • Postcapillary cause
    • = Pulmonary venous hypertension
    • = findings located in pulmonary venous circulation + between capillary bed and left atrium
    • Left atrial obstruction (usually MV disease, left heart failure, left atrial tumor)
    • Mediastinal fibrosis (narrowing of pulmonary veins), may also affect precapillary vessels
    • Pulmonary veno-occlusive disease (idiopathic)
Pathogenesis:
  • hyperkinetic CAUSES
    • L-to-R shunt
    • High cardiac output states: thyrotoxicosis, chronic anemia
  • OBLITERATIVE CAUSES
    • vascular = precapillary pulmonary hypertension:
      • Primary plexogenic pulmonary arteriopathy = Primary pulmonary hypertension (PPH)
      • Arteritis (eg, Takayasu)
      • Embolization
        • –chronic thromboembolic disease
        • –tumor
          • lymphangitic carcinomatosis
        • –parasites, eg, schistosomiasis
          • hepatosplenomegaly
        • –talc crystals
          • micronodular opacities
          • perihilar fibrotic masses
      • Persistent fetal circulation
      • Pulmonary capillary hemangiomatosis
    • pleuropulmonic disease
      • Chronic interstitial lung disease
        • = cor pulmonale:
        • COPD, emphysema, chronic bronchitis, asthma, bronchiectasis, malignant infiltrate, granulomatous disease, cystic fibrosis, end-stage fibrotic lung, S/P lung resection, idiopathic hemosiderosis, alveolar proteinosis, alveolar microlithiasis
      • Pleural disease + chest deformity: fibrothorax, thoracoplasty, kyphoscoliosis
    • vasoconstrictive
      • Chronic alveolar hypoxia
        • = hypoxic pulmonary arterial hyperperfusion:
        • chronic high altitude, sleep apnea, chronic hypercapnea due to hypoventilation from neuromuscular disease/obesity
      • Portopulmonary hypertension (rare)
  • Chronic pulmonary venous hypertension
Cor Pulmonale
mnemonic: TICCS BEV
  • Thoracic deformity
  • Idiopathic: primary pulmonary hypertension (1%)
  • Chronic pulmonary embolism
  • COPD
  • P.586

  • Shunt (ASD, VSD, etc)
  • Bronchiectasis
  • Emphysema
  • Vasculitis
Pulmonary Venous Hypertension (PVH)
  • = INCREASED VENOUS PULMONARY PRESSURE = VENOUS CONGESTION
  • = postcapillary pulmonary hypertension = primary findings located within pulmonary venous circulation between capillary bed + left atrium
Dx: uniform/widely variable elevation of pulmonary capillary wedge pressure (PCWP) >15 mm Hg
Cause:
  • LEFT VENTRICULAR INFLOW TRACT OBSTRUCTION
    • normal-sized heart with right ventricular hypertrophy
    • prominent pulmonary trunk
    • @ proximal to mitral valve:
      • normal-sized left atrium
      • pulmonary veins
        • TAPVR below the diaphragm
        • Primary pulmonary venoocclusive disease (PVOD)
        • Stenosis of individual pulmonary veins
        • Atresia of common pulmonary vein
      • mediastinum
        • Fibrosing mediastinitis (may also affect precapillary vessels)
        • Constrictive pericarditis
      • left atrium
        • Cor triatriatum
        • Left atrial mass: tumor, clot
        • Supravalvular ring of left atrium
    • @ at mitral valve level = mitral valve stenosis
      • enlarged left atrium
      • 1. Rheumatic mitral valve stenosis ±regurgitation (99%)
      • enlarged left atrial appendage
      • 2. Congenital mitral valve stenosis
      • 3. Parachute mitral valve (= single bulky papillary muscle)
  • LEFT VENTRICULAR FAILURE
    • ABNORMAL PRELOAD with secondary mitral valve incompetence (= volume overload)
      • Aortic valve regurgitation
      • Eisenmenger syndrome (= R-to-L shunt in VSD)
      • High-output failure:
        • noncardiac AVM (cerebral AVM, vein of Galen aneurysm, large pulmonary AVM, hemangioendothelioma of liver, iatrogenic), thyrotoxicosis, anemia, pregnancy
    • ABNORMAL afterload
      • (= pressure overload)
      • = LV outflow tract obstruction
      • Hypoplastic left heart syndrome
      • Aortic stenosis (supravalvular, valvular, anatomic subaortic)
      • Interrupted aortic arch
      • Coarctation of the aorta
    • DISORDERS OF CONTRACTION AND RELAXATION
      • Endocardial fibroelastosis
      • Glycogen storage disease (Pompe disease)
      • Cardiac aneurysm
      • Cardiomyopathy
        • congestive (alcohol)
        • hypertrophic obstructive cardiomyopathy (HOCM), particularly in IDM
          • asymmetric septal hypertrophy (ASH)
          • idiopathic hypertrophic subaortic stenosis (IHSS)
    • MYOCARDIAL ISCHEMIA
      • Anomalous left coronary artery
      • Coronary artery disease (CAD)
Histo:
  • primary changes: venous medial hypertrophy + intimal proliferation, marked thickening of venous internal elastic lamina
  • secondary changes: capillary bed congestion with adjacent vascular proliferation, interlobular septal and pleural edema + fibrosis, lymphatic dilatation, alveolar hemosiderosis, paraseptal venous infarcts adjacent to complete venous occlusion
Cx: secondary pulmonary arterial hypertension
  • equalization of pulmonary vascularity (PCWP 13–15 mm Hg)
  • cephalization of pulmonary vascularity (PCWP 16–18 mm Hg)
  • interstitial pulmonary edema (PCWP 19–24 mm Hg)
    • = fluid within peribronchovascular connective tissue:
    • peribronchial thickening/cuffing
    • indistinct vessel margins
    • Kerley B lines = short horizontal reticulations within lateral subpleural lung bases
    • Kerley A lines = 3–4-cm-long lines of interlobular septal thickening radiating from hila to mid and upper lung zones
    • perihilar haze = hilar interstitial edema
    • thickened pleural fissures/pseudoeffusion = fluid within subpleural connective tissue
  • alveolar pulmonary edema (PCWP ≥ 25 mm Hg) = bilateral perihilar and basilar airspace opacification
  • small pleural effusions
Cardiomegaly
  • CHF
  • Multivalvular disease
  • Pericardial effusion
    • absence of pulmonary venous hypertension + hydrostatic edema
Cardiothoracic Ratio
  • = widest transverse cardiac diameter ÷ widest inside thoracic diameter
  • = primarily a measure of LV dilatation
<0.45 normal
0.45—0.55 mild cardiomegaly
>0.55 moderate/severe cardiomegaly
Falsely normal: with LV enlargement of up to 66%, moderate enlargement of LA + RV
Falsely elevated: in expiration, in recumbent position
P.587

Vascular Pedicle Width
  • = distance on a horizontal line between (1) point where right mainstem bronchus + SVC cross and (2) point where left subclavian artery crosses horizontal line
48 ± 5 mm normal
>53 mm in 60% of cardiogenic edema,
in 85% of volume overload
Cardiomegaly in Newborn
  • NONCARDIOGENIC
    • metabolic:
      • Ion imbalance in serum levels of sodium, potassium, and calcium
      • Hypoglycemia
    • decreased ventilation
      • Asphyxia
      • Transient tachypnea
      • Perinatal brain damage
    • erythrocyte function
      • Anemia
      • Erythrocythemia
    • endocrine
      • Glycogen storage disease
      • Thyroid disease: hypo-/hyperthyroidism
    • infant of diabetic mother
    • arteriovenous fistula
      • Vein of Galen aneurysm
      • Hepatic angioma
      • Chorioangioma
  • CARDIOGENIC
    • Arrhythmia
    • Myo-/pericarditis
    • Cardiac tumor
    • Myocardial infarction
    • Congenital heart disease
Abnormal Heart Chamber Dimensions
  • LEFT VENTRICULAR VOLUME OVERLOAD
    • VSD
    • PDA
    • Mitral incompetence
    • Aortic incompetence
  • LEFT VENTRICULAR HYPERTROPHY
    • Coarctation
    • Aortic stenosis
  • RIGHT VENTRICULAR VOLUME OVERLOAD
    • ASD
    • Partial APVR/total APVR
    • Tricuspid insufficiency
    • Pulmonary insufficiency
    • Congenital/acquired absence of pericardium
    • [Ebstein anomaly]–not truly RV
  • RIGHT VENTRICULAR HYPERTROPHY
    • Pulmonary valve stenosis
    • Pulmonary hypertension
    • Tetralogy of Fallot
    • VSD
  • Fixed subvalvular aortic stenosis
  • Hypoplastic left/right ventricle, common ventricle
  • Congestive cardiomyopathy
Right Atrial Enlargement
Cause: tricuspid stenosis/regurgitation, ASD, atrial fibrillation, dilated cardiomyopathy, Ebstein anomaly, pulmonary atresia
  • RA enlarges in rightward + posterior direction
PA CXR:
  • prominent round superior border at junction with SVC
  • >5.5 cm from midline to most lateral RA margin
  • >2.5 cm from right vertebral margin
  • >50% vertical height of RA compared with cardiovascular mediastinal height (from top of aortic arch to base of heart)
LAT CXR:
  • sharp horizontal interface with lung above RV
  • displacement of heart posterior to IVC mimicking LV enlargement
Right Ventricular Enlargement
Cause: pulmonary valve stenosis, cor pulmonale, ASD, tricuspid regurgitation, dilated cardiomyopathy, secondary to LV failure
  • RV enlarges in anterior, superior + leftward direction causing levorotation of heart
PA CXR:
  • Only extreme dilatation causes recognizable signs on frontal view!
  • straightening/convexity of left upper cardiac contour
  • upturned cardiac apex
  • left upper cardiac margin parallels left mainstembronchus as a long convex curvature
  • increased distance between left upper cardiac margin + left mainstem bronchus
  • small appearance of rotated aortic arch + SVC
  • large appearance of main pulmonary artery
LAT CXR:
  • prominent convexity of anterior heart border >1/3 distance from anterior cardiophrenic sulcus to sternal angle
Left Atrial Enlargement
Cause:
  • acquired: mitral stenosis/regurgitation, LV failure, LA myxoma
  • congenital: VSD, PDA, hypoplastic left heart
  • LA enlarges in multiple directions
PA CXR:
  • right retrocardiac double density with inferomedial curvature (earliest sign)
  • >7.0 (female)/7.5 (male) cm distance between midpoint of undersurface of left mainstem bronchus + right lateral LA shadow
  • left retrocardiac double density
  • >75° splaying of carina with horizontal orientation of distal left mainstem bronchus
  • P.588

  • enlarged left-convex left atrial appendage ± calcifications (in 90% due to rheumatic heart disease)
LAT CXR:
  • increased convexity of posterosuperior cardiac margin
  • posterosuperior atrial convexity crosses vertical plane formed by tracheal midline + upper lobe bronchus
  • posterior displacement of barium-filled esophagus
  • posterior displacement of LUL bronchus
Left Ventricular Enlargement
Cause:
  • pressure overload: hypertension, aortic stenosis
  • volume overload: aortic or mitral regurgitation, VSD
  • wall abnormalities: LV aneurysm, hypertrophic cardiomyopathy
  • LV enlarges in posterior, inferior + leftward direction
PA CXR:
  • leftward displacement of downturned cardiac apex = left ventricular configuration
  • depression of left hemidiaphragm + gastric bubble (with diaphragmatic inversion)
LAT CXR:
  • increased convexity of posteroinferior cardiac margin
  • posterior cardiac margin projects >1.8 cm posterior to IVC measured at a point 2 cm above intersection of IVC with right hemidiaphragm (Hofman-Rigler rule)
Neonatal cardiac failure
  • LEFT-SIDED OBSTRUCTIVE LESIONS
    • Segmental hypoplasia of aorta
    • Critical coarctation of the aorta
    • Aortic valve stenosis
    • Asymmetrical septal hypertrophy/hypertrophic obstructive cardiomyopathy
    • Mitral valve stenosis
    • Cor triatriatum
  • VOLUME OVERLOAD
    • Congenital mitral valve incompetence
    • Corrected transposition with left (= tricuspid) AV valve incompetence
    • Congenital tricuspid insufficiency
    • Ostium primum ASD
  • MYOCARDIAL DYSFUNCTION/ISCHEMIA
    • Nonobstructive cardiomyopathy
    • Anomalous origin of LCA from pulmonary trunk
    • Primary endocardial fibroelastosis
    • Glycogen storage disease (Pompe disease)
    • Myocarditis
  • NONCARDIAC LESIONS
    • AV fistulas: hemangioendothelioma of liver, AV fistula of brain, vein of Galen aneurysm, large pulmonary AV fistula
    • Transient tachypnea of the newborn
    • Intraventricular/subarachnoid hemorrhage
    • Neonatal hypoglycemia (low birth weight, infants of diabetic mothers)
    • Thyrotoxicosis (transplacental passage of LATS hormone)
Congestive Heart Failure & Cardiomegaly
mnemonic: Ma McCae & Co.
  • Myocardial infarction
  • anemia
  • Malformation
  • cardiomyopathy
  • Coronary artery disease
  • aortic insufficiency
  • effusion
  • Coarctation
Congenital Cardiomyopathy
mnemonic: CAVE GI
  • Cystic medial necrosis of coronary arteries
  • Aberrant left coronary artery/Absent coronary a.
  • Viral myocarditis
  • P.589

  • Endocardial fibroelastosis
  • Glycogen storage disease (Pompe)
  • Infant of diabetic mother/Ischemia
Approach to Acquired Heart Disease
Criteria Mild-Moderate Cardiomegaly Moderate–Severe Cardiomegaly
C/T ratio 0.45-0.55 >0.55
LA enlargement pressure overload volume overload
     mitral stenosis    mitral insufficiency
     decreased LV compliance
     hypertrophic cardiomyopathy
     restrictive cardiomyopathy
Enlargement of ascending aorta pressure overload volume overload
     aortic stenosis    aortic insufficiency
Normal LA + aorta myocardial myocardial
     acute infarction    dilated cardiomyopathy
     hypertrophic cardiomyopathy    ischemic cardiomyopathy
     restrictive cardiomyopathy
  pericardial pericardial
     constrictive pericarditis    pericardial effusion
Acquired heart disease
  • LA enlargement = MV disease
  • dilated ascending aorta = aortic valve disease
  • RA enlargement = tricuspid valve disease
Pressure Overload
  • Systemic hypertension
  • Aortic stenosis
  • Mitral stenosis
Decreased Compliance
  • Myocardial infarction
  • Hypertrophic cardiomyopathy
  • Restrictive cardiomyopathy
Volume Overload
  • Aortic insufficiency
  • Mitral insufficiency
  • Tricuspid insufficiency
Aorta
Enlarged Aorta
  • PA CXR:
    • aortic knob >4.0 cm measured from indented trachea to most lateral margin of aorta
    • right convex contour above RA margin + lateral displacement of SVC (= dilatation of ascending aorta)
  • INCREASED VOLUME LOAD
    • Aortic insufficiency
    • PDA
  • POSTSTENOTIC DILATATION
    • Valvular aortic stenosis
  • INCREASED INTRALUMINAL PRESSURE
    • Coarctation
    • Systemic hypertension
  • MURAL WEAKNESS/INFECTION
    • Cystic media necrosis: Marfan/Ehlers-Danlos syndrome
    • Congenital aneurysm
    • Syphilitic aortitis
    • Mycotic aneurysm
    • Atherosclerotic aneurysm (compromised vasa vasorum)
  • LACERATION OF AORTIC WALL
    • Traumatic aneurysm
    • Dissecting hematoma
Aortic Wall Thickening
  • Intramural hematoma
    • = aortic dissection without intimal tear
  • Aortitis
    • segments of aortic arch + branch vessels
  • Atherosclerotic plaque
    • irregular narrowing of aortic lumen
  • Adherent thrombus
Aortic Calcifications
Intimal Calcification
Cause: part of atherosclerotic plaque
Associated with: inflammatory cells, lipid, vascular smooth muscle cells
Site: within perimeter of internal elastic lamina
  • discrete punctate lesion of radiograph
Medial Calcification
Cause: aging, diabetes, end-stage renal disease, neuropathy, genetic syndromes
Associated with: elastin + vascular smooth muscle cells
  • linear deposit along elastic lamellae resembling railroad tracks (when severe)
Double Aortic Arch
  • Most common + serious type of a complete vascular ring; usually isolated condition
Embryology: failure of regression of either arch
Incidence: 55% of all vascular rings
image
Edwards’ Hypothetical Aortic Arch Development
image
Right Aortic Arch with Aberrant Left Subclavian Artery
image
Right Aortic Arch with Mirror-image Branching
image
Double Aortic Arch
image
Aberrant Left Pulmonary Artery
P.590

Age: usually detected in infancy
  • usually asymptomatic
  • stridor, dyspnea, recurrent pneumonia
  • dysphagia (less common than respiratory symptoms, more common after starting baby on solids)
Location: descending aorta in 75% on left, in 25% on right side; smaller arch anterior in 80%; right arch larger + more cephalad than left in 80%
  • two separate arches arise from single ascending aorta
  • each arch joins to form a common descending aorta
  • trachea in midline:
    • impressions may be present on both sides of trachea: usually R > L (in older children)
    • trachea narrowed and displaced posteriorly with small anterior impression
  • Esophagogram:
    • broad horizontal posterior indentation at the level of 3rd/4th thoracic vertebra (by right arch crossing obliquely to join left arch)
    • bilateral esophageal indentations with a reversed S-shaped configuration (= right indentation higher than left)
  • CT:
    • “four-artery sign” = each arch gives rise to 2 dorsal subclavian + 2 ventral carotid arteries evenly spaced around trachea on section cephalad to aortic arch
    DDx: right arch with aberrant left subclavian artery (indistinguishable by esophagogram when dominant arch on right side)
Right Aortic Arch
Incidence: 1–2%
Embryology: persistence of right aortic arch and right descending aorta + regression of left aortic arch
Course: to right of trachea + esophagus, over right mainstem bronchus; crosses lower thoracic spine; passes through left hemidiaphragm
Right Aortic Arch
Incidence: 1–2%
Embryology: persistence of right aortic arch and right descending aorta + regression of left aortic arch
Course: to right of trachea + esophagus, over right mainstem bronchus; crosses lower thoracic spine; passes through left hemidiaphragm
P.591

Incidence of right aortic arch in CHD:
1. Truncus arteriosus 35%
2. Pulmonary atresia 25%
3. Tetralogy of Fallot 20%
4. Tricuspid atresia 15%
5. DORV 12%
6. TGV 8%
7. Large VSD 2%
Rare anomalies:
  • Corrected transposition
  • Pseudotruncus
  • Asplenia
  • Pink tetralogy
Right Aortic Arch with Aberrant Left Subclavian Artery
  • = RAA with ALSA
  • = interruption of embryonic left arch between left CCA and left subclavian artery
  • Most common type of right aortic arch anomaly
  • 2nd most common cause of vascular ring after double aortic arch
Incidence: 1:2,500; 35–72% of right aortic arch anomalies
Associated with: congenital heart disease in 5–12%:
   1. Tetralogy of Fallot (2/3 = 8%)
   2. ASD ± VSD (1/4 = 3%)
   3. Coarctation (1/12 = 1%)
  • • usually asymptomatic (loose ring around trachea + esophagus)
  • • may be symptomatic in infancy/early childhood provoked by bronchitis + tracheal edema
  • • may be symptomatic in adulthood provoked by torsion of aorta
  • left common carotid artery is first branch of ascending aorta
  • left subclavian artery arises from descending aorta via the remnant of the left dorsal aortic root
  • bulbous configuration of origin of LSA (= remnant of embryonic left arch) = retroesophageal aortic diverticulum = diverticulum of Kommerell (N.B.: originally described as diverticular outpouching at origin of right subclavian artery with left aortic arch):
    • small rounded density left lateral to trachea
    • impression on left side of esophagus simulating a double aortic arch (by aortic diverticulum or ductus/ligamentum arteriosum)
  • vascular ring (= left ductus extends from aortic diverticulum to left pulmonary artery):
    • impression on tracheal air shadow (by right aortic arch)
    • right esophageal indentation (by right aortic arch)
    • masslike density silhouetting top of aortic arch just posterior to trachea on LAT CXR (by aberrant left subclavian artery)
    • broad posterior impression on esophagus (left subclavian artery/aortic diverticulum)
    • small anterior impression on trachea (by left common carotid artery)
    • descending aorta on right side
Right Aortic Arch with Mirror-image Branching
  • 2nd most common aortic arch anomaly: 24–60%
  • = interruption of embryonic left arch between left subclavian artery and descending aorta; dorsal to left ductus arteriosus
Interruption of Left Aortic Arch Distal to Ductus Arteriosus = Type 1 (common)
Associated with: cyanotic CHD in 98%:
   1. Tetralogy of Fallot (87%)
   2. Multiple defects (7.5%)
   3. Truncus arteriosus (2–6%)
   4. Transposition (1–10%)
   5. Tricuspid atresia (5%)
   6. ASD ± VSD (0.5%)
  • 25% of patients with tetralogy have right aortic arch!
  • 37% of patients with truncus arteriosus have right aortic arch!
  • NO vascular ring, NO retroesophageal component
  • NO structure posterior to trachea
  • R arch impression on tracheal air shadow
  • NORMAL barium swallow
Interruption of Left Aortic Arch Proximal to Ductus Arteriosus = Type 2 (rare)
  • = true vascular ring (if duct persists); rarely associated with CHD
Right Aortic Arch with Isolated Left Subclavian Artery
  • 3rd most common right aortic arch anomaly: 2%
  • = interruption of embryonic left arch between
    • left CCA and left subclavian artery and
    • left ductus and descending aorta
    • resulting in a connection of left subclavian artery with left pulmonary artery
Associated with: tetralogy of Fallot
  • left common carotid artery arises as the first branch
  • left subclavian artery attaches to left pulmonary artery through PDA
  • NO vascular ring, NO retroesophageal component
  • • congenital subclavian steal syndrome
Right Aortic Arch with Aberrant Left Brachiocephalic Artery
  • Similar in appearance to R aortic arch + aberrant L subclavian artery
Left Aortic Arch
Left Aortic Arch with Aberrant Right Subclavian Artery
  • = right subclavian artery arises as 4th branch from proximal descending aorta
Incidence: 0.4–2.3% of population; in 37% of Down syndrome children with CHD;
♢ Most common congenital aortic arch anomaly!
Associated with: (1) Absent recurrent pharyngeal n.
(2) CHD in 10–15%
Course: (a) behind esophagus (80%)
(b) between esophagus + trachea (15%)
(c) anterior to trachea (5%)
P.592

  • • asymptomatic/dysphagia lusoria (rare)
  • soft-tissue opacity crossing the esophagus obliquely upward toward the right shoulder (PATHOGNOMONIC)
  • masslike opacity in right paratracheal region
  • rounded opacity arising from superior aortic margin posterior to trachea + esophagus on LAT CXR
  • dilated origin of aberrant right subclavian artery (in up to 60%) = diverticulum of Kommerell = remnant of embryonic right arch
  • unilateral L-sided rib notching (if aberrant right subclavian artery arises distal to coarctation)
Anomalous Innominate Artery Compression Syndrome
  • = origin of R innominate artery to the left of trachea coursing to the right
  • anterior tracheal compression
  • • ablation of right radial pulse by rigid endoscopic pressure
  • posterior tracheal displacement
  • focal collapse of trachea at fluoroscopy
  • pulsatile indentation of anterior tracheal wall by innominate artery on MRI
Rx: surgical attachment of innominate artery to manubrium
image
Patterns of Vascular Compression of Esophagus and Trachea
Vascular rings
  • = anomaly characterized by encirclement of trachea + esophagus by aortic arch + branches
  • Usually symptomatic lesions
    • • chronic stridor, wheezing, recurrent pneumonia
    • • dysphagia, failure to thrive
    • Double aortic arch with R descending aorta + L ductus arteriosus
    • R aortic arch with R descending aorta + aberrant L subclavian artery + persistent L ductus/ligamentum arteriosum
      N.B.: left obliterated ductus arteriosus (= ligament of Botallo) passes from L pulmonary a. to descending aorta/L subclavian a.
      • • symptoms + radiographic findings identical to double aortic arch
      • P.593

      • indentation on right lateral esophageal wall (by aortic arch)
      • impression on the anterolateral esophageal wall (by ligament of Botallo)
      • origin of L subclavian artery frequently dilated
    • L arch with L descending aorta + R ductus/ligamentum
    • Aberrant L pulmonary artery = “pulmonary sling”
    • Frequency of CXR findings:
      • frontal CXR:
        • right aortic arch (85%)
        • focal indentation of distal trachea (73%)
      • lateral CXR:
        • anterior tracheal bowing (92%)
        • increased retrotracheal opacity (79%)
        • focal tracheal narrowing (77%)
  • OCCASIONALLY SYMPTOMATIC LESIONS
    • Anomalous R innominate artery
    • Anomalous L common carotid artery/common trunk
    • R aortic arch with L descending aorta + L ductus/ligamentum
  • USUALLY ASYMPTOMATIC LESIONS
    • L aortic arch + aberrant R subclavian artery
    • L aortic arch with R descending aorta
    • R aortic arch with R descending aorta + mirror-image branching
    • R aortic arch with R descending aorta + aberrant L subclavian artery
    • R aortic arch with R descending aorta + isolation of L subclavian artery
    • R aortic arch with L descending aorta + L ductus/ligamentum
Aortic stenosis
  • ACQUIRED
    • Takayasu aortitis
    • Radiation aortitis
    • Aortic dissection
    • Infected aortic aneurysm with abscess
    • Pseudoaneurysm from laceration
    • Atherosclerosis (rare)
    • Syphilitic aortitis (rare)
  • CONGENITAL
    • Williams syndrome
    • Neurofibromatosis
    • Rubella
    • Mucopolysaccharidosis
    • Hypoplastic left heart syndrome
Abnormal Left Ventricular Outflow Tract
  • LVOT = area between IVS + aML from aortic valve cusps to mitral valve leaflets
  • Membranous subaortic stenosis
    • = crescent-shaped fibrous membrane extending across LVOT + inserting at aML
    • diffuse narrowing of LVOT
    • abnormal linear echoes in LVOT space (occasionally)
  • Prolapsing aortic valve vegetation
  • Narrowed LVOT (<20 mm)
    • Long-segment subaortic stenosis
      • aortic valve closure in early systole with coarse fluttering
      • high-frequency flutter of mitral valve in diastole (aortic regurgitation)
      • symmetric LV hypertrophy
    • ASH/IHSS
      • asymmetrically thickened septum bulging into LV + LVOT
      • systolic anterior motion of aML (SAM)
    • Mitral stenosis
    • Endocardial cushion defect
Situs
  • = “position/site/location” referring to the position of the atria and viscera relative to the midline
    • systemic/right atrium
      • has a broad-based appendage
      • receives blood from IVC
      • has terminal crest + coarse pectinate muscles
    • pulmonary/left atrium
      • has a small narrow appendage
      • receives blood from pulmonary veins
  • SITUS SOLITUS = normal/usual situs
    •on right side: √ systemic atrium
    √ trilobed lung
    √ liver
    √ gallbladder
    √ IVC
    •on left side: √pulmonary atrium
    √bilobed lung
    √stomach
    √single spleen
    √aorta
    Associated with:
    (a) levocardia : 0.6–0.8% chance for CHD
    (b) dextrocardia : 95% chance for CHD
  • SITUS INVERSUS
    • = mirror-image arrangement of situs solitus
    Frequency: 0.01%
    •on left side: √systemic atrium
    √trilobed lung
    √liver
    √gallbladder
    √IVC
    •on right side: √pulmonary atrium
    √bilobed lung
    √stomach
    √ single spleen
    √aorta
    Associated with:
    • dextrocardia = situs inversus totalis (usual variant): 3–5% chance for CHD, eg, Kartagener syndrome (in 20%)
    • levocardia (extremely rare): 95% chance for CHD
  • SITUS AMBIGUUS = HETEROTAXIA
    • = visceral malposition + dysmorphism associated with indeterminate atrial arrangement
    • Subclassification:
      • Asplenia syndrome = double right-sidedness = right isomerism = Ivemark syndrome
        P.594

        • both lungs have 3 lobes
        • eparterial bronchi = main bronchus passes superior to ipsilateral main pulmonary artery
      • Polysplenia syndrome = double left-sidedness = left isomerism
        • both lungs have 2 lobes
        • hyparterial bronchi = main bronchus passes inferior to ipsilateral main pulmonary artery
Associated with: CHD in 50–100%
image
Situs Anomalies
Cardiac Malposition
  • location of heart other than within left hemothorax in situs solitus
  • location of heart within left hemithorax when other organs are ambiguous
  • Determined by base-apex axis; no assumption is made regarding cardiac chamber/vessel arrangement
  • POSITION OF CARDIAC APEX
    • Levocardia = apex directed leftward
    • Dextrocardia = apex directed rightward
    • Mesocardia = vertical/midline heart (usually with situs solitus)
    • atrial septum characteristically bowed into left atrium in cardiac situs solitus with dextrocardia + cardiac situs inversus with levocardia (DDx: juxtapositioned atrial appendages)
  • CARDIAC DISPLACEMENT
    • by extracardiac factors (eg, lung hypoplasia, pulmonary mass)
    • Dextroposition
      • suggests hypoplasia of ipsilateral pulmonary artery (PAPVR implies scimitar syndrome)
    • Levoposition
    • Mesoposition
  • CARDIAC INVERSION
    • alteration of normal relationship of chambers
    • D-bulboventricular loop
    • L-bulboventricular loop
  • TRANSPOSITION
    • = alteration of anterior-posterior relationship of great vessels
Cardiac tumor
Prevalence: 0.001–0.03% (autopsy series)
  • Rare often asymptomatic tumors until very large!
  • • symptoms of cardiopulmonary diseases:
    • • congestive heart failure: dyspnea, orthopnea, peripheral edema, paroxysmal nocturnal dyspnea
    • • palpitations, heart murmur
    • • cough, chest pain
  • • symptoms caused by peripheral emboli to cerebral/systemic/coronary circulation:
    • • syncope
  • • weight loss, fever, malaise
Location: intracavitary (obstruction, emboli), intramural (arrhythmia), pericardial (tamponade)
CXR:
  • cardiomegaly, pericardial effusion
  • signs of CHF
  • abnormal cardiac contour
  • pleural effusion
Benign Heart Tumor in Adults
  • More common than malignant neoplasms
  • Myxoma (most common primary cardiac tumor; 50% of all primary cardiac tumors)
  • Papillary fibroelastoma (10% of all primary cardiac tumors; most common valvular tumor)
  • Lipoma
  • Hydatid cyst (uncommon):
    • localized bulge of left cardiac contour
    • curvilinear/spotty calcifications (resembling myocardial aneurysm)
Cx: may rupture into cardiac chamber/pericardium
Malignant Heart Tumors
Prevalence: 25% of all cardiac tumors in adults;
10% of all cardiac tumors in children
  • Sarcoma:
    • Majority of primary malignant cardiac neoplasms!
    • 2nd most common primary cardiac neoplasm
  • Rhabdomyosarcoma
    • Most common primary in children
  • P.595

  • Metastatic disease
    • 20–1000 times more frequent than primary tumor!
      • to peri-/epicardium lung > breast > lymphoma > leukemia (most common)
      • myocardium: malignant melanoma; secondary extension to myocardium from epicardium
      • endocardial/intracavitary (in only 5%)
  • Lymphoma (rare)
  • Malignant teratoma
  • Multiple cardiac myxomas
Congenital Cardiac Tumor
Incidence: 1:10,000
  • Rhabdomyoma (58%): usually multiple masses
  • Teratoma (20%): intrapericardiac, extracardiac
    • multicystic mass
  • Fibroma (12%): intramural
    May be associated with: Gorlin syndrome
    Location: free LV wall/interventricular septum
    • may be pedunculated
    • calcification and cystic degeneration centrally
    • tendency for slow growth
    Cx: fetal hydrops secondary to obstruction, pericardial effusion, fetal arrhythmia, fetal death
  • Hemangioma (arise from R atrium, pericardial effusion, skin hemangiomas), lymphangioma, neurofibroma, myxoma, mesothelioma:
    • mass-occupying lesion impinging upon cardiac cavities
Cardiac Tumor by Location
  • ENDOCARDIAL/INTRACAVITARY
    • Myxoma
    • Thrombus
    • Myofibroblastic sarcomas (MFH, leiomyosarcoma, fibrosarcoma, myxosarcoma)
  • VALVULAR
    • Papillary fibroelastoma
    • Vegetations
    • Thrombus
    • Myxoma
  • MYOCARDIAL/INTRAMURAL
    • Rhabdomyoma: multifocal, high signal on T2WI
    • Fibroma: always solitary, calcifications, cystic degeneration, low signal on T2WI
  • ENDO-/MYO-/EPICARDIAL
    • Lipoma
    • Sarcoma
    • Primary cardiac lymphoma: right heart, multifocal, extension into pericardium
Cardiac calcifications
Detected by:
  • fluoroscopy (at low-beam energies ≤75 kVp is 57% sensitive)
  • < digital subtraction fluoroscopy
  • < conventional CT < ultrafast CT (96% sensitive)
  • @ Coronary arteries (see below)
  • @ Cardiac valves
    • Valvar calcification means stenosis — its amount is proportionate to degree and duration of stenosis!
  • Aortic valve
    • Valve calcium best seen on lateral view!
    • good correlation between amount of calcium and degree of stenosis:
      • ⇒ heavy calcification = significant stenosis
      • ⇒ no calcification = aortic stenosis unlikely
    Cause: congenital bicuspid valve (70–85%) > atherosclerotic degeneration > rheumatic aortic stenosis (rare), syphilis, ankylosing spondylitis
    Location: above + anterior to a line connecting carina + anterior costophrenic angle (lateral view)
    • Congenital bicuspid aortic valvular stenosis
      • • calcium first detected at an average age of 28 years
      • In patients <30 years aortic valve calcifications are mostly due to a bicuspid aortic valve!
      • usually extensive cluster of heavy dense calcific deposits:
        • nearly circular calcification with interior linear bar (DIAGNOSTIC)
      • poststenotic dilatation of ascending aorta
    • Isolated rheumatic aortic stenosis
      • • calcium first detected at an average age of 47 years
      • In patients 30–60 years of age aortic valve calcification suggests rheumatic valve disease!
      • cluster of heavy dense calcific deposits without bicuspid contour
    • Degenerative aortic stenosis
      • • calcium first detected at an average age of 54 years
      • In patients >65 years aortic valve calcification in 90% due to atherosclerosis!
      • curvilinear shape of calcium outlining tricuspid leaflets
      • diffuse dilatation + tortuosity of aorta (NO poststenotic dilatation)
  • Mitral valve (MV)
    Cause: rheumatic heart disease (virtually always), infected endocarditis, tumor attached to mitral valve, mitral valve prolapse
    Location: inferior to a line connecting carina + anterior costophrenic angle (on lateral view)
    • • calcium first detected in early thirties when patients become overtly symptomatic
    • a severely calcified MV is usually stenotic, but MV stenosis frequently exists without calcium
    • delicate calcification similar to coronary arteries (DDx: calcium in RCA/LCX)
    • superior-to-inferior motion
  • Pulmonic valve
    Cause: tetralogy of Fallot, pulmonary stenosis, atrial septal defect
    • calcific pattern similar to calcified mitral valve
  • Tricuspid valve (extremely rare)
    Cause: rheumatic heart disease, septal defect, tricuspid valve defect, infective endocarditis
  • @ Annulus
    • = valve rings serve as fibrous skeleton of the heart for attachment of myocardial fibers + cardiac valves
    • Mitral annulus
      Cause: degenerative (physiologic in elderly)
      Age: >65 years; M:F = 1:4
      May be associated with: mitral valve prolapse
      P.596

      • Commonly associated with:
        • aortic valve calcium (= aortic stenosis), hypertension, hypertrophic cardiomyopathy
      • dense bandlike calcification starting at posterior aspect + progressing laterally frequently forming a “reversed C”/“O”/“U”/“J”
      Cx: mitral insufficiency (due to impaired anterior mitral leaflet), atrial fibrillation, heart block (due to infiltration into posterior wall conduction pathway)
      Prognosis: doubles risk of stroke
    • Aortic annulus
      • usually in combination with degenerative aortic valve calcification
    • Tricuspid annulus
      Associated with: long-standing RV hypertension
      Location: right AV groove
      • bandlike C-shaped configuration
  • @ Pericardium
    Cause: idiopathic pericarditis, rheumatic fever (5%), tuberculosis, viruses, uremia, trauma, radiotherapy to mediastinum
    Location: calcification over less pulsatile right-sided chambers along diaphragmatic surface, atrioventricular grooves, pulmonary trunk
    • 50% of patients with constrictive pericarditis show pericardial calcifications!
    • clumpy amorphous calcium deposits, frequently in atrioventricular groove
    • diffuse eggshell calcification sparing LA (not covered by pericardium)
    Cx: constrictive pericarditis
  • @ Myocardium
    Cause: infarction, aneurysm, rheumatic fever, myocarditis
    Frequency: in 8% post myocardial infarction; M > F
    Location: apex/anterolateral wall of LV (coincides with LAD vascular distribution + typical location of LV aneurysms)
    • thin curvilinear contour outlining the aneurysm
    • shaggy laminated calcification suggests calcification of associated mural thrombus
    • coarse amorphous calcifications caused by trauma, cardioversion, infection, endocardial fibrosis
  • @ Interventricular septum
    Location: triangular fibrous area between mitral + tricuspid annuli (= trigona fibrosa) representing the basal segment of interventricular septum, closely related to bundle of His
    Always associated with:
    • heavy calcification of mitral annulus/aortic valve
    Cx: heart block
  • @ Left atrial wall
    Cause: rheumatic endocarditis
    • diffuse sheetlike form
      • • patient usually in CHF + atrial fibrillation
      • curvilinear calcification sparing interatrial septum + posterolateral wall on right side
      • shaggy nodular deposits in atrial appendage
      Cx: mural thrombus formation + emboli
      • LA wall calcification indicates atrial fibrillation!
    • localized form
      • nodular calcific scar in posterior wall (= McCallum patch) due to injury from a forceful jet in mitral valve insufficiency
  • @ Cardiac tumor
    • atrial myxoma (in 5–10% calcified), rhabdomyoma, fibroma, angioma, osteosarcoma, osteoclastoma
  • @ Endocardium
    Cause: cardiac aneurysm, thrombus, endocardial fibroelastosis
  • @ Pulmonary artery
    Cause: severe precapillary pulmonary arterial hypertension, syphilis
  • @ Ductus arteriosus
    (a) in adults: indicates patency of ductus with associated long-standing precapillary pulmonary hypertension
    (b) in children: ductus likely closed
    • calcium deposition in ligament of Botallo
Coronary Artery Calcification
  • The amount of coronary calcification correlates with the extent of atherosclerosis!
  • The absence of calcification implies the absence of angiographically significant coronary vessel stenosis!
Cause: (1) arteriosclerosis of intima
(2) Mönckeberg medial sclerosis (exceedingly rare)
Histo: calcified subintimal plaques
Pathophysiology:
  • injury to endothelium allows circulating histiocytes to lodge in vessel wall where they are transformed into macrophages; these accumulate lipids (“fatty streaks” beneath surface endothelium); lipids calcify; the thin fibrous cap overlying lipid deposits may rupture allowing circulating blood to mount a thrombogenic reaction resulting in narrowing of lumen
  • Calcium is deposited as calcium hydroxyapatite in hemorrhagic areas within atheromatous plaques!
Location: “coronary artery calcification triangle” = triangular area along mid left heart border, spine, and shoulder of LV containing left main coronary artery, proximal portions of LAD + LCX calcifications
Frequency (autoptic): LAD (93%), LCX (77%), main LCA (70%), RCA (69%)
CXR (detection rate up to 42%):
  • Indicating more severe coronary artery disease
  • parallel calcified lines (lateral view)
Fluoroscopy: (promoted as inexpensive screening test)
  • asymptomatic population
    • calcifications in 34% in asymptomatic male individuals
    • in 35% of patients with calcifications exercise test will be positive (without calcifications only in 4% positive)
    • P.597

    • calcifications indicate >50% stenosis with 72-76% sensitivity, 78% specificity); frequency of coronary artery calcifications with normal angiogram increases with age; predictive values in population <50 years as good as exercise stress test
  • symptomatic population
    • — in 54% of symptomatic patients with ischemic heart disease
    • In symptomatic patients 94% specificity for obstructive disease (>75% stenosis) of at least one of the three major vessels!
CT:
(a) electron beam: threshold of +130 HU
(b) spiral CT: threshold of +90 HU
Clinical outcome:
  • for coronary calcifications detected at fluoroscopy: 5.4% event risk at 1 year (vs. 2.1% without calcification)
  • for electron beam CT a calcification score of ≥100 is highly predictive to identify patients with events
Prognosis: 58% 5-year survival rate with and 87% without calcifications
Pericardium
Pericardial Effusion
  • = pericardial fluid >50 mL
  • • dyspnea, fatigue
  • • symptoms of cardiac tamponade (50%)
Etiology:
  • SEROUS FLUID = transudate congestive heart failure, hypoalbuminemia, irradiation, Dressler syndrome, postpericardiotomy syndrome, myxedema
  • BLOOD = hemopericardium
    • iatrogenic: cardiac surgery/catheterization, anticoagulants, chemotherapy, radiation
    • trauma: penetrating/nonpenetrating
    • acute myocardial infarction/rupture
    • rupture of ascending aorta/pulmonary trunk
    • coagulopathy
    • neoplasm: mesothelioma, sarcoma, teratoma, fibroma, angioma, metastasis (lung, breast, lymphoma, leukemia, melanoma)
  • LYMPH
    • neoplasm, congenital, cardiothoracic surgery, obstruction of hilum/SVC
  • FIBRIN = exudate
    • infection: viral, pyogenic, tuberculous
    • uremia: 18% in acute uremia; 51% in chronic uremia; dialysis patient
    • collagen vascular disease: SLE, rheumatoid arthritis, acute rheumatic fever
    • hypersensitivity
mnemonic: CUM TAPPIT RV
  • Collagen vascular disease
  • Uremia
  • Metastasis
  • Trauma
  • Acute myocardial infarction
  • Purulent infection
  • Post MI syndrome
  • Idiopathic
  • Tuberculosis
  • Rheumatoid arthritis
  • Virus
  • CXR:
    • normal with fluid <250 mL/in acute pericarditis
    • “water bottle configuration” = symmetrically enlarged cardiac silhouette
    • loss of retrosternal clear space
    • “fat-pad sign” = separation of retrosternal from epicardial fat line >2 mm (15%) by water density
    • rapidly appearing cardiomegaly + normal pulmonary vascularity
    • “differential density sign” = increase in lucency at heart margin secondary to slight difference in contrast between pericardial fluid + heart muscle
    • diminished cardiac pulsations
  • CT:
    • fluid of water/higher (blood, infection) density
  • MR:
    • transudative fluid is hypointense on T1WI + hyperintense on T2WI
    • proteinaceous/hemorrhagic fluid is hyperintense compared with transudate on T1WI
    • inflammatory pericarditis of uremia/TB may demonstrate pericardial thickening
  • ECHO:
    • separation of epi- and pericardial echoes extending into diastole (rarely behind LA)
    • volume estimates by M-mode:
      • separation only posteriorly = <300 mL
      • separation throughout cardiac cycle = 300–500 mL
      • plus anterior separation = >1000 mL
Pneumopericardium
Etiology: shearing mechanism of injury of the heart during blunt trauma
Path: tear in fibrous pericardium, usually along the course of the phrenic nerve, allows pneumomediastinal air to enter
  • thick shaggy soft-tissue density of fibrous pericardium separated by air from cardiac density
  • air limited to distribution of pericardial reflection
Pericardial Tumor
  • Pericardial teratoma: benign tumor of infants + children
  • Pericardial mesothelioma: malignant tumor of adulthood
DDx: pericardial invasion (sarcoma, lymphoma)
Vena Cava
Vena cava anomalies
Circumaortic Left Renal Vein
Prevalence: 1.5–8.7%
Etiology: persistence of anterior intersubcardinal + posterior intersupracardinal anastomosis
  • venous collar encircling aorta
  • superior left renal vein crosses aorta anteriorly
  • P.598

  • inferior left renal vein receives left gonadal vein + crosses aorta posteriorly 1–2 cm below the superior left renal vein
Significance: preoperative plan for nephrectomy
Duplicated IVC
  • = DOUBLE IVC
Prevalence: 0.2–3%
Etiology: persistence of both supracardinal veins
√ small/equal-sized left IVC formed by left iliac vein
√ crossover to right IVC via left renal vein/or more inferiorly
√ crossover usually anterior/rarely posterior to aorta
Significance: recurrent pulmonary embolism after IVC filter placement
DDx: left gonadal v./ a., inferior mesenteric v.
DOUBLE IVC WITH RETROAORTIC RIGHT RENAL VEIN AND AZYGOS CONTINUATION OF IVC
Etiology: persistence of left supracardinal v. and dorsal limb of renal collar + regression of ventral limb + failure of formation of right subcardinal-hepatic anastomosis
DOUBLE IVC WITH RETROAORTIC RIGHT RENAL VEIN AND HEMIAZYGOS CONTINUATION OF IVC
Etiology: persistence of left lumbar + thoracic supracardinal v. + left suprasubcardinal anastomosis + failure of formation of right subcardinal-hepatic anastomosis
  • right IVC and right renal vein join the left IVC and continue cephalad as the hemiazygos vein
  • hemiazygos vein follows alternative pathways:
    • crosses posterior to aorta at T8-9 and joins the rudimentary azygos vein
    • continues cephalad + joins coronary vein via persistent left SVC
    • accessory hemiazygos continuation to left brachiocephalic vein
  • hepatic segment of IVC drains into right atrium
Interrupted IVC with Azygos/Hemiazygos Continuation
see AZYGOS CONTINUATION
Left IVC
  • = TRANSPOSITION OF IVC = SOLITARY LEFT IVC
Prevalence: 0.2–0.5%
Etiology: persistence of left + regression of right supracardinal vein
  • left IVC usually joins left renal vein
  • crossover as left renal vein usually anterior/rarely posterior to aorta
DDx: left-sided paraaortic adenopathy
Significance: difficult transjugular access to infrarenal IVC filter placement
Persistent Left SVC
  • = BILATERAL SVCs
Prevalence: 0.3% of general population;
4.3–11% of patients with CHD
Etiology: failure of regression of left anterior + common cardinal veins + left sinus horn
May be associated with: ASD, azygos continuation of IVC
Course: lateral to aortic arch, anterior to left hilum
  • left SVC drains into enlarged coronary sinus (common)
  • left SVC drains into LA (rare) creating a R-to-L shunt (increased prevalence of CHD)
  • hemiazygos arch formed by left superior intercostal vein + persistent left SVC (20%)
  • absent/small left brachiocephalic vein (65%)
  • absence of right SVC (10–18%)
  • anastomosis between right + left anterior cardinal veins (in 35%)
Retroaortic Left Renal Vein
Prevalence: 1.8–2.1%
Etiology: persistence of posterior intersupracardinal anastomosis + regression of anterior intersubcardinal anastomosis
  • crossover usually below/occasionally at level of right renal vein
IVC Obstruction
  • INTRINSIC OBSTRUCTION
    • neoplastic (most frequent)
      • Renal cell carcinoma (in 10%), Wilms tumor
      • Adrenal carcinoma, pheochromocytoma
      • Pancreatic carcinoma, hepatic adenocarcinoma
      • Metastatic disease to retroperitoneal lymph nodes (carcinoma of ovary, cervix, prostate)
    • nonneoplastic
      • Idiopathic
      • Proximally extending thrombus from femoroiliac veins
      • Systemic disorders: coagulopathy, Budd-Chiari syndrome, dehydration, infection (pelvic inflammatory disease), sepsis, CHF
      • Postoperative/traumatic phlebitis, ligation, plication, clip, cava filter, severe exertion
  • INTRINSIC CAVAL DISEASE
    • neoplastic
      • Leiomyoma, leiomyosarcoma, endothelioma
    • nonneoplastic
      • Congenital membrane
  • EXTRINSIC COMPRESSION
    • neoplastic
      • Retroperitoneal lymphadenopathy (adults) due to metastatic disease, lymphoma, granulomatous disease (TB)
      • Renal + adrenal tumors (children)
      • Hepatic masses
      • Pancreatic tumor
      • Tumor-induced desmoplastic reaction (eg, metastatic carcinoid)
    • nonneoplastic
      • Hepatomegaly
      • Tortuous aorta/aortic aneurysm
      • Retroperitoneal hematoma
      • Massive ascites
      • Retroperitoneal fibrosis
  • P.599

  • FUNCTIONAL OBSTRUCTION
    • Pregnant uterus
    • Valsalva maneuver
    • Straining/crying (in children)
    • Supine position with large abdominal mass
  • COLLATERAL PATHWAYS
    • Deep pathway: ascending lumbar veins to azygos vein (right) + hemiazygos vein (left) + intravertebral, paraspinal, extravertebral plexus (Batson plexus)
    • Intermediate pathway: via periureteric plexus + left gonadal vein to renal vein
    • Superficial pathway: external iliac vein to inferior epigastric vein + superior epigastric vein + internal mammary vein into subclavian vein
    • Portal pathway: retrograde flow through internal iliac vein + hemorrhoidal plexus into inferior mesenteric vein + splenic vein into portal vein
image
Noninfectious Vasculitides
Vasculitis
= inflammation and necrosis of vessel wall
  • LARGE-VESSEL VASCULITIS
    • Giant cell (temporal) arteritis
    • Takayasu disease
  • MEDIUM-SIZED–VESSEL VASCULITIS
    • Polyarteritis nodosa
    • Kawasaki disease
    • Drug-induced vasculitis:
      • –methamphetamine
      • –cocaine: neurovascular, cardiovascular complications, aortic dissection, venous thrombosis, mesenteric artery thrombosis, renal infarction
  • SMALL-VESSEL VASCULITIS
    • ANCA-associated small-vessel vasculitis
      • (= antineutrophil cytoplasmic autoantibodies)
      • Wegener granulomatosis
      • Churg-Strauss syndrome
      • Microscopic polyangitis
    • immune-complex small-vessel vasculitis
      • Henoch-Schönlein purpura
      • Essential cryoglobulinemic vasculitis
      • Cutaneous leukocytoclastic angitis
      others: lupus, rheumatoid, Sjögren, Behçet, Goodpasture, serum sickness, drug-induced, hypocomplementemic urticaria
    • inflammatory bowel disease vasculitis
Multiple Aneurysms
  • Polyarteritis nodosa
  • Rheumatoid vasculitis
  • Systemic lupus erythematosus
  • Churg-Strauss syndrome
Cardiac surgery
Surgical Procedures
  • AORTICOPULMONARY WINDOW SHUNT
    • = side-to-side anastomosis between ascending aorta and left pulmonary artery (reversible procedure)
    • Tetralogy of Fallot
  • BLALOCK-HANLON PROCEDURE
    • = surgical creation of ASD
    • Complete transposition
  • BLALOCK-TAUSSIG SHUNT
    • = end-to-side anastomosis of subclavian artery to pulmonary artery, performed ipsilateral to innominate artery/opposite to aortic arch
    • Modified Blalock-Taussig shunt uses synthetic graft material such as polytetrafluoroethylene (Gore-Tex®) in an end-to-side anastomosis between subclavian artery + ipsilateral branch of pulmonary artery
    • Tetralogy of Fallot, tricuspid atresia with pulmonic stenosis
  • FONTAN PROCEDURE
    • external conduit from right atrium to pulmonary trunk (= venous return enters pulmonary artery directly)
    • closure of ASD: floor constructed from flap of atrial wall and roof from piece of prosthetic material
    • Tricuspid atresia
  • GLENN SHUNT
    • = end-to-side shunt between distal end of right pulmonary artery and SVC; reserved for patients with cardiac defects in which total correction is not anticipated
    • Tricuspid atresia
  • P.600

  • NORWOOD PROCEDURE
    • construction of “neoaorta” from aortic arch + descending aorta + main pulmonary artery supplying coronary and systemic circulation
    • communication between RV as systemic ventricle and systemic circulation
    • shunt between innominate artery + main pulmonary artery to control pulmonary arterial blood flow
    • excision of distal ductus arteriosus + atrial septum to prevent pulmonary venous hypertension
    • Hypoplastic left heart syndrome
  • POTT SHUNT
    • = side-to-side anastomosis between descending aorta + left pulmonary artery
    • Tetralogy of Fallot
  • MUSTARD PROCEDURE
    • removal of atrial septum
    • pericardial baffle placed into common atrium such that systemic venous blood is rerouted into left ventricle and pulmonary venous return into right ventricle and aorta
    • Complete transposition
  • RASHKIND PROCEDURE
    • = balloon atrial septostomy
    • Complete transposition
  • RASTELLI PROCEDURE
    • external conduit (Dacro) with porcine valve connecting RV to pulmonary trunk
    • Transposition
  • WATERSTON-COOLEY SHUNT
    • = side-to-side anastomosis between ascending aorta and right pulmonary artery;
      • extrapericardial (WATERSTON)
      • intrapericardial (COOLEY)
    • Tetralogy of Fallot
Postoperative Thoracic Deformity
  • ON RIGHT SIDE
    • Systemic-PA shunt: Blalock-Taussig shunt, Waterston-Cooley shunt, Glenn shunt, central conduit shunt
    • Atrial septectomy: Blalock-Hanlon procedure
    • VSD repair: through RA
    • Mitral valve commissurotomy
  • ON LEFT SIDE
    • PDA
    • Coarctation
    • PA banding
    • Mitral valve commissurotomy
    • Systemic-PA shunt: Blalock-Taussig shunt, Pott shunt
Heart Valve Prosthesis
  • Starr-Edwards
    • caged ball
    • Predictable performance from large long-term experience
  • Bjørk-Shiley/Lillehei-Kaster/St. Jude
    • tilting disk
    • Excellent hemodynamics, very low profile, durable
  • Hancock/Carpentier-Edwards (= porcine xenograft) Ionescu-Shiley (= bovine xenograft)
    • Low incidence of thromboembolism, no hemolysis, central flow, inaudible
Pulsus Alternans
= alternating arterial pulse height with regular cardiac rhythm
  • Intrinsic myocardial abnormality
    • severe left ventricular dysfunction (CHF, aortic valvular disease, hypothermia, hypocalcemia, hyperbaric stress, ischemia)
  • Alternating end-diastolic volumes abnormalities in venous filling + return (obstructed venous return, IVC balloon
Mustard Procedure (lateral view into opened right atrium)
P.601

Cardiovascular Anatomy
image
Normal Blood Pressures
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Development of Major Blood Vessels
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Right Ventricle Viewed from Front
P.602

Heart size
Cardiothoracic Ratio
  • = widest transverse cardiac diameter ± widest inside thoracic diameter
< 0.5 = normal in >1 month old (45% sensitive, 85% specific, 59% accurate)
< 0.6 = normal in <1 month old
Purpose: measurement of LV dilatation
Dependent on:
  • lung volume: CT ratio enlarges in expiration
  • patient position: CT ratio increases on supine film
Pitfalls:
  • no change unless LV volume increases by >2/3
  • no change in moderate enlargement of LA / RV
Heart valve positions
PA CXR:
  • reference line = oblique line drawn from distal left mainstem bronchus to right cardiophrenic angle
  • aortic valve resides in profile superior to this line overlying the thoracic spine
  • pulmonic valve just inferior to left mainstem bronchus
  • mitral valve resides inferior to this line centrally located within cardiac silhouette
  • tricuspid valve inferior to this line more basilar and midline
LAT CXR:
  • reference line = oblique line drawn from carina / right pulmonary artery shadow to anterior cardiophrenic sulcus
  • aortic valve resides superior to this line
  • pulmonic valve anterior + superior to aortic valve
  • mitral valve resides inferoposteriorly to this line
  • tricuspid valve inferior to this line anteriorly
Atria
Right Atrium
  • Eustachian valve = valve of IVC
    • –located at junction of IVC + RA
    • –directs blood from IVC to foramen ovale in fetus
    • thin linear structure, not routinely imaged
  • Crista terminalis
    • –line of fusion between anterior trabeculated portion + smooth-walled posterior portion of RA
    • vertically oriented smooth muscular ridge
Interatrial Septum
  • thin septum, difficult to image
  • may contain small amount of fat sparing fossa ovalis
DDx: Lipomatous hypertrophy of interatrial septum
√ characteristic dumbbell shape due to sparing of fossa ovalis
√ abnormal amount of fat in older / obese adults
Left Atrium
  • ridge of smooth muscle (± bulbous tip) at junction of left atrial appendage and entrance of left superior pulmonary vein
Aortic arch branching patterns
  • “Standard” branching pattern (65–75%)
    • brachiocephalic trunk, left CCA, left subclavian artery
  • Common origin of brachiocephalic trunk + left CCA (13%)
  • Bovine aortic arch (9%)
    • = origin of left CCA from brachiocephalic trunk
  • Vertebral artery (usually left) arising from aortic arch (3%)
  • Left and right brachiocephalic trunks (1%)
  • Aberrant right subclavian artery as the last branch of the aortic arch (<1%)
image
Heart Valve Positions
P.603

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Parasternal Long-Axis View
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Parasternal Long-and Short-Axis
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Diagram of the Relationship of the four Cardiac Valves in Cross Section
Echocardiogram of Aortic Root
Aortic root dimension measured at enddiastole at R-wave of ECG 2.1-4.3 cm
  increased in: aneurysm of aorta, aortic insufficiency
Aortic cusp separation   1.7-2.5 cm
  decreased in: aortic stenosis, low stroke volume
  increased in: aortic insufficiency
Left atrial diameter measured at moment of mitral valve opening 2.3-4.4 cm
Eccentricity index of aortic valve cusps ratio of anterior to posterior dimension(rarely used) <1.3
Ratio of LA-to-aorticroot dimension   0.87-1.11
Echocardiogram of Right and left ventricle
Rv end-diastolic dimension at R-wave of ECG 0.7-2.3 cm
  increased in: RV volume overload
Septal thickness end-diastolic thickness at R-wave of ECG 0.9
± 0.06 cm
  decreased in: CAD
  increased in: asymmetric septal hypertrophy, IHSS
LV end-diastolic dimension at R-wave of ECG 4.6
± 0.54 cm
LVPW thickness end-diastolic thickness at peak of R-wave 0.94 ± 0.09 cm
  increased in: LV hypertrophy
LV end-systolic dimension   2.9
± 0.5 cm
IVS:LVPW thickness   <1.3
Fractional shortening (EDD - ESD)/EDD × 100
  for LV   25-42%
  for IVS   28-62%
  for LVPW   36-70%
P.604

Cervical Aortic Arch
Associated with: right aortic arch (in 2/3)
  • • pulsatile neck mass
  • • upper airway obstruction
  • • dysphagia
  • mediastinal widening
  • absence of normal aortic knob
  • aortic arch near lung apex
  • tracheal displacement to opposite side + anteriorly
  • apparent cutoff of tracheal air column (secondary to crossing of descending aorta to side opposite of arch)
DDx: carotid aneurysm
Aortic isthmus variants
Aortic Isthmus
  • = narrowing of the aorta in newborn between left subclavian artery and ductus arteriosus
Age: up to 2 months of age
Prognosis: aortic isthmus disappears due to cessation of flow through ductus arteriosus + increased flow through narrowed region
Aortic Spindle (16%)
  • = congenital narrowing of the aorta at the ligamentum arteriosum with distal fusiform dilatation
Ductus Diverticulum
  • = localized bulge along anteromedial aspect of aortic isthmus
Origin: remnant of enlarged mouth of ductus arteriosus / result of traction from ligamentum arteriosum
Frequency: in 33% of infants, in 9% of adults
  • focal bulge with smooth uninterrupted margins:
    • gently sloping symmetric shoulders (classic ductus diverticulum)
    • shorter steeper slope superiorly + more gentle slope inferiorly (atypical ductus diverticulum)
DDx: posttraumatic false aneurysm
Prominent Bronchial-intercostal Trunk
 
Coronary arteries
Anatomy of Right Coronary Artery (RCA)
  • arises from anterior right coronary sinus;
  • travels within right atrioventricular sulcus;
  • rounds the acute margin of the heart
  • Conus artery (CB)
    • = 1st branch from RCA (in 50% directly from aorta) to supply RVOT
  • Sinoatrial node artery (SANA)
    • = 2nd branch from RCA (in >50%)
  • Acute / RV marginal branches (M1, M2, etc)
    • have an anterior course
  • Posterior descending artery (PDA)
    • originates from RCA near crux or from a distal acute marginal branch;
    • supplies posterior third of ventricular septum + diaphragmatic segment of LV;
    • supplies blood to posteromedial papillary muscle
  • Atrioventricular node artery (AVNA)
    • = small branch to AV node
  • Posterolateral segment arteries (PLSA)
    • supplies posterolateral wall of LV
image
Normal Aortic Isthmus Variants in 45° LAO Projection
Anatomy of Left Coronary Artery (LCA)
  • arises from left posterior coronary sinus
  • Left main coronary artery (LM)
    • 0.5–2.0 cm short stem before bi- / trifurcation
  • Left anterior descending (LAD)
    • travels within anterior interventricular groove, gives blood supply to anterolateral papillary muscle
    • (a) Diagonal branches (D1, D2, etc)
      • arise from LAD and course over anterolateral wall of LV
      mnemonic: Diagonals from LAD
      occasionally trifurcation into LAD + LCx
      + ramus intermedius (in a course similar to D1)
    • (b) Septal branches (S)
      • for anterior interventricular septum
  • Left circumflex artery (LCx)
    • travels within left atrioventricular sulcus;
    • terminates at obtuse margin of heart
    • (a) Obtuse marginal branches (OM1, OM2, etc)
      • for lateral wall of LV
    • (b) Left atrial circumflex artery (LACX)
      • for atrium
Coronary Artery Territory
septum = LAD
anterior wall = LAD
lateral wall = LCx
posterior wall = RCA
inferior / diaphragmatic wall = RCA
apex + inferolateral wall = watershed areas
Coronary Artery Dominance
  • determined by the artery that crosses the crux and serves as origin of the posterior descending artery (PD), which supplies the inferior portion of LV:
  • — from RCA in 85% (= right dominance)
  • — from LCx in 8% (= left dominance)
  • — RCA + LCA = codominance / balanced supply (7%)
Coronary Arteriography
Contrast agents:
  • Monomeric ionic contrast material:
    P.605

    • negative inotropic = depression of myocardial contractility due to hyperosmolality of sodium + decrease in total calcium
    • peripheral vasodilatation
  • Meglumine diatrizoate (contains small quantities of sodium citrate + EDTA)
  • Nonionic contrast material = slight increase in LV contractility
Dose: 3–10 mL
Mortality: 0.05%
Risk factors associated with death:
  • Multiple ventricular premature contractions
  • Congestive heart failure
  • Systemic hypertension
  • Severe triple-vessel coronary artery disease (highest risk)
  • LV ejection fraction <30%
  • Left main coronary artery stenosis
Clues for projection:
45–70° LAO:
  • ribs slanting to left side of image
  • catheter in descending aorta on right side of image
15–30° RAO:
  • ribs slanting to right side of image
  • catheter in descending aorta on left side of image
Technique:
  • 20–30° of cranial / caudal angulation variably used
  • Catheter in left coronary orifice:
    • (a) LAO + caudocranial angulation:
      • proximal 1/3 of LAD + origin of first diagonal branch
    • (b) LAO + craniocaudal angulation = “spider view”:
      • LCA, proximal LCx, first marginal / diagonal branches
    • (c) RAO + craniocaudal angulation:
      • proximal third of LCx + origin of its branches
    • (d) RAO + caudocranial angulation:
      • separation of LAD from diagonal branches
  • Catheter in right coronary artery orifice: LAO plusmn; RAO
False-negative interpretation:
  • eccentric lesion in 75%
  • foreshortening of vessel
  • overlap of other vessels remedied by angulated projections: improved diagnosis (50%), upgrade to more significant stenosis (30%), lesion unmasked (20%)
image
AP View of Heart and Coronary Arteries
image
Anomalies of the Coronary Arteries
Coronary Artery Collaterals
  • intracoronary COLLATERALS
    • = filling of a distal portion of an occluded vessel from the proximal portion
    • tortuous course outside the normal path
  • intercoronary COLLATERALS
    • = between different coronary arteries / between branches of the same artery
    P.606

    image
    Right and Left Coronary Artery Angiograms
    P.607

    image
    Coronary Artery Angiograms of Varying Dominance
    Location: on epicardial surface, in atrial / ventricular septum, in myocardium
    • Proximal RCA to distal RCA
      • by way of acute marginal branches
      • from sinoatrial node artery (SANA) to atrioventricular node artery (AVNA) = Kugel collateral
    • RCA to LAD
      • between PDA and LAD through ventricular septum / around apex
      • conus artery (1st branch of RCA) to proximal part of LAD
      • acute marginals of RCA to right ventricular branches of LAD
    • Distal RCA to distal LCx
      • posterolateral segment artery of RCA to distal LCx (in AV groove)
      • AVNA of RCA to LCx (through atrial wall)
      • posterolateral branch of RCA to obtuse marginal branches of LCx (over left posterolateral ventricular wall)
    • Proximal LAD to distal LAD
      • proximal diagonal to distal diagonal artery of LAD
      • proximal diagonal to LAD directly
    • LAD to obtuse marginal of LCx
Coronary Artery Anomalies
Incidence: 0.3–1%; in 25% responsible for nontraumatic sudden death in young adults
Anomaly of Origin
  • High takeoff (6%)
    • = origin of RCA / LCA above the junctional zone between sinus + tubular part
  • Multiple ostia
    • RCA + conus branch arise separately
    • LAD + LCx arise separately without LCA (0.41%)
  • Single coronary artery (0.0024–0.044%)
  • Anomalous origin from the pulmonary artery
    • Bland-White-Garland syndrome
      • = LCA arises from PA + RCA arises from aorta
      Prevalence: 1:300,000 live births
      • collateral circulation between RCA + LCA
      • coronary steal into PA
      Prognosis: death in 1st year of life in 90%
  • Origin of coronary artery from opposite sinus / noncoronary sinus
    • RCA arising from left coronary sinus (0.03–0.17%)
    • LCA arising from right coronary sinus (0.09–0.11%)
    • LCx / LAD arising from right coroanry sinus (0.32–0.67%)
    • LCA / RCA arising from noncoronary sinus
    may take the following course:
    • interarterial (between aorta + pulmonary trunk) with a high risk of sudden cardiac death
    • retroaortic
    • prepulmonic
    • septal (subpulmonic / beneath RVOT
P.608

image
Pericardial Sinuses & Recesses
Anomaly of Coronary Artery Course
  • Myocardial bridging
    • = band of myocardial muscle overlying a segment of a coronary artery
  • Duplication of arteries, eg., LAD
Anomaly of Termination
  • Coronary artery fistula (0.1–0.2%)
    • = communication between coronary artery (RCA in 60%, LCA in 40%, both in <5%) and cardiac chamber (RV in 45%, RA in 25%) / pulmonary artery (in 15%) / coronary sinus / SVC
    • dilated tortuous coronary artery
    • single / multiple communications / network of fine vessels
    Cx: myocardial ischemia
  • Coronary arcade
    • = angiographically demonstrable communication between RCA and LCA in the absence of a coronary artery stenosis
    • prominent straight connection near crux
    DDx: tortuous collateral vessel
  • Extracardiac termination
    Cause: atherosclerotic CAD
    Receiver: bronchial, internal mammary, pericardial, anterior mediastinal, superior / inferior phrenic, intercostal arteries
Pericardium
Pericardial thickness: 1-3 mm
  • FIBROUS PERICARDIUM
    • = outer fibrous layer
  • SEROUS PERICARDIUM
    • = inner serous sac forming the pericardial cavity
    • contains 20–50 mL of serous fluid
    • (a) inner visceral layer = epicardium
      • intimately connected to heart + epicardial fat
    • (b) outer parietal layer
      • lines fibrous pericardium
Pericardial sinuses and Recesses
  • = extensions of pericardial cavity
  • Recesses of pericardial cavity proper
    • Postcaval recess (23%*)
      • behind and right lateral to SVC
    • Right pulmonic vein recess (29%*)
      • behind and right lateral to SVC
    • Left pulmonic vein recess (60%*)
      • behind and right lateral to SVC
  • Transverse sinus
    • posterior to ascending aorta and pulmonary trunk + above left atrium (95%*)
    • 1. Superior aortic recess
      • along ascending aorta; may be divided into anterior, posterior, right lateral portion
      DDx: aortic dissection on NECT
    • 2. Left pulmonic recess
      • below left pulmonary artery + posterolateral to proximal right pulmonary artery
    • 3. Right pulmonic recess
      • below right pulmonary artery + above left atrium
    • 4. Inferior aortic recess
      • between ascending aorta + inferior SVC / right atrium
      • extending down to level of aortic valve
  • Oblique sinus (89%*)
    • behind left atrium + anterior to esophagus
    • separated from transverse sinus by double reflection of pericardium (and fat) between right + left superior pulmonic veins
    • 1. Posterior pericardial recess (67%*)
      • behind distal right pulmonary artery + medial to bronchus intermedius
        P.609

        DDx: lymph nodes, esophageal / thymic process, vascular abnormality, pericardial cyst / tumor
      • * = percentages give depiction on HRCT
image
Development of the Major-Venous System
Embryogenesis Of Vena Cava
Time of development: 6-8th week of embryonic life
Origin:
  • Vitelline (omphalomesenteric) venous system:
    • blood from yolk sac to sinus venosus
  • Umbilical venous system
    • blood from chorionic villi to sinus venosus via ductus venosus
  • Intraembryonic cardinal venous system
    • continuous appearance + regression of 3 paired embryonic veins
    • (1) Cardinal veins
      • join to form common cardinal vein, which enters left + right sinus horns
      • (a) anterior cardinal veins
        • drain the cranial region
      • (b) posterior cardinal veins
        • drain body of embryo + mesonephros + anterior extremities
      Location: dorsolateral part of urogenital fold
    • (2) Subcardinal veins
      • drain urogenital system of metanephros + suprarenal glands
        Location: ventromedial to posterior cardinal veins + ventrolateral to aorta
      • — Intersubcardinal anastomoses form anterior to aorta below superior mesenteric artery and connect left + right subcardinal veins
    • (3) Supracardinal veins
      • drain body wall via intercostal veins
      Location: dorsomedial to posterior cardinal vein + dorsolateral to aorta
      • CRANIAL
        • azygos vein on the right
          • drains 4–11 right intercostal veins
        • portion of superior intercostal vein
          • drains 2–3 left intercostal veins
        • accessory hemiazygos
          • drains 4–7 left intercostal veins
        • hemiazygos vein
          • drains left 8–11 intercostal veins
      • — CAUDAL: lumbar veins
P.610

Inferior Vena Cava
  • Hepatic = posthepatic segment
    Origin: terminal part of right vitelline vein
  • Suprarenal segment
    Origin: subcardinal-hepatic anastomosis
  • Renal segment
    Origin: part of right subcardinal vein + supracardinal-subcardinal anastomoses
  • Infrarenal segment
    Origin: right supracardinal / sacrocardinal vein
Venous system of lower extremity
Deep Veins of Lower Extremity
  • 3 paired stem veins of the calf accompany the arteries as venae commitantes + anastomose freely with each other:
  • 1. Anterior tibial veins
    • draining blood from dorsum of foot, running within extensor compartment of lower leg close to interosseous membrane
  • 2. Posterior tibial veins
    • formed by confluence of superficial + deep plantar veins behind ankle joint
  • 3. Peroneal veins
    • directly behind + medial to fibula
  • 4. Calf veins
    • Soleal muscle veins
      • baggy valveless veins in soleus muscle (= sinusoidal veins); draining into posterior tibial + peroneal veins or lower part of popliteal vein
    • Gastrocnemius veins
      • thin straight veins with valves; draining into lower + upper parts of popliteal vein
  • 5. Popliteal vein
    • formed by stem veins of lower leg
  • 6. Femoral / superficial femoral vein
    • continuation of popliteal vein; receives deep femoral vein about 9 cm below inguinal ligament
  • 7. Deep femoral vein
    • draining together with superficial femoral vein into common femoral vein; may connect to popliteal vein (38%)
  • 8. Common femoral vein
    • formed by confluence of deep + superficial femoral vein; becomes external iliac vein as it passes beneath inguinal ligament
image
Superficial Venous System of Lower Extremity
image
Deep Venous System of Lower Extremity
P.611

image
Inguinal Arterial Anatomy (right side)
Superficial Veins of Lower Extremity
  • Greater saphenous vein
    • formed by union of veins from medial side of sole of foot with medial dorsal veins; ascends in front of medial malleolus; passes behind medial condyles of tibia + femur
    • (a) Posterior arch vein
      • connected to deep venous system by communicating veins
    • (b) Anterior superficial tibial vein
    • (c) Posteromedial superficial thigh vein
      • often connects with upper part of lesser saphenous vein
    • (d) Anterolateral superficial thigh vein
    • (e) Tributaries in fossa ovalis
      • superficial inferior epigastric vein
      • superficial external pudendal vein
      • superficial circumflex iliac vein
  • Lesser saphenous vein
    • originates at outer border of foot behind lateral malleolus as continuation of dorsal venous arch; enters popliteal vein between heads of gastrocnemius in popliteal fossa within 8 cm of knee joint (60%) or joins with greater saphenous vein via posteromedial / anterolateral superficial thigh veins (20%)
Communicating = Perforating Veins
  • >100 veins in each leg
  • MEDIAL
    • Submalleolar communicating vein
    • Cockett group
      • group of 3 veins located 7, 12, 18 cm above the tip of medial malleolus connecting posterior arch vein with posterior tibial vein
    • Boyd vein
      • located 10 cm below knee joint connecting main trunk of greater saphenous vein to posterior tibial veins
    • Dodd group
      • group of 1 or 2 veins passing through Hunter canal (= subsartorial canal) to join greater saphenous vein with superficial femoral vein
  • LATERAL
    • Lateral communicating vein
      • located from just above lateral malleolus to junction of lower-to-mid thirds of calf connecting lesser saphenous vein with peroneal veins
    • Posterior mid-calf communicating veins
      • located posteriorly 5 + 12 cm above os calcis joining lesser saphenous vein to peroneal veins
    • Soleal + gastrocnemius points
      • joining short saphenous vein to soleal / gastrocnemius veins
Contents of femoral triangle
mnemonic: NAVEL (from lateral to medial)
  • Nerve
  • Artery
  • Vein
  • Empty space
  • Lymphatics
Pulsatility
  • = assessment of vascular resistance (increased resistance reduces diastolic flow)
  • Can be assessed in vessels too small / tortuous to be imaged (Doppler angle unnecessary)!
  • Index should be calculated for each of several cardiac cycles (5 heartbeats adequate) an average value taken
    • S = A = maximal systolic shift
    • D = B = end-diastolic frequency shift
  • 1. Full pulsatility index of Gosling (PIF) = 1/A02 SAi2
  • 2. Simplified pulsatility index (PI) = (S - D)/mean
  • 3. Resistance index (RI) = Pourcelot index = (S- D)/S or 1 - (D/S)
  • 4. Stuart index = A/B ratio = S/D ratio
  • 5. B/A ratio = B(100%)/A
image
Pulsatility
P.612

image
Doppler Waveforms of Hepatic Veins
P.613

Cardiovascular Disorders
Aberrant Left Pulmonary Artery
  • = LEFT PULMONARY ARTERY SLING
Embryology: failure of development/obliteration of left 6th aortic arch (= vascular pedicle for left lung); left lung parenchyma maintains a connection with right lung leading to development of a collateral branch of the right pulmonary artery to supply the left lung
Site: left PA passes above right mainstem bronchus + between trachea and esophagus on its way to left lung (= sling around proximal right main bronchus + distal trachea)
Age at presentation: neonate to adulthood
Classification:
  • Normal bronchial pattern
  • Malformation of bronchotracheal tree
    Associated with:
    • “napkin-ring trachea” = absent pars membranacea (50%)
    • Stenosis of long tracheal segment
    • PDA (most common), ASD, persistent left SVC
  • • stridor (most common), wheezing, apneic spells, cyanosis
  • • respiratory infection
  • • feeding problems
  • deviation of trachea to left
  • “inverted-T” appearance of mainstem bronchi = horizontal course secondary to lower origin of right mainstem bronchus
  • anterior bowing of right mainstem bronchus
  • “carrot-shaped trachea” = narrowing of tracheal diameter in caudad direction resulting in functional tracheal stenosis
  • obstructive emphysema/atelectasis of RUL + LUL
  • low left hilum
  • separation of trachea + esophagus at hilum by soft-tissue mass
  • anterior indentation on esophagogram
Amyloidosis
  • = extracellular deposits of insoluble fibrillar protein
  • • asymptomatic/CHF (restrictive cardiomyopathy), arrhythmia
CXR:
  • normal/generalized cardiomegaly
  • pulmonary congestion
  • pulmonary deposits of amyloid
NUC:
  • striking uptake of Tc-99m pyrophosphate greater than bone (50–90%)
ECHO:
  • granular sparkling appearance of myocardium
  • LV wall thickening
  • decreased LV systolic + diastolic function
Anomalous Left Coronary Artery
  • = left coronary artery arises from pulmonary trunk (left sinus of Valsalva)
Hemodynamics:
  • with postnatal fall in pulmonary arterial pressure perfusion of LCA drops (ischemic left coronary bed), collateral circulation from RCA with flow reversal in LCA
    • adequate collateral circulation = lifesaving
    • inadequate collateral circulation = myocardial infarction
    • large collateral circulation = L-to-R shunt with volume overload of heart
  • • episodes of sweating, ashen color (angina symptomatology)
  • • ECG: anterolateral infarction
  • • continuous murmur (if collaterals large)
  • dilatation of LV
  • enlargement of LA
  • normal pulmonary vascularity/redistribution
Rx: (1) Ligation of LCA at its origin from pulmonary trunk
(2) Ligation of LCA + graft of left subclavian artery to LCA
(3) Creation of an AP window + baffle from AP window to ostium of LCA
DDx: endocardial fibroelastosis, viral cardiomyopathy (NO shocklike symptoms)
Anomalous Pulmonary Venous Return
  • = lack of an appropriate connection of the primitive pulmonary vein to the left atrium with persistence of fetal pulmonary + systemic connections
Total Anomalous Pulmonary Venous Return
  • = entire pulmonary venous return directed to RA = TAPVR = admixture lesion because of the combination of cyanosis + increased pulmonary vascularity (L-to-R and R-to-L shunt)
Embryology: anomalous connection between pulmonary veins and systemic veins secondary to embryologic failure of the common pulmonary vein to join the posterior wall of the left atrium
Prevalence: 2% of CHD
Age: symptomatic in 1st year of life
  • • cyanosis
Hemodynamics:
  • obstruction along the pulmonary venous pathway
    image
Associated with:
  • asplenia; ASD/patent foramen ovale (necessary for survival), bronchopulmonary sequestration, pulmonary arteriovenous malformation, cystic adenomatoid malformation
MR:
  • retroatrial sign = presence of veins posterior to LA
  • small LA without pulmonary venous connections
  • patent foramen ovale/ASD
  • anomalous vein with variable location
Overall prognosis: 75% mortality rate within 1 year of birth if untreated
P.614

Supradiaphragmatic TAPVR (82%)
Type I = SUPRACARDIAC TAPVR (52%)
= drainage into left brachiocephalic vein/right + left persistent SVC/azygos vein by way of a vertical vein; <10% obstructed
Type II = CARDIAC TAPVR (30%)
= drainage into coronary sinus (80%)/RA
Hemodynamics:
  • functional L-to-R shunt from pulmonary veins to RA
  • increased pulmonary blood flow (= overcirculation)
  • obligatory R-to-L shunt via usually patent foramen ovale/ASD restores oxygenated blood to left side
  • normal systemic venous pressure with increased flow through widened SVC
  • after birth CHF secondary to
    • mixture of systemic + pulmonary venous blood in RA
    • volume overload of RV
Age: presentation <1 years of age
  • • cyanosis
  • • neck veins undistended (shunt level distally)
  • • R ventricular heave (= increased contact of enlarged RV with sternum)
  • • systolic ejection murmur (large shunt volume)
  • overall heart size notably normal:
    • slightly enlarged RV (= volume overload with time)
    • normal/enlarged RA
    • normal LA (= ASD acts as escape valve)
  • dilated SVC + left vertical vein:
    • “figure of 8”/“snowman” configuration of cardiac silhouette (= dilated SVC + left vertical vein)
    • pretracheal density on lateral film (= left vertical vein)
  • increased pulmonary blood flow (= overcirculation)
  • absent connection of pulmonary veins to LA
Sub-/Infradiaphragmatic TAPVR (12%)
  • = type III
  • = drainage into portal vein/IVC/ductus venosus/left gastric vein with constriction of descending pulmonary vein by diaphragm en route through esophageal hiatus leading to pulmonary venous hypertension + RV pressure overload; >90% obstructed
Age: presentation in neonatal period
  • • intense cyanosis + respiratory distress (R-to-L shunt through ASD)
Prognosis: death within a few days of life
Associated with: asplenia syndrome (80%), polysplenia
  • unique appearance of pulmonary edema + pulmonary venous congestion with normal-sized heart (DDx: hyaline membrane disease)
  • low anterior indentation on barium-filled esophagus
Mixed Type of TAPVR (6%)
  • = type IV
  • = with various connections to R side of heart (6%)
Partial Anomalous Pulmonary Venous Return
  • = PAPVR = only a portion of the pulmonary venous circulation connects to the LA
  • May occur in isolation
N.B.: venous return almost never obstructed!
Prevalence: 0.3–0.5% of patients with CHD
Age: presentation later in life than TAPVR
May be associated with:
  • Atrial septal defect (25%)
    • RUL pulmonary vein enters SVC/RA (66%)
      • 90% of patients with sinus venosus type ASD have PAPVR
      • 50% of patients with PAPVR have sinus venosus type ASD
      • RUL vein courses in a horizontal direction
    • LUL pulmonary vein enters brachiocephalic vein (33%)frequently associated with: ostium secundum type ASD (10–15%)
      • vertical mediastinal density lateral to aortic knob extending upward and medially with smooth curvilinear border (DDx: persistent left SVC)
  • Hypogenetic lung as a component of congenital pulmonary venolobar syndrome
    • = Scimitar Syndrome
    • = lower part/all of the hypogenetic lung is drained by an anomalous vein
    • Anomalous vein drains into:
      • IVC below right hemidiaphragm (33%)
      • suprahepatic portion of IVC (22%)
      • hepatic veins
      • portal vein (11%)
      • azygos vein
      • coronary sinus
      • right atrium (22%)
      • left atrium = “meandering pulmonary vein”
        • Drainage into suprahepatic portion of IVC/right atrium may be a clue for interruption of intrahepatic portion of IVC!
    May be associated with: systemic arterialization of the lung without sequestration
    Location: almost exclusively on right side
    • tubular structure paralleling the right heart border in the configuration of a Turkish sword = “scimitar” (PA view)
    • shift of heart + mediastinum into right chest
  • • acyanotic
  • • ASD symptomatology
  • radiographic findings similar to ASD
  • anomalous course of draining vein
  • enlargement of draining site: SVC, IVC, azygos vein
CECT:
  • nodular/tubular opacity (= anomalous vein), which opacifies in phase with pulmonary vein
Aortic Aneurysm
Cause:
  • Atherosclerosis (73–80–90%): descending aorta
  • Traumatic (15–20%): following transection; descending aorta
  • Congenital (2%): aortic sinus, post coarctation, ductus diverticulum
  • Syphilis (19%): ascending aorta + arch
  • Mycotic = bacterial dissection; anywhere
  • P.615

  • Cystic media necrosis (Marfan/Ehlers-Danlos syndrome, annuloaortic ectasia): ascending aorta
  • Inflammation of media + adventitia:
    • Takayasu arteritis, giant cell arteritis, relapsing polychondritis, rheumatic fever, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, psoriasis, ulcerative colitis, systemic lupus erythematosus, scleroderma, Behçet disease, radiation
  • Increased pressure:
    • systemic hypertension, aortic valve stenosis
  • Abnormal volume load: severe aortic regurgitation
True Aneurysm
  • = permanent dilatation of all layers of weakened but intact wall
False Aneurysm
  • = focal perforation with all layers of wall disrupted; escaped blood contained by adventitia/perivascular connective tissue + organized blood
Fusiform Aneurysm (80%)
  • = circumferential involvement
Saccular aneurysm
  • = involvement of portion of wall
Abdominal Aortic Aneurysm (AAA)
  • There is no consensus regarding the definition of an atherosclerotic AAA!
  • = focal widening >3 cm (ultrasound literature); twice the size of normal aorta/>4 cm [Bergan, Ann Surg 1984]
  • Normal size of abdominal aorta >50 years of age:
    • 12–19 mm in women; 14–21 mm in men
Prevalence: 1.4–8.2% in unselected population; in 6% >80 years of age; in 6–20% of patients with signs of atherosclerotic disease; M > F; Whites:Blacks = 3:1
Cause: ? genetic (10-fold increase in risk as first-degree relative of patient with AAA); structural defect of aortic wall caused by increased proteolysis; copper deficiency
Risk factors: male sex, age >75 years, white race, prior vascular disease, hypertension, cigarette smoking, family history, hypercholesterolemia
Age: >60 years; M:F = 5–9:1
Associated with:
  • visceral + renal artery aneurysm (2%)
  • isolated iliac + femoral artery aneurysm (16%):
  • common iliac (89%), internal iliac (10%), external iliac (1%)
  • stenosis/occlusion of celiac trunk/SMA (22%)
  • stenosis of renal artery (22–30%)
  • occlusion of inferior mesenteric artery (80%)
  • occlusion of lumbar arteries (78%)
Growth rate of aneurysm of 3–6 cm in diameter:
  • 0.39 cm/year
  • • asymptomatic (30%)
  • • abdominal mass (26%)
  • • abdominal pain (37%)
  • Imaging should provide information about:
    • the proximal extent of the aneurysm, which determines the site of clamping of the aorta (origin of renal arteries)
    • the course of the left renal vein (retroaortic?)!
Location: infrarenal (91–95%) with extension into iliac arteries (66–70%)
Plain film:
  • mural calcification (75–86%)
US:
  • >98 % accuracy in size measurement
NCCT:
  • perianeurysmal fibrosis (10%), may cause ureteral obstruction
  • “crescent sign” = peripheral high-attenuating crescent in aneurysm wall (= acute intramural hematoma) = sign of impending rupture
CECT:
  • ruptured aneurysm
    • anterior displacement of kidney
    • extravasation of contrast material
    • fluid collection/hematoma within posterior pararenal + perirenal spaces
    • free intraperitoneal fluid
    • perirenal “cobwebs”
  • contained leak
    • laminated mural calcification
    • periaortic mass of mixed/soft-tissue density
    • lateral “draping” of aneurysm around vertebral body
    • focal discontinuity of calcifications (unreliable)
    • indistinct aortic wall (unreliable)
Angio (AP + LAT filming):
  • focally widened aortic lumen >3 cm
  • apparent normal size of lumen secondary to mural thrombus (11%)
  • mural clot (80%)
  • slow antegrade flow of contrast medium
  • Contained rupture = extraluminal hematoma/cavity
    • absent parenchymal stain = avascular halo
    • displacement + stretching of aortic branches
Cx:
  • Rupture (25%)
    • into retroperitoneum: commonly on left
    • into GI tract: massive GI hemorrhage
    • into IVC: rapid cardiac decompensation
    Incidence: aneurysm <4 cm in 10%, 4–5 cm in 23%, 5–7 cm in 25%, 7–10 cm in 46%, >10 cm in 60%
    • sudden severe abdominal pain ± radiating into back
    • faintness, syncope, hypotension
    Prognosis: 64–94% die before reaching hospital
    Increased risk: size >6 cm, growth >5 mm /6 months, pain + tenderness
    • The exact moment of rupture is unpredictable!
    • Cause of death in 1.3% of men >65 years!
  • Peripheral embolization
  • Infection
  • Spontaneous occlusion of aorta
Prognosis: 17% 5-year survival without surgery,
50–60% 5-year survival with surgery
Rx: surgery recommended if >5 cm in diameter; 4–5% surgical mortality for nonruptured, 30–80% for ruptured aneurysm
P.616

Postoperative Cx:
  • Left colonic ischemia (1.6%) with 10% mortality
  • Renal failure (14%)
  • 0–8% mortality rate for elective surgery
Atherosclerotic Aneurysm
Incidence: most common cause of aortic aneurysms; leading cause of thoracic aortic aneurysm
Histo: diseased intima with secondary degeneration + fibrous replacement of media; ultimately wall of aneurysm composed of acellular + avascular connective tissue
Pathophysiology:
  • progressive weakening of media results in vessel dilatation + increased tension of vessel wall (law of Laplace = tensile stress varies with product of blood pressure and radius of vessel); compromise of mural vascular nutrition (vasa vasorum) causes further degeneration + progressive dilatation
Age: elderly; M > F
  • • asymptomatic (most)
  • • chest pain; symptoms related to compression of adjacent structures (dysphagia, hoarseness, lobar atelectasis, pneumonia, parenchymal hemorrhage, superior vena cava syndrome)
Location: distal abdominal aorta (66%) > iliac a. > popliteal a. > common femoral a. > aortic + descending thoracic aorta > carotid a. > ascending aorta
Site: (1) infrarenal aorta (associated with thoracic aneurysm in 29%)
(2) descending thoracic aorta distal to left subclavian artery
(3) thoracoabdominal
  • fusiform (80%), saccular (20%)
  • frequently contain calcified thrombus with irregular inner contour
Cx: rupture (cause of death in 50%): usually unrestrained + fatal in thoracic location
Degenerative Aneurysm
  • = medial degeneration
  • Most common cause of aneurysm in ascending aorta
Cause: (1) genetically transmitted metabolic disorder: Marfan syndrome, Ehlers-Danlos syndrome
(2) acquired: result of repetitive aortic injury + repair associated with aging
Inflammatory Aortic Aneurysm
  • = defined as triad of
    • thickened wall of aneurysm
    • extensive perianeurysmal + retroperitoneal fibrosis
    • dense adhesions of adjacent abdominal organs
Frequency: 3-10% of all AAAs; M:f = 6:1 to 30:1
Mean age: 62-68 years
  • abdominal/back pain
  • weight loss + anorexia (20–41%)
  • elevated ESR (40–88%)
  • tender pulsatile abdominal mass (15–30%)
Comorbidities: arterial hypertension (34–69%), arterial occlusive disease (10–47%), diabetes mellitus (3–13%), coronary artery disease (33–55%)
Size: usually small at presentation because of early symptomatology
CT:
  • rind of homogeneous soft-tissue density surrounding aorta anteriorly + laterally
  • contrast enhancement (DDx from hematoma)
  • entrapment of ureters (10–21%)
US:
  • sonolucent halo around aorta
Cx: enlargement + rupture (lower rate than in noninflammatory aneurysm)
Leaking Aortic Aneurysm
  • • acute chest pain
At risk for rupture: symptomatic > asymptomatic aneurysm; mycotic aneurysm; thoracic aortic aneurysm > 6 cm
MR:
  • irregular aneurysm wall
  • extra-aortic blood
  • pleural effusion containing high signal intensity on T1WI (methemoglobin)
  • admixture of lower-intensity blood products + fat in mediastinum
Cx: rupture into left pleural space (descending thoracic aorta); rupture into pericardium/ mediastinum (ascending thoracic aorta)
Mycotic Aneurysm
Incidence: 2.6% of all abdominal aneurysms
  • PRIMARY MYCOTIC ANEURYSM (rare)
    • unassociated with any demonstrable intravascular inflammatory process
  • SECONDARY MYCOTIC ANEURYSM
    • = aneurysm due to nonsyphilitic infection
    Predisposing factors:
    • IV drug abuse
    • Bacterial endocarditis (12%)
    • Immunocompromise (malignancy, alcoholism, steroids, chemotherapy, autoimmune disease, diabetes)
    • Atherosclerosis
    • Aortic trauma caused by accidents/aortic valve surgery/coronary artery bypass surgery/arterial catheterization
    Mechanism:
    • septicemia with abscess formation via vasa vasorum
    • septicemia with abscess formation via vessel lumen
    • direct extension of contiguous infection (of sternum/spine) weakening + destroying part of the aortic wall
    • preexisting intima laceration (trauma, atherosclerosis, coarctation)
    P.617

    Organism: S. aureus (53%), Salmonella (33-50%), nonhemolytic Streptococcus, Pneumococcus, Gonococcus, Mycobacterium (contiguous spread from spine/lymph nodes)
    Histo: loss of intima + destruction of internal elastic lamella; varying degrees of destruction of muscularis of media + adventitia
    • • frequently insidious, fever (NO acute chest pain)
    • • positive blood culture in 50%
Site: ascending aorta near sinus of Valsalva > abdominal visceral artery > intracranial artery > lower/upper extremity artery
  • true (majority)/false aneurysm
  • saccular structure arising eccentrically from aortic wall with rapid enlargement
  • interrupted ring of aortic wall calcification
  • periaortic gas collection
  • adjacent vertebral/sternal osteomyelitis
  • adjacent reactive lymph node enlargement
Cx: (1) Life-threatening rupture + hemorrhage (75%)
(2) Uncontrolled sepsis if untreated
Rx: surgery
Prognosis: 67% overall mortality
Syphilitic Aneurysm
Spectrum:
  • Uncomplicated syphilitic aortitis
  • Syphilitic aortic aneurysm (mostly saccular)
  • Syphilitic aortic vasculitis (aortic regurgitation)
Incidence: 12% of patients with untreated syphilis
Onset: 10–30 years after initial spirochete infection
Histo: chronic inflammation of aortic adventitia + media beginning at vasa vasorum + leading to obstruction of vasa vasorum followed by nutritional impairment of media + loss of elastic fibers + smooth muscle fibers
  • positive venereal disease research laboratory (VDRL) test
  • positive microhemagglutination assay — Treponema pallidum (MHA-TP) test
Location: ascending aorta (36%), aortic arch (34%), proximal descending aorta (25%), distal descending aorta (5%), aortic sinuses (<1 %)
  • asymmetric enlargement of aortic sinuses (DDx to medial degeneration with symmetric enlargement)
  • saccular (75%)/fusiform (25%) aneurysm
  • pencil-thin dystrophic aortic wall calcification (up to 40%) most severe in ascending aorta, frequently obscured by thick coarse irregular calcifications of secondary atherosclerosis
Prognosis: death in 2%, rupture in up to 40%; death within months of onset of symptoms if untreated
Thoracic Aortic Aneurysm
  • Most common vascular cause of mediastinal mass!
    • 10% of mediastinal masses are of vascular origin!
  • Average diameter of thoracic aorta (increasing with age):
    • aortic root: 3.6 cm
    • ascending aorta 1 cm proximal to arch: 3.5 cm
    • arch 2.9 cm
    • proximal descending aorta: 2.6 cm
    • middle descending aorta: 2.5 cm
    • distal descending aorta: 2.4 cm
Definitions: diameter of 4–5 cm = aortic ectasia
diameter of >5 cm = aortic aneurysm
Frequency: 25% of all aneurysms
Cause: atherosclerosis (80%)
Associated with: hypertension, coronary artery disease, abdominal aneurysm (30%)
Mean age: 65 years; M:F = 3:1
  • • substernal/back/shoulder pain (26%)
  • • SVC syndrome (venous compression)
  • • dysphagia (esophageal compression)
  • • stridor, dyspnea (tracheobronchial compression)
  • • hoarseness (recurrent laryngeal nerve compression)
Location: arch > descending aorta
  • mediastinal mass with proximity to aorta
  • wide tortuous aorta
  • curvilinear peripheral calcifications (75%)
  • circumferential/crescentic mural thrombus
  • Angio: may show normal caliber secondary to mural thrombus
Cx: (1) Rupture into mediastinum, pericardium, either pleural sac, extrapleural space
“√” high-attenuation fluid
(2) Aortobronchopulmonary fistula
“√” consolidation of lung adjacent to aneurysm
⋄ Most aneurysms rupture when >10 cm in size
Prognosis: 1-year survival 57%, 3-year survival 26%, 5-year survival 19% (60% die from ruptured aneurysm, 40% die from other causes)
Rx: operative repair considered if >6 cm in diameter
Surgical mortality: 10%
Traumatic Aortic Pseudoaneurysm
  • = CHRONIC AORTIC PSEUDOANEURYSM
  • 2nd most common form of thoracic aortic aneurysm
  • Most common type occurring in young patients
Location vs Type of Thoracic Aortic Aneurysm
Location Type
sinus of Valsalva (1) congenital
(2) infective endocarditis
effacement of sinotubular junction (1) Marfan syndrome
(2) idiopathic aortoannular ectasia
(3) tertiary syphilis
aortic root mycotic aneurysm
aortic root + ascending aorta cystic medial necrosis (eg, Marfan syndrome)
ascending aorta syphilis
aortic arch atherosclerosis
ligamentum arteriosum trauma
descending aorta atherosclerosis
P.618

Incidence: 2.5% of patients who survive initial trauma of acute aortic transection
  • usually calcified
  • may contain thrombus
Cx: (1) progressive enlargement
(2) rupture (even years after insult)
Complications of Endovascular Stent-Graft Repair
  • Endoleak (2–45%)
    • = leakage into the aneurysm outside stent-graft
    • Type 1 = incomplete fixation of stent-graft to aortic wall at the proximal/distal attachment site
    • Type 2 = retrograde flow via parent artery (eg, lumbar/inferior mesenteric artery)
    • Type 3 = endograft defect with disruption of either metallic support/fabric
    Prognosis: enlargement of leak, aneurysm rupture
  • Graft kinking
    Cause: diminishing diameter of aneurysm after stent-graft implantation also decreases length of aneurysm
    Associated with: distal migration of stent-graft
  • Graft infection
    • interval development of perigraft soft-tissue attenuation/air
    Rx: antibiotics + total excision of infected graft
  • Graft thrombosis (3–19%)
    • = intraluminal circular/semicircular thrombus
    Prognosis: spontaneous shrinkage, development of complete thrombosis
  • Graft occlusion
  • Shower embolism (4–17%)
    Cause: mural thrombus dispersed by delivery system
    Prognosis: perioperative death
  • Colon necrosis
    Cause: occlusion of inferior mesenteric artery by stent-graft
  • Aortic dissection
    Cause: retrograde injury by delivery system
Aortic Dissection
  • = spontaneous longitudinal separation of aortic intima and adventitia by circulating blood having gained access to the media of the aortic wall splitting it in two
Path: destruction of media leads to formation of a false channel:
(1) Transverse tear in weakened intima (95–97%)
“⋄” The diagnosis relies primarily on visualization of an intimal flap + blood flow within a false lumen
(2) Primary hemorrhage into aortic wall WITHOUT intimal tear (3–5–13%) = INTRAMURAL AORTIC HEMATOMA see below
Pathogenesis:
  • intimal tear results from combination of following factors:
  • (a) media degeneration decreases cohesiveness within aortic wall
  • (b) persistent aortic motion secondary to a beating heart stresses the aortic wall
  • (c) hydrodynamic forces accentuated by hypertension
Incidence: 3:1,000 (more common than all ruptures of thoracic + abdominal aorta combined); 1:205 autopsies; 2,000 cases/year in USA
Peak age: 60 years (range 13–87 years); M:F = 3:1
Predisposed: (cystic medial necrosis/disease of aortic wall)
  • Starts in fusiform aneurysms in 28%
  • Does not occur in aneurysms <5 cm in diameter
  • 1. Hypertension (60–90%)
  • 2. Marfan syndrome (16%)
  • 3. Ehlers-Danlos syndrome
  • 4. Relapsing polychondritis
  • 5. Valvular aortic stenosis
  • 6. Turner syndrome
  • 7. Behçet disease
  • 8. Coarctation NOT syphilis
  • 9. Bicuspid aortic valve
  • 10. S/P prosthetic valve
  • 11. Trauma (rare)
  • 12. Catheterization
  • 13. Pregnancy
  • 14. Aortitis (eg, SLE)
  • 15. Cocaine abuse
  • In women 50% of dissections occur during pregnancy!
  • • sharp tearing intractable anterior/posterior chest pain (75–95%) radiating to jaw, neck, low back (DDx: myocardial infarction)
  • • murmur ± bruit (65%) from aortic regurgitation
  • • asymmetric peripheral pulses + blood pressures (59%)
  • • absent femoral pulses (25%), reappearing after reentry
  • • pulse deficit: in up to 50% of type A dissection, in 16% of type B dissection
  • • hemodynamic shock (25%)
  • • neurologic deficits (25%): hemiplegia, paraparesis (due to compromise of anterior spinal artery of Adamkiewicz)
  • • persistent oliguria
  • • congestive heart failure (rare) due to acute aortic insufficiency
  • • recurrent arrhythmias/right bundle branch block
  • • signs of pericardial tamponade: clouded sensorium, extreme restlessness, dyspnea, distended neck veins
image
Aortic Dissection
Types:
DeBakey Classification:
Type I (29–34%) = ascending aorta + portion distal to arch
Type II (12–21%) = ascending aorta only
Type III (50%) = descending aorta only
subtype III A = up to diaphragm
subtype III B = below diaphragm
Stanford Classification: (preferable as it effects treatment recommendations)
Type A (60–70%) = ascending aorta ± arch in first 4 cm in 90%
Type B (30–40%) = descending aorta only
mnemonic: Affec aortaffects aortascending aorta and arch;
B begins beyond brachiocephalic vessels;
I = II + III
P.619

Clinical classification:
(1) Acute aortic dissection: <2 weeks old
(2) Chronic aortic dissection: >2 weeks old
Flow velocities (average):
  • • 13.4 cm/sec in true lumen
  • • 3.1 cm/sec in false lumen
  • • retrograde flow more common in false lumen
Location of dissection (following helical flow pattern):
  • –on anterior + right lateral wall of ascending aorta just distal to aortic valve (65%)
  • –on superior + posterior wall of transverse aortic arch (10%)
  • –on posterior + left lateral wall of upper descending aorta distal to left subclavian artery (20%)
  • –more distal aorta (5%) usually terminating in left iliac artery (80%)/right iliac artery (10%) [involvement of left renal artery in 50%]
  • An exit/distal tear/reentry occurs in 10%!
Atypical configurations of intimal flap:
  • circumferential intimal flap due to dissection of entire intima
  • filiform intimal flap creating an extremely narrow true lumen (±} ischemic complications)
  • mural calcification of false lumen (in chronic dissection)
  • three-channel aorta (= Mercedes-Benz sign) due to two false channels
  • intimointimal intussusception
CXR (best assessment from comparison with serial films):
  • normal CXR in 25%
  • “calcification sign” = inward displacement of atherosclerotic plaque by >4-10 mm from outer aortic contour (7%), can only be applied to contour of descending aorta secondary to projection, may be misleading in presence of periaortic soft-tissue mass/hematoma
  • disparity in size between ascending + descending aorta
  • irregular wavy contour/indistinct outline of aorta
  • widening of superior mediastinum to >8 cm due to hemorrhage/enlarging false channel (40-80%)
  • cardiac enlargement (LV hypertrophy/hemopericardium)
  • left pleural effusion (27%)
  • atelectasis of lower lobe
  • rightward displacement of trachea/endotracheal tube
ECHO:
  • transesophageal echocardiography (tee): 95–100% sensitive + 77–97% specific
    • Ultrasound can be performed at bedside!
    False-positive (33%): reverberation artifacts from calcified aortic wall
  • transthoracic US: 59–85% sensitive + 63–96% specific for type A dissection; poorer for type B
  • intravascular in conjunction with aortography to differentiate true from false lumen
    • intimal flap (seen in more than one view)
    • pericardial fluid
    • aortic insufficiency
False-positives: reverberation echoes from aneurysmal ascending aorta/calcified atheromatous plaque, postoperative periaortic hematoma
Angio (86–88% sensitive, 75–94% specific):
  • Largely replaced by noninvasive cross-sectional imaging techniques
Superior to any other technique in demonstrating
  • entry + reentry points (in 50%)
  • branch vessel involvement + coronary
  • aortic insufficiency
  • visualization of intimal/medial flap radiolucency within opacified aorta
  • “double barrel aorta” (87%) = opacification lumens
  • abnormal catheter position outside anticipated aortic course
  • compression of true lumen by false channel (72–85%)
  • aortic valvular regurgitation (30%)
  • increase in aortic wall thickness >6–10 mm
  • obstruction of aortic branches: left renal artery (25–30%)
  • ulcerlike projections caused by truncated branches
  • slower blood flow in false lumen
False-negative: complete thrombosis/slow blood flow of false channel (10%), intimal flap not tangential to x-ray beam
False-positive: thickening of aortic wall due to aneurysm, aortitis, adjacent neoplasm/hemorrhage
CECT (87–100% sensitive, 87–100% specific):
  • within 4 hours (if patient responds rapidly to medical Rx); detection as accurate as angio with single-level dynamic scanning
  • crescentic high-attenuation clot within false lumen
  • internally displaced intimal calcification (DDx: calcification of thrombus on luminal surface or within)
  • intimal flap separating two aortic channels (may be seen without contrast in anemic patients)
  • entry tear = most proximal split/discontinuity in intimal flap
False-negative: inadequate contrast opacification, thrombosed lumen misinterpreted as aortic aneurysm with mural thrombus
False-positive: perivenous streaks secondary to beam hardening + motion, cardiac/aortic motion artifacts, opacified normal sinus of Valsalva, normal pericardial recess mistaken for thrombus, mural thrombus in a fusiform aortic aneurysm, periaortic fibrosis, anemia with apparent high attenuation of aortic wall
MR (95–100% sensitive, 90–100% specific):
Advantage: large field of view in any plane; contrast material not necessary
Disadvantage: longer imaging time; difficulty monitoring acutely ill patients; image degradation from motion (uncooperative patient, atrial fibrillation)
P.620

SE images:
  • intimal flap of medium intensity outlined by signal voids of rapidly flowing blood in true + false lumen
  • intimal flap more difficult to detect in the presence of slow flow/thrombus (false lumen has intermediate intensity instead of flow void)
  • “cobwebs” (25%) traversing the corners of the false lumen = bands of medial elastic lamellae spanning the junction of the dissecting septum with the outer wall of the false lumen
GRE images:
  • lower-intensity intimal flap between high-intensity channels of flowing blood
  • intermediate signal from thrombosed lumen
  • aortic valve insufficiency = conical area of signal loss from aortic valve into LV during systole (2° to intravoxel dephasing caused by turbulence)
Cx: (1) Retrograde dissection (in Stanford type A)
(a) aortic insufficiency
(b) occlusion of coronary artery (8%)
(c) internal rupture into RV, LA, vena cava, pulmonary artery producing large L-to-R shunt
(2) Occlusion/transient obstruction of major aortic branches (in up to 27%)
(a) static obstruction
√ flap enters branch-vessel origin
(b) dynamic obstruction = flap spares branch-vessel origin but covers it like a curtain
√collapsed true lumen outlined by a C-shaped flap envelope which is concave toward false lumen (ischemic configuration)
(3) External rupture of aorta into pleural cavity/pericardial sac: 70% mortality (= most common cause of death within 24 hours)
(4) Development of aneurysm (15%) of the true/false lumen
⋄Organs may receive their blood supply through either the true or false lumen or both!
Rx:
  • Reducing peak systolic pressure to 120–70 mm Hg (adequate alone for type III = B, which rarely progresses proximally): death from rupture of aortic aneurysm in 46% of hypertensive + 17% of normotensive patients
    Survival rate: 40–70% (with medical/surgical management)
  • Immediate surgical graft reinforcement of aortic wall (Type I, II = A) preventing rupture + progressive aortic valve insufficiency
Nonsurgical survival rate: <10%
Postsurgical mortality: 10–35%
Cx: myocardial infarction, stroke, respiratory insufficiency, pulmonary embolism, aortic rupture, pseudoaneurysm, graft infection
Prognosis without Rx:
  • immediate death (3%); death within: 1 day (20–30%), 1 week (50–62%), 3 weeks (60%), 1 month (75%), 3 months (80%), 1 year (80–95%)
Prognosis with Rx:
  • 5–10% mortality rate following timely surgery; 40% 10-year survival rate after leaving hospital
DDx: penetrating ulcer of thoracic aorta (= atherosclerotic lesion of mid-descending aorta with ulceration extending through intima into aortic media)
Intramural Aortic Hematoma (3–13%)
  • = ATYPICAL AORTIC DISSECTION = IMH
  • = aortic dissection without rupture of intima (? early stage/variant of aortic dissection)
Cause: hemorrhage of vasa vasorum
Path: clotted intramural blood within aortic media; NO/(?) slow flow within the false channel
  • • signs + symptoms + classification identical to classic dissection
  • NO intimal flap
NECT (necessary):
  • cuff/crescent of high attenuation
  • displacement of intimal calcification
CECT:
  • mural region of low attenuation with smooth border maintaining a constant circumferential relationship with aortic wall
MR:
  • crescent-shaped eccentric wall thickening
Aortography: not useful!
Cx: ulcerlike projection with progression to open dissection/saccular or fusiform aneurysm
Rx: (1) emergency surgical repair for type A hematoma (probably represents early stage with development of classic aortic dissection)
(2) observation for type B hematoma (may heal completely)
DDx:
  • Acutely thrombosed false lumen of dissection (tendency to spiral longitudinally around aorta)
  • Atheromatous mural thrombus (irregular internal border)
  • Focal periaortic soft-tissue mass (irregular external border)
    • idiopathic periaortic fibrosis
    • periaortic lymphoma
  • Aortic motion artifact simulating type A IMH
Aortic Prosthetic Graft Infection
Incidence: 1.3–6% of prosthetic graft procedures
Classification:
  • PERIGRAFT INFECTION (2–6%)
    • • fever, chills, leukocytosis
    • • groin swelling, heat, tenderness, pulsatile mass, draining sinus tract
  • aortoenteric FISTULA (0.6–2%)
    • • acute/chronic GI bleeding (may be occult)
    • • sepsis
  • • may be temporally remote (up to 10 years): median time of 3 years to manifestation (70% occur after 1st year)
  • • intracavitary signs: malaise, back pain, fever, elevated sedimentation rate, hydronephrosis, ischemia from clotted graft
Normal postoperative course:
  • ring of fat attenuation in early postoperative period <5 mm between aneurysm wall and graft
  • P.621

  • Complete resolution of hematoma by 3 months
  • Disappearance of ectopic gas complete by 4–7 weeks
CT (94% sensitive, 85% specific, 91% accurate):
  • perigraft fluid
  • perigraft soft-tissue attenuation with indistinctness of graft margins
  • ectopic gas (fistulous communication with bowel/gas-producing organism)
  • pseudoaneurysm (25%)
  • focal bowel wall thickening (indicates fistula)
  • >5 mm soft tissue between graft + surrounding wrap (beyond 7th postoperative week)
  • focal discontinuity of calcified aneurysmal wrap
False positives:
  • perigraft hematoma in early postoperative period, pseudoaneurysm (in 15–20%)
NUC:
  • uptake of Tc-99m hexametazine labeled leukocytes (drawbacks: not performed quickly, hepatobiliary excretion)
Prognosis: 17–75% mortality; 30–50% morbidity
Dx: positive culture from needle aspirate (incubation period should be up to 14 days as organisms may be slow-growing)
Aortic Regurgitation
  • = AORTIC INSUFFICIENCY
Cause:
  • INTRINSIC AORTIC VALVE DISEASE
    • Congenital bicuspid valve
    • Rheumatic endocarditis
    • Bacterial endocarditis (perforation/prolapse of cusp)
    • Myxomatous valve associated with cystic medial necrosis
    • Aortic valve prolapse
    • Prosthetic valve: mechanical break, thrombosis, paravalvular leak
  • PRIMARY DISEASE OF ASCENDING AORTA
    • Dilatation of aortic annulus
      • Syphilitic aortitis
      • Rheumatoid arthritis
      • Rheumatoid variants:
        • Ankylosing spondylitis (5–10%)
        • Reiter disease
        • Psoriatic arthritis
      • Relapsing polychondritis
      • Familial connective tissue disease:
        mnemonic: HOME
        • Homocystinuria
        • Osteogenesis imperfecta
        • Marfan syndrome
        • Ehlers-Danlos syndrome
    • Laceration = aortic dissection
      • Deceleration trauma
      • Hypertension
Pathogenesis: progressive enlargement of diastolic + systolic LV dimensions result in increase in myocardial fiber length + increase in stroke volume; decompensation occurs if critical limit of fiber length is reached
  • • “water-hammer pulse” = twin-peaked pulse
  • • systolic ejection murmur + high-pitched diastolic murmur
  • • Austin Flint murmur = soft mid-diastolic or presystolic bruit
  • LV enlargement (cardiothoracic ratio >0.55) + initially normal pulmonary vascularity (DDx: congestive cardiomyopathy, pericardial effusion)
  • normal aorta (in intrinsic valve disease)
  • dilatation of aorta (in systemic disease):
    • ± calcification of ascending aorta (in aortic wall disease)
    • ± enlarged aortic arch + tortuous descending aorta
  • increased pulsations along entire aorta
ECHO:
  • aortic root dilatation
  • high frequency flutter of aML (occasionally pML) during first 2/3 of diastole (CHARACTERISTIC)
  • high frequency diastolic flutter of IVS (uncommon)
  • diastolic flutter of aortic valve (SPECIFIC, but rare)
  • premature aortic valve opening (high diastolic LV pressure)
  • decreased MV opening (aML pushed posteriorly by regurgitant aortic jet)
  • premature closure of mitral valve (high diastolic LV pressure produces MV closure before beginning of systole in severe acute aortic insufficiency)
  • LV dilatation + large amplitude of LV wall motion (volume overload, increased ejection fraction):
End-systolic LV diameter Action
<50 mm yearly follow-up
50–54 mm 4- to 6-month follow-up
>55 mm valve replacement
Doppler:
  • slope of peak diastolic to end-diastolic velocity decrease >3 m/sec2 in severe aortic regurgitation
  • area of color Doppler regurgitant flow
  • ratio of width of regurgitant beam to width of aortic root is good predictor of severity (color Doppler)
Aortic rupture
  • = blood leakage through aortic wall
  • 1. Spontaneous rupture of aortic aneurysm
    Pathogenesis: small clefts occur at a fragile site within inner thrombus gradually expanding to outer layer of thrombus with gradual seepage of flowing blood into mural thrombus and aneurysmal wall
CT:
  • high-attenuation crescent sign (71%)
  • 2. Spontaneous rupture of descending thoracic aorta
    Predisposed: hypertension and atherosclerosis, NO preformed aneurysm!
    Pathogenesis: pressure atrophy of media due to overlying intimal atheromatous plaque causing localized ballooning of aortic wall prior to perforation
  • 3. Traumatic rupture/transection of thoracic aorta
    Cause: blunt trauma to thoracic aorta
    see BLUNT TRAUMA TO THORACIC AORTA
Aortic Stenosis
  • Aortic valve area decreased to <0.8 cm2 = 0.4 cm2/m2 BSA (normal 2.5–3.5 cm2)
P.622

  • ACQUIRED AORTIC STENOSIS
    • Rheumatic valvulitis (almost invariably associated with mitral valve disease)
    • Fibrocalcific senile aortic stenosis (degenerative)
  • CONGENITAL AORTIC STENOSIS (most common)
    • = most frequent CHD associated with IUGR
    • 1. Subvalvular AS (15–30%)
    • 2. Valvular AS (60–70%): degeneration of bicuspid valve most common cause
    • 3. Supravalvular AS (rare)
Pathogenesis: increased gradient across valve produces LV hypertrophy and diminished LV compliance; increased muscle mass may outstrip coronary blood supply (subendocardial myocardial ischemia with angina); LV decompensation leads to LV dilatation + pulmonary venous congestion
  • • asymptomatic for many years
  • • angina, syncope, heart failure
  • • systolic murmur
  • • carotid pulsus parvus et tardus
  • • diminished aortic component of 2nd heart sound
  • • sudden death in severe stenosis (20%) after exercise (diminished flow in coronary arteries causes ventricular dysrhythmias + fibrillation)
  • poststenotic dilatation of ascending aorta (in 90% of acquired, in 70% of congenital AS)
  • normal-sized/enlarged LV (small LV chamber with thick walls)
  • @ in adults >30 years
    • calcification of aortic valve (best seen on RAO); indicates gradient >50 mm Hg
    • discrete enlargement of ascending aorta (NO correlation with severity of stenosis)
    • calcification of mitral annulus
    • “left ventricular configuration” = concavity along mid left lateral heart border + increased convexity along lower left lateral heart border
  • @ in children/young adults
    • prominent ascending aorta
    • left ventricular heart configuration
  • @ in infancy:
    • left ventricular stress syndrome
ECHO:
  • thickened + calcified aortic valve with multiple dense cusp echoes throughout cardiac cycle (right > noncoronary > left coronary cusp)
  • decreased separation of leaflets in systole with reduced opening orifice (13–14 mm = mild AS;
  • 8–12 mm = moderate AS; <8 mm = severe AS)
  • ± doming in systole
  • dilated aortic root
  • increased thickness of LV wall (= concentric LV hypertrophy)
  • hyperdynamic contraction of LV (in compensated state)
  • decreased mitral EF slope (reduced LV compliance)
  • LA enlargement
  • increased aortic valve gradient (Doppler)
  • decreased aortic valve area (unreliable)
DDx: calcification of aortic annulus in elderly/calcified coronary artery ostium (thickened cusp echoes only in diastole)
Prognosis: depends on symptomatology (angina, syncope, CHF)
Subvalvular Aortic Stenosis
  • = SUBAORTIC STENOSIS
  • (a) Anatomic/fixed subaortic stenosis
    Associated with: cardiac defects in 50% (usually VSD)
    Type I : thin 1–2-mm membranous diaphragmatic stenosis, usually located within 2 cm or less of valve annulus
    Type II: thick collarlike stenosis
    Type III : irregular fibromuscular stenosis
    Type IV : “tunnel subaortic stenosis” = fixed tunnel-like narrowing of LVOT = excessive thickening of only upper ventricular septum with normal mitral valve motion
  • (b) Functional/dynamic subaortic stenosis
    • Asymmetric septal hypertrophy (ASH)
    • Idiopathic hypertrophic subaortic stenosis (IHSS)
    • Hypertrophic obstructive cardiomyopathy (HOCM) may occur in infants of diabetic mothers
  • no dilatation of ascending aorta
  • asymmetrically thicker ventricular septum than free wall of LV (95%)
  • normal/small left + right ventricular cavities (95%)
  • lucent subaortic filling defect in systole
  • focal convexity of left upper-mid cardiac margin = anterior aspect of ventricular septum (rare)
  Congenital Rheumatic Degenerative
Clinically apparent <30 years 30-60 years >65 years
Valve calcifications
   first appearance 25 years 47 years 54 years
   pattern nodular/bicuspid nodular nodular/tricuspid
   on CXR >90% (40-65 years) <10% >90% (>65 years)
Aortic ectasia ascending Ao ascending Ao entire Ao
ECHO:
  • coarse systolic flutter of valve cusps
  • P.623

  • opening of leaflets followed by rapid inward move in mid systole, leaflets may remain in partially closed position through latter portion of systole (to appose borders of the flow jet)
  • systolic anterior motion of mitral valve
Cx: mitral regurgitation (secondary to abnormal position of anterolateral papillary muscle preventing complete closure of MV in systole)
Valvular Aortic Stenosis
  • = fusion of commissures between cusps
Degree: mild: >0.7 cm2; moderate: 0.5–0.7 cm2; severe: <0.5 cm2
Congenital types:
  • (a) bicuspid/unicuspid (in 95%): in 1–2% of population; M > F; commonly associated with coarctation of the aorta
  • (b) tricuspid (5%)
  • (c) dysplastic thickened aortic cusps
  • valvular calcifications (in 60% of patients >24 years of age)
  • @ IN INFANT with critical aortic stenosis:
    • • intractable CHF in first days/weeks of life with severe dyspnea
    • • may simulate neonatal sepsis
      Associated with: L-to-R shunts (ASD, VSD)
    • marked cardiomegaly (thickened wall of LV)
    • pulmonary venous hypertension
    • decreased ejection fraction
    • doming of thickened valve cusps
    • dilated ascending aorta
    Rx: emergency surgical dilatation
  • @ IN CHILD:
    • • asymptomatic until late in life
    • normal pulmonary vascularity
    • LV configuration with normal size of heart
    • large posterior noncoronary cusp, smaller fused right + left cusps
    • doming of thickened valve cusps
    • eccentric jet of contrast
    • poststenotic dilatation of ascending aorta
ECHO:
  • increase in echoes from thickened deformed leaflets (maximal during diastole)
  • decrease in leaflet separation
Supravalvular Aortic Stenosis
Types:
  • localized hourglass narrowing just above aortic sinuses
  • discrete fibrous membrane above sinuses of Valsalva
  • diffuse tubular hypoplasia of ascending aorta + branching arteries
Associated with: peripheral PS, valvular + discrete subvalvular AS, Marfan syndrome, Williams syndrome, infantile hypercalcemia syndrome
  • small ascending thoracic aorta
  • dilatation + tortuosity of coronary arteries (may undergo early atherosclerotic degeneration secondary to high pressure)
ECHO:
  • narrowing of supravalvular aortic area (normal root diameter: 20–37 mm)
  • normal movement of cusps
Aortopulmonic Window
  • = defect in septation process characterized by large round/oval communication between left wall of ascending aorta + right wall of pulmonary trunk
  • • clinically resembles PDA
CXR:
  • shunt vascularity
  • cardiomegaly (LA + LV enlarged)
  • diminutive aortic knob
  • prominent pulmonary trunk
Angio (left ventriculogram/aortogram in AP/LAO projection):
  • defect several mm above aortic valve
  • pulmonary valve identified (DDx to truncus arteriosus)
Arteriosclerosis Obliterans
  • = ASO = HARDENING OF THE ARTERIES
Prevalence: 2.4 million people in USA; in 1978 12% of autopsies had ASO as leading cause of death (excluding MI)
Etiology: unknown
Contributing factors:
  • aging, diabetes (16–44%), hypertension, atherosclerosis
Effect of hyperlipidemia:
  • high-density lipoproteins (HDL) have a protective effect: carry 25% of blood cholesterol
  • low-density lipoproteins (LDL): carry 60% of blood cholesterol
Histo: deposition of lipids, blood products, carbohydrates, begins as disruption of intimal surface; fatty streaks (as early as childhood); fibrous plaques (as early as 3rd decade); thrombosis, ulceration, calcification, aneurysm
Age: 50–70 years; M > F (after menopause)
Clinical classification:
  • Intermittent claudication = ischemic symptoms with exercise: calf, thigh, hip, buttock
  • Ischemic symptoms at rest (indicative of multisegment disease)
  • • cramping/burning/aching pain
  • • cold extremity
  • • paresthesia
  • • trophic changes: hair loss, thickened nails
  • • ulcer, gangrene
  • • decreased/absent pulses
Location: medium + large arteries; frequently at bifurcations; most frequent:
  • superficial femoral artery in adductor canal (diabetics + nondiabetics)
  • aortoiliac segment (nondiabetics)
  • tibioperoneal trunk (diabetics)
Prognosis: accelerated by diabetes (34% will require amputation), hypertension, lipoprotein abnormalities, heart disease (decreased cardiac output resulting in increased blood viscosity from polycythemia), chronic addiction to tobacco (11.4% will require amputation), intermittent claudication (5–7% require amputation if nondiabetic = 1–2% per year), ischemic ulcer/rest pain (19.6% require amputation)
P.624

Atrial Septal Defect
  • Most common congenital cardiac defect after bicuspid aortic valve!
Incidence: 8–10–14% of all CHD; M:F = 1:3
Age: presentation frequently > age 40 secondary to benign course
(a) mildly symptomatic (60%): dyspnea, fatigue, palpitations
(b) severely symptomatic (30%): cyanosis, heart failure
Embryology:
  • Septum primum = at 4th week membrane grows from dorsal atrial wall toward endocardial cushions
  • Ostium primum = temporary orifice between septum primum + endocardial cushions close to AV valves; it becomes obliterated by 5th week
  • Ostium secundum = multiple small coalescing fenestrations in center of septum primum
  • Septum secundum = membrane developing on right side of septum primum + covering part of ostium secundum
  • Foramen ovale = orifice limited by septum secundum + septum primum
  • Foramen ovale flap = lower edge of septum primum (patent in 6%, probe-patent in 25%); not considered an ASD
  • OSTIUM SECUNDUM ASD (70%)
    • = exaggerated resorptive process of septum primum leads to absence/fenestration of the foramen ovale flap (Chiari network)
    Location: in the center of the atrial chamber at fossa ovalis
    Size: large defect of 1–3 cm in diameter
    May be associated with:
    • prolapsing mitral valve (20–30%), pulmonary valve stenosis, tricuspid atresia, TAPVR, hypoplastic left heart, interrupted aortic arch
  • OSTIUM PRIMUM ASD (20%)
    • = defect of atrioventricular endocardial cushion
    Location: lower end of septum inferior to fossa ovalis (at outlet portion of atrial septum) adjacent to atrioventricular valves
    Almost always associated with:
    • cleft mitral valve, common atrioventricular canal complex, Down syndrome, anterior fascicular block
  • SINUS VENOSUS ASD (10%)
    • = defect of the superior inlet portion of the atrial septum
    Location: superior to fossa ovalis near entrance of superior vena cava (SVC straddles ASD)
    Associated with: partial anomalous pulmonary venous return in 90% (RUL pulmonary veins connect to SVC/right atrium), Holt-Oram syndrome, Ellis-van Creveld syndrome
  • Coronary Sinus Defect
    • normal coronary sinus typically absent (unroofed), persistent SVC drains directly into LA
  • Lutembacher SYNDROME = ASD + mitral stenosis
    Hemodynamics:
    • no hemodynamic perturbance in the fetus; after birth physiologic increase in LA pressure + greater compliance of RA and RV create a L-to-R shunt (shunt volume may be 3–4 times that of systemic blood flow); volume overload of RV is well tolerated in childhood, leads to RV dilatation, right heart failure; diastolic pressure differences in atria determine direction of shunt; pulmonary pressure remains normal for decades; after 40 years of age onset of pulmonary hypertension causes increased R-to-L shunting (Eisenmenger syndrome); pulmonary hypertension in young adulthood (6%)
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  • • repeated respiratory infections
  • • feeding difficulties
  • • atrial arrhythmias: atrial flutter + atrial fibrillation increases with age
  • • thromboembolism
  • • asymptomatic; occasionally discovered by routine CXR
  • • right ventricular heave
  • • fixed splitting of second heart sound with accentuation of pulmonary component (ejection murmur grade II/VI) heard at 2nd left intercostal space along PA
  • • ECG: right axis deviation + some degree of right bundle branch block
  • • exertional dyspnea after development of pulmonary arterial hypertension (= Eisenmenger syndrome)
  • • cyanosis may occur (shunt reversal to R-to-L shunt), typically during 3rd–4th decade
  • • right heart failure in patients >40 years
CXR:
  • normal (if shunt <2 × systemic blood flow)
  • overcirculation = increase in pulmonary blood flow (if pulmonary-to-systemic blood flow ≥2:1)
  • cardiomegaly:
    • heart small compared with pulmonary vascularity = closing shunt
    • heart large compared with pulmonary vascularity = intercurrent myocardial/aortic disease
  • loss of visualization of SVC (= clockwise rotation of heart due to RV hypertrophy)
  • small appearing aorta with normal aortic knob
  • P.625

  • normal size of LA after shunt reversal (due to immediate decompression into RA) in Eisenmenger syndrome:
    • enlargement of PA + central pulmonary arteries
    • RV enlargement
  • “hilar dance” = increased pulsations of central pulmonary arteries (DDx: other L-to-R shunts)
ECHO:
  • paradoxical interventricular septal motion (due to volume overload of RV)
  • direct visualization of ASD (= lack of echoes of atrial septum) in subcostal view
  • diastolic blood flow from interatrial septum crossing RA + tricuspid valve observed by color Doppler
MR:
  • discrete area of interruption of the normal intermediate-intensity interatrial septum
    CAVE: normal thinning of fossa ovalis can cause drop-out of atrial signal
  • area of signal loss from atrial septum into RA (due to turbulent jet) on GRE
  • ratio of stroke volumes in aorta to PA (measurement of flow volume on cine phase-contrast images)
Angio:
  • RA fills with contrast shortly after LA is opacified (on levophase of pulmonary angio in AP or LAO projection)
  • injection into RUL pulmonary vein to visualize exact size + location of ASD (LAO 45° + C-C 45°)
Prognosis:
  • Mortality: 0.6% in 1st decade; 0.7% in 2nd decade; 2.7% in 3rd decade; 4.5% in 4th decade; 5.4% in 5th decade; 7.5% in 6th decade; median age of death is 37 years
  • Spontaneous closure: 22% in infants <1 year; 33% between ages 1 and 2 years; 3% in children >4 years
Cx: (1) Tricuspid insufficiency (secondary to dilatation of AV ring)
(2) Mitral valve prolapse
(3) Atrial fibrillation (in 20% 1st presenting symptom in patients > age 40)
Rx: (if vascular changes still reversible = resistance of pulmonary-to-systemic system ≤0.7); 1% surgical mortality
1. Surgical patch closure
2. Rashkind foam + stainless steel prosthesis
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Atrial Septal Defects
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Normal Newborn Heart
image
Ostium Secundum Defect
image
Sinus Venosus Defect
image
Ostium Primum Defect
Beneficial ASD
  • = secundum type ASD serves an essential compensatory function in:
  • 1. Tricuspid atresia
    • RA blood reaches pulmonary vessels via ASD + PDA; improvement through Rashkind procedure
  • 2. TAPVR
    • significant shunt volume only available through ASD (VSD/PDA much less reliable)
  • 3. Hypoplastic left heart
    • systemic circulation maintained via RV with oxygenated blood from LA through ASD into RA
Azygos Continuation of IVC
  • = INTERRUPTED IVC WITH AZYGOS/HEMIAZYGOS CONTINUATION = ABSENCE OF THE HEPATIC SEGMENT OF THE IVC WITH AZYGOS CONTINUATION
Prevalence: 0.6%
Etiology: formation failure of right subcardinal-hepatic anastomosis with atrophy of right subcardinal vein + shunting of blood from supracardinal-subcardinal anastomosis to cranial portion of supracardinal vein (= retrocrural azygos vein)
P.626

May be associated with:
  • polysplenia syndrome (more common), asplenia syndrome (rare), indeterminate situs (= situs ambiguus), persistent left SVC, dextrocardia, transposed abdominal viscera, duplicated IVC, retroaortic left renal vein, congenital pulmonary venolobar syndrome
  • absence of hepatic ± infrahepatic IVC:
    • drainage of hepatic veins into right atrium via supra-/posthepatic segment of IVC (N.B.: IVC shadow present on LAT CXR!)
  • drainage of iliac + renal veins via azygos/hemiazygos vein:
    • right renal artery crosses anterior to “IVC” on US
  • both gonadal veins drain into ipsilateral renal vein (since postcardinal-subcardinal anastomosis does not contribute to formation of IVC)
CXR:
  • enlargement of azygos arch to >7 mm
  • widening of right paraspinal stripe contiguous with azygos arch (= enlarged paraspinal + retrocrural azygos veins)
  • widening of left paraspinal stripe (= enlarged hemiazygos vein)
DDx: right-sided paratracheal mass with retrocrural adenopathy
Bacterial Endocarditis
Predisposed:
  • Rheumatic valve disease
  • Mitral valve prolapse with mitral regurgitation
  • Aortic stenosis, mitral stenosis, aortic regurgitation, mitral regurgitation
  • Most CHD (VSD, TOF) except ostium secundum ASD
  • Previous endocarditis
  • Drug addicts:
    • endocarditis of tricuspid valve causes multiple septic pulmonary emboli
  • Bicuspid aortic valve:
    • responsible for 50% of aortic valvular bacterial endocarditis
  • Prosthetic valve:
    • 4% incidence of bacterial endocarditis
    • exaggerated valve motion (= disintegration of suture line + regurgitation)
Valve Vegetations
ECHO:
  • usually discrete focal echodensities with sharp edges; may show fuzzy/shaggy nonuniform thickening of cusps (vegetations) in systole + diastole
  • may appear as shaggy echoes that prolapse when the valve is closed (DDx to mitral valve prolapse)
Buerger Disease
  • = THROMBANGITIS OBLITERANS
  • = idiopathic recurrent segmental obliterative vasculitis of small + medium-sized peripheral arteries + veins (panangitis)
Incidence: <1% of all chronic vascular diseases; more common in Israel, Orient, India
Etiology: unknown
Histo:
  • acute stage: multiple microabscesses within fresh/organizing thrombus; all layers of vessel wall inflamed but intact; internal elastic lamina may be damaged; multinucleated giant cells within microabscesses (PATHOGNOMONIC)
  • subacute stage: thrombus organization with little residual inflammation
  • chronic stage: lumen filled with organized recanalized thrombus, fibrosis of adventitia binds together artery, vein, and nerve
Associated with: cigarette smoking (95%)
  • • instep claudication ± distal ulceration (symptoms abate on cessation of smoking + return on its resumption)
  • • Raynaud phenomenon (33%)
Location: legs (80%), arms (10–20%)
Site: starts in palmar + plantar vessels with proximal progression
  • superficial + deep migratory thrombophlebitis (20–33%)
  • arterial occlusions, tapered narrowing of arteries
  • abundant corkscrew-shaped collaterals
  • direct collateral following the path of the original artery (Martorell sign) in 80%
  • skip lesions = multiple segments involved with portions of arterial wall remaining unaffected
  • absence of generalized arteriosclerosis/arterial calcifications (90%)
Cardiac arrest
  • = sudden cessation of cardiac pump function
CT:
  • layering of static blood within heart + great vessels
  • hematocrit effect = sedimentation of RBCs
  • good depiction of cardiac anatomy (due to cessation of cardiac motion)
CECT:
  • contrast pooling in dependent portion of venous system (right atrium, hepatic veins)
  • no contrast in pulmonary artery + left heart structures
Rx: prompt cardiopulmonary resuscitation
Cardiac Fibroma
  • = FIBROMATOSIS = FIBROUS HAMARTOMA
    • = FIBROELASTIC HAMARTOMA
  • = congenital neoplasm/hamartoma of the heart
Incidence: 100 cases reported; 2nd most common benign cardiac neoplasm of childhood (after rhabdomyoma)
Age: 0–56 years (mean age, 13 years); 33% in children <1 year of age/in utero; 15% in adolescents + adults
Increased prevalence in: Gorlin (= basal cell nevus) syndrome
  • • heart failure, cardiac murmur (33%), arrhythmia
  • • NO embolism; asymptomatic (33%)
Path: 2–10-cm large single round bulging well-circumscribed tumor within ventricular myocardium; foci of calcification/ossification (50%)
Histo: collection of fibroblasts interspersed among large amounts of collagen; numerous elastic fibers (>50%); NO foci of cystic change/hemorrhage/necrosis
Location: ventricular septum > left ventricular free wall
P.627

  • cardiomegaly
  • focal cardiac bulge (with tumor in free ventricular wall)
  • ± pericardial effusion
ECHO:
  • noncontractile echogenic heterogeneous solid mass:
    • mean diameter >5 cm; may obliterate cardiac chamber
    • multifocal dystrophic central tumor calcifications
  • affected myocardium hypokinetic
DDx: focal hypertrophic cardiomyopathy, hypertrophy of ventricular septum
CT:
  • homogeneous mural mass of soft-issue attenuation
  • sharply marginated/infiltrative
  • calcifications (25%)
  • variable enhancement
MR:
  • iso-/hyperintense homogeneous discrete mural mass/myocardial thickening on T1WI
  • hypointense on T2WI
  • no/little hetero- or homogeneous enhancement
Prognosis:
  • sudden death (due to invasion/compression of cardiac conduction system resulting in arrhythmia)
    • 2nd most common primary cardiac tumor associated with sudden death (after endodermal heterotopia of AV node)
  • may remain stable in size for years/regress
Rx: surgical excision/partial resection
DDx in infants: rhabdomyoma (multiple masses)
DDx in children: rhabdomyosarcoma (no calcification, cystic or necrotic tumor, invasion of pulmonary veins or pericardial space)
Cardiac Hemangioma
  • = rare benign vascular tumor of the heart
Prevalence: 5–10% of benign cardiac tumors
Association: Kasabach-Merritt syndrome (multiple systemic hemangiomas, recurrent thrombocytopenia, consumptive coagulopathy)
Path: predominantly intramural spongy mass/well-circumscribed endocardial-based soft mass growing into pericardial space; may contain fat
Histo: capillary (= smaller capillary-like vessels); cavernous (= multiple thin-walled dilated vessels); arteriovenous (= thick-walled dysplastic arteries + veins + capillaries)
  • • asymptomatic
  • • dyspnea on exertion, chest pain, right-sided CHF
  • • arrhythmia, syncope, pericarditis, sudden death
  • ± pericardial effusion
US:
  • hyperechoic mass
CT:
  • heterogeneous intensely enhancing mass
MR:
  • intermediate intensity on T1WI + hyperintense on T2WI
Angio:
  • vascular blush in capillary + arteriovenous type
  • no enhancement for cavernous type
Prognosis: spontaneous regression possible
Rx: surgical resection (for symptomatic lesion)
Cardiac Lipoma
  • = very rare benign neoplasm
Incidence: 60 reported cases
Age: typically in adults
  • mostly symptomatic
  • dyspnea (in intracavitary lipoma secondary to blood flow obstruction, in pericardial lipoma secondary to displacement of lung)
  • arrhythmia (involvement of conduction system)
Path: encapsulated spherical/elliptical solitary mass, often very large (up to 4,800 g) by the time the come to clinical attention; multiple lipomas in CHD, tuberous sclerosis
Histo: mature adipocytes surrounded by capsule
Location:
  • broad-based from epicardial surface growing into pericardial space
  • broad-based from endocardial surface growing into cardiac chamber
  • interatrial septum
  • cardiomegaly, globular-shaped heart
  • echogenic/hypoechoic broad-based nonmobile mass
  • round mass with smooth contour
  • homogeneous mass of ≤ -50 HU in cardiac chamber/pericardial space
  • homogeneous mass of increased signal intensity ± a few thin septations on T1WI:
    • decreasing intensity with fat saturation
  • no enhancement
Rx: surgical resection
DDx: lipomatous hypertrophy of interatrial septum (infiltrative, at level of fossa ovalis with sparing of fossa ovalis, >2 cm thick in transverse dimension, composed of brown fat, not a true neoplasm, associated with advanced age + obesity)
Cardiac Paraganglioma
  • = extremely rare, usually benign sporadic neoplasm arising from intrinsic cardiac sympathetic paraganglial (chromaffin) cells
Incidence: <50 cases
Age: 18-85 (mean, 40) years
  • catecholamine-producing tumor (in the majority):
    • headache, arterial hypertension, palpitations, flushing
    • elevated levels of urinary norepinephrine, vanillylmandelic acid, total metanephrine
    • elevated levels of plasma norepinephrine, epinephrine
Associated with:
  • additional paragangliomas (in 20%) in carotid body, adrenal gland, bladder, paraaortic
  • metastases to bone (in 5%)
Path: 2-14 cm large encapsulated/poorly circumscribed and infiltrative highly vascular mass; necrotic in 60%
Histo: monomorphic tumor composed of nests of paraganglial cells (= “Zellballen”) surrounded by sustentacular cells
Location: posterior wall of left atrium > roof of left atrium > atrial cavity > interatrial septum > ventricle
Site: epicardial surface of the base of the heart with tendency to involve coronary arteries
P.628

CXR:
  • middle mediastinal mass splaying carina simulating left atrial enlargement (for typically located tumor)
ECHO:
  • large echogenic left atrial mass
  • compression of SVC, encasement of coronary aa.
DDx: myxoma (broad base of attachment, softer)
NUC (I-131 or I-123 MIBG):
  • for total body imaging with a sensitivity of 90%
NECT:
  • circumscribed/ill-defined heterogeneous mass:
    • hypoattenuating
    • isoattenuating to cardiac structures (may be missed)
  • ± tumor calcifications
  • ± extracardiac extension
CECT:
  • Premedicate patient with alpha- and beta-blockers as contrast material can trigger a hypertensive crisis!
  • markedly enhancing mass adherent to/involving left atrium/anterior to aortic root
  • central area of low attenuation (in 50%) from necrosis
MR:
  • mass iso-/hypointense to myocardium on T1WI
  • very hyperintense mass on T2WI
  • intense often heterogeneous enhancement
Cardiac Sarcomas
  • Majority of primary malignant cardiac neoplasms!
  • 2nd most common primary cardiac neoplasm
Mean age: 41 years; extremely rare in infants + children
  • right-sided heart inflow obstruction
    • 1. Angiosarcoma (37%): tumor in right atrium
  • mitral valve obstruction (tumor in left atrium)
    • 2. Undifferentiated sarcoma (24%)
    • 3. Malignant fibrous histiocytoma (11–24%)
    • 4. Leiomyosarcoma (8–9%): tends to invade pulmonary veins + mitral valve
      Age: 5–10 years earlier than other sarcomas
    • 5. Primary cardiac osteogenic sarcoma (3–9%)
DDx: myxoma (at fossa ovalis)
  • • dyspnea, pericardial tamponade, arrhythmia, syncope, peripheral edema, sudden death
  • • embolic phenomena, chest pain, pneumonia, fever
  • cardiomegaly
  • CHF
  • pleural effusion, pericardial effusion
  • focal cardiac mass
  • pulmonary consolidation
Metastatic to: lung, lymph nodes, bone, liver, brain, bowel, spleen, adrenal gland, pleura, diaphragm, kidney, thyroid, skin
Prognosis: mean survival of 3 month to 1 year
Angiosarcoma
Frequency: most common cardiac sarcoma
Age: typically in middle-aged men
Path: frequently hemorrhagic + necrotic mass, often adherent to pericardium
Histo: endothelial cells lining ill-defined vascular spaces
  • right-sided heart failure, tamponade
  • fever, weight loss
  • bloody fluid on pericardiocentesis (rarely with malignant cells)
Metastases at presentation: in 66–89%
Location: right atrial free wall + involvement of pericardium (80%)
  • well-defined mass protruding into a cardiac chamber
    • usually originating from right atrium with sparing of atrial septum
    • areas of central necrosis communicating with cardiac chamber
    • low-attenuation mass on CT
    • heterogeneous contrast enhancement
    • heterogeneous MR signal:
      • “cauliflower appearance” = local nodular hyperintense areas interspersed within areas of intermediate signal intensity on T1WI + T2WI
  • diffusely infiltrative mass extending along epicardial surface
    • obliterated pericardial space (hemorrhage + necrotic tumor debris)
    • “sunray appearance” = linear contrast enhancement along vascular lakes on MR
Prognosis: 12–30 months survival
Undifferentiated Sarcoma
  • = PLEOMORPHIC SARCOMA = ROUND CELL SARCOMA = SPINDLE CELL SARCOMA
Age: 45 years (neonates to elderly)
  • pulmonary congestion
Location: left atrium
  • large irregular hypodense intracavitary mass
  • polypoid mass isointense to myocardium
  • thickening/irregularity of myocardium (due to tumor infiltration)
  • tendency to involve valves
  • hemorrhagic mass replacing the pericardium (similar to angiosarcoma)
Cardiac Tamponade
  • = significant compression of heart by fluid contained within pericardial sac resulting in impaired diastolic filling of ventricles
Cause: see PERICARDIAL EFFUSION
  • • tachycardia
  • • pulsus paradoxus = exaggeration of normal pattern = drop in systolic arterial pressure >10 mm Hg during inspiration (secondary to increase in right heart filling during inspiration at the expense of left heart filling)
  • • elevated central venous pressure with distended neck veins (jugular distension)
  • • shortness of breath
  • • falling blood pressure
  • • distant heart sounds/friction rub
  • • ECG: reduced voltage, ST elevation, PR depression, nonspecific T-wave abnormalities
  • normal lung fields + normal pulmonary vascularity
  • rapid enlargement of heart size
  • distension of SVC, IVC, hepatic + renal veins
  • periportal edema
  • P.629

  • hepatomegaly
CT:
  • right + left ventricles of equal size (due to equalization of ventricular pressures)
Doppler-US:
  • episodes of high-velocity hepatopetal flow separated by long intervals of minimal flow
ECHO:
  • diastolic collapse of RV
  • cyclical collapse of either atrium
Rx: pericardiocentesis/pericardial drainage
Cardiac Thrombus
  • Left Atrial Thrombus
    Associated with: mitral valve disease
    • • atrial fibrillation
    Site: atrial appendage
    • atrial dilatation
    • irregular/lobulated border
    • microcavitations
    • laminated appearance
  • Left Ventricular Thrombus
    Site: region of ventricular dyskinesia/aneurysm (from prior myocardial infarction)
    • homogeneous attenuation on CT
    • heterogeneous signal on SE MR images
    • low-signal intensity on GRE MR images
DDx: myxoma (heterogeneous texture on CT)
Cardiomyopathy
  • = heart disease as the result of myocardial dysfunction
Dilated and Ischemic Cardiomyopathy
  • = Congestive Cardiomyopathy
Etiology:
  • Idiopathic
  • Myocarditis: viruses, bacteria
  • Alcoholism
  • Pregnancy/post partum
  • Endocardial fibroelastosis = thickened endocardium + reduced contractility
  • Infants of diabetic mothers
  • Inborn error of metabolism: glycogenosis, mucolipidosis, mucopolysaccharidosis
  • Coronary artery disease: myocardial infarction, anomalous origin of left coronary artery, coronary calcinosis
  • Muscular dystrophies
  • • tendency for CHF when EF <40 %
  • global 4-chamber enlargement
  • poor ventricular contractility (reduced EF)
  • LA enlargement without enlargement of LA appendage
  • bilateral atrioventricular valve insufficiency
ECHO:
  • enlarged LV with global hypokinesis
  • IVS and LVPW of equal thickness with decreased amplitude of motion
  • low-profile/“miniaturized” mitral valve
  • mildly enlarged LA (elevated end-diastolic LV pressure)
  • enlarged hypokinetic right ventricle
Obstructive Cardiomyopathy (10%)
  • = hypertrophic cardiomyopathy
  • = disorder of cardiac muscle characterized by nondilated symmetric/eccentric hypertrophy of either/both ventricles in the absence of cardiac/systemic disease
  • SYMMETRIC/CONCENTRIC HYPERTROPHY (2–20%)
    • = HYPERTENSIVE HYPERTROPHIC CARDIOMYOPATHY
    • • commonly in older women
    • (a) midventricular
    • (b) diffuse
    • (c) apical
    • marked concentric LV hypertrophy
    • small LV cavity
  • ASYMMETRIC SEPTAL HYPERTROPHY (ASH)
    • = IDIOPATHIC HYPERTROPHIC SUBAORTIC STENOSIS (IHSS) = SUBAORTIC STENOSIS
    • = HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY
    • basal/upper part of LV septum disproportionately thickened
    • anterolateral wall of LV often also abnormally thick
    • Most common + clinically significant form!
    • • systolic obstruction of LV outflow tract
  • APICAL HYPERTROPHY (2–33%)
    • = myocardial wall thickening confined to apical portion of LV with sparing of septum
    • • usually clinically benign (no obstruction to LV flow)
    • • giant inverted T-wave
    • Left ventriculography:
      • spade-shaped deformity of LV cavity
Hemodynamics:
  • LV hypertrophy leads to subaortic stenosis, abnormal diastolic function, myocardial ischemia
  • rapid blood flow through narrow outflow tract causes the anterior leaflet of mitral valve to displace anteriorly toward septum during systole (Venturi effect)
  • mitral regurgitation (from displaced MV leaflet)
Etiology: autosomal dominant transmission (>50%); sporadic form
Histo: derangement of myocardial fibers
Age: 3rd–5th decade; occasionally infants + elderly
  • • dyspnea (75%) from elevated LV diastolic pressure
  • • angina (66%) from LV outflow obstruction + decreased flow through intramural coronary arteries
  • • fatigue
  • • syncope from arrhythmia/decreased cardiac output during exercise because of LV outflow obstruction
  • • systolic murmur
  • normal heart size
  • LA enlargement with mitral insufficiency (in 30%)
  • prominent left midheart border (septal hypertrophy)
  • ± mild pulmonary venous hypertension
MR:
  • marked thickening of LV wall + small LV cavity
  • average end-diastolic thickness of septal + posterolateral wall = 23.5 mm + 11.4 mm; ratio 2.1
  • increased LV mass (estimated by cine MRI)
  • large + prolonged signal void from site of obstruction toward aortic valve within normally high-intensity flowing blood (due to turbulent flow during systole)
  • P.630

  • substantially elevated ejection fraction
  • prolonged systolic contact of the anterior mitral valve leaflet with the septum
  • systolic flow void from mitral valve into left atrium due to mitral valve regurgitation (on cine MRI)
  • impairment of LV relaxation (= abnormal LV stiffness) leads to poor early diastolic filling
ECHO (modality of choice):
  • IVS >14 mm thick; posterolateral wall >11 mm thick; IVS:LVPW thickness >1.3:1
  • systolic anterior motion of mitral valve (SAM) causing narrowed LVOT in systole
  • midsystolic closure of aortic valve
  • increased LVOT gradient with late systolic peaking on Doppler
Prognosis: 4% annual mortality rate (sudden death in young patients); progressive LV dilatation, atrial arrhythmia, intractable CHF
Rx: beta-blockers, calcium antagonists, myomectomy of hypertrophied septum, cardiac transplantation
Restrictive Cardiomyopathy
  • = infiltrative process that impairs the myocardial ability to relax normally + impairs ventricular filling
Etiology: (a) idiopathic: endomyocardial fibroelastosis
(a) infiltrative disease: amyloidosis, hemochromatosis, sarcoidosis, glycogen storage disease, Löffler’s hypereosinophilic endocarditis, Gaucher disease
(b) constrictive pericarditis
  • varying degrees of pulmonary venous hypertension
  • dilatation of RA + IVC reflecting high RV filling pressure (DDx: constrictive pericarditis)
  • ± LA enlargement
Chronic Venous Stasis Disease
  • = CHRONIC VENOUS INSUFFICIENCY
  • = insufficiency/incompetence of venous valves in deep venous system of lower extremity
Cause:
  • postphlebitic valvular incompetence: destruction of valve apparatus results in short thickened valves secondary to scar formation
  • primary valvular incompetence: shallow elongated redundant valve cusps prevent effective closure
Associated with: incompetent venous valves in the calf (secondary to pressure dilatation from stasis in deep venous system) leading to superficial vein varicosities
  • • edema, induration (= fluid exudation from increased capillary pressure)
  • • ulceration (from minor trauma + decreased diffusion of oxygen secondary to fibrin deposits around capillaries)
  • • skin hyperpigmentation (= breakdown products of exudated RBCs)
  • • aching pain
  • venous reflux on descending venography with Valsalva:
    • (a) 82% in deep venous system alone
    • (b) 2% in saphenous vein alone
    • (c) 16% in both
    • bilateral in 75%
Grade: 1 = minimal incompetence = to level of upper thigh
2 = mild incompetence = to level of lower thigh
3 = moderate incompetence = to level of knee
4 = severe incompetence = to level of calf veins
image
Coarctation of the Aorta
Coarctation Of Aorta
  • = localized obstruction at the junction of aortic arch and descending aorta secondary to a fibrous ridge protruding into aortic lumen
  • M:F = 4:1; rare in Blacks
  • LOCALIZED COARCTATION
    • = ADULT/postductal/juxtaductal TYPE [former classification]
    • = short discrete narrowing close to ligamentum arteriosum (most common type)
    • Coexistent cardiac anomalies uncommon!
    Location: most frequent in juxtaductal portion of arch
    • • incidental finding late in life
    • •ductus usually closed
    • shelflike lesion at any point along the aortic arch
    • narrow isthmus above the lesion
    • poststenotic aortic dilatation distally
  • TUBULAR HYPOPLASIA
    • = INFANTILE/preductal/DIFFUSE TYPE [former classification]
    • = hypoplasia of long segment of aortic arch after origin of innominate artery
    • Coexistent cardiac anomalies common!
    • • CHF in neonatal period (in 50%)
    • patent ductus arteriosus
Hemodynamics:
fetus : no significant change because only 10% of cardiac output flows through aortic isthmus
neonate : determined by how rapidly the ductus closes; without concurrent VSD overload of LV leads to CHf in 2nd/3rd week of life
Collateral circulation: via subclavian artery and its branches:
  • intercostals
  • anterior spinal artery
  • lateral thoracic
  • internal mammary
  • scapular artery
  • transverse cervical artery
In 50% associated with:
  • Bicuspid aortic valve (in 25-50%), which may result in calcific aortic valve stenosis (after 25 years of age) + bacterial endocarditis
  • P.631

  • Intracardiac malformations: PDA (33%), VSD (15%), aortic stenosis, aortic insufficiency, ASD, tGV, ostium primum defect, truncus arteriosus, double-outlet right ventricle
  • Noncardiac malformations (13%): turner syndrome (13-15%)
  • Cerebral berry aneurysms
  • Mycotic aneurysm distal to CoA
Prognosis: 11% mortality prior to 6 months of age
Rx: ages 3-5 years are ideal time for operation (late enough to avoid restenosis + early enough before irreversible hypertension occurs); surgical correction past 1 year of age decreases operative mortality drastically; 3-11% perioperative mortality
Procedures:
  • Resection + end-to-end anastomosis
  • Patch angioplasty
  • Subclavian flap (Waldhausen procedure) using left subclavian artery as a flap
Postsurgical Cx:
  • Residual coarctation (in 32%)
  • Subsequent obstruction (rare)
  • Mesenteric arteritis: 2-3 days after surgery secondary to paradoxical hypertension from increased plasma renin
    • abdominal pain, loss of bowel control
  • Chronic persistent hypertension symptomatic CoA
Symptomatic CoA
  • Second most common cause of CHf in neonate (after hypoplastic left heart)
Time: (a) toward the end of 1st week of life in “critical stenosis”
(b) more commonly presents in older child
  • • lower extremity cyanosis (in tubular hypoplasia)
  • • left ventricular failure (usually toward end of 1st week of life)
  • generalized cardiomegaly
  • increased pulmonary vascularity (L-to-R shunt through PDA/VSD)
  • pulmonary venous hypertension/edema
  • “figure 3 sign” hidden by thymus
Asymptomatic CoA
  • • headaches (from hypertension)
  • • claudication (from hypoperfusion)
  • “figure 3 sign” = indentation of left lateral margin of aortic arch in the region of aortic-pulmonic window (at site of coarctation and poststenotic dilatation)
  • “reverse 3 sign” on barium esophagogram
  • elevated left ventricular apex (secondary to left ventricular hypertrophy)
  • scalloped contouring of soft-tissues posterior to sternum (= dilated tortuous internal mammary arteries) on LAT CXR (in 28%)
  • dilatation of brachiocephalic vessels + aorta proximal to stenosis
  • obscuration of superior margin of aortic arch
  • inferior rib notching (in 75%; mostly in adults over age 20; unusual before age 6)
Location: ribs 3–9 (most pronounced in 3rd + 4th ribs, less pronounced in lower ribs); 1st + 2nd rib do not participate because they have arteries originating from subclavian a.
Site: central + lateral thirds of posterior rib
(a) bilateral
(b) unilateral on left side: left aortic arch with aberrant right subclavian artery below CoA
(c) unilateral on right side: right aortic arch with anomalous left subclavian artery below CoA
Congenital Absence Of Pulmonary Valve
Massive regurgitation between pulmonary artery and RV
In 90% associated with: VSD, tetralogy of Fallot (50%)
  • • cyanosis (not in immediate newborn period)
  • • repeated episodes of respiratory distress
  • • continuous murmur
  • • ECG: right ventricular hypertrophy
  • prominent main, right, and left pulmonary artery
  • RV dilatation (increased stroke volume)
  • partial obstruction of right/left mainstem bronchus (compression by vessel)
  • right-sided aorta (33%)
Congestive Heart Failure
  • = increase in circulating blood volume with diminishing cardiac function leads to elevation of microvascular pressure of lung
Incidence: most common cause of interstitial + airspace edema of lungs
Cause:
  • back pressure from LV: long-standing systemic hypertension, aortic valve disease, coronary artery disease, cardiomyopathy, myocardial infarction
  • obstruction proximal to LV: mitral valve disease, LA myxoma, cor triatriatum
Histo:
  • Interstitial phase: fluid in loose connective tissue around conducting airways and vessels + engorgement of lymphatics
  • Alveolar phase: increase in alveolar wall thickness
  • Alveolar airspace phase: alveoli filled with fluid + loss of alveolar volume; pulmonary fibrosis upon organization of intraalveolar fibrin (if chronic)
  • large heart
  • Interstitial pulmonary edema (invariably precedes alveolar edema)
    • •NO abnormal physical finding
    • • hypoxemia (ventilation-perfusion inequality)
    • loss of sharp definition of vascular markings
    • thickening of interlobular septa (pulmonary venous wedge pressure 17–20 mm Hg)
    • poorly defined increased bronchial wall thickness
    • thickening of interlobar fissures (due to fluid in subpleural connective tissue layer)
  • Airspace edema
    Cause: acute LA pressure elevation with volume of capillary filtration exceeding that of lymphatic drainage
    • • severe dyspnea/orthopnea
    • • tachypnea + cyanosis
    • P.632

    • • dry cough/copious frothy sputum
    • •hypoxemia (vascular shunting)
    • poorly defined patchy acinar opacities
    • coalescence of acinar consolidation, particularly in medial third of lung
    • butterfly/bat-wing distribution of consolidation
    • (= consolidated hilum + uninvolved lung cortex)
  • Flow inversion
    Cause: chronic elevation of LA pressure (as in left heart failure/mitral valve disease)
    Pathophysiology:
    • long-standing elevation of LA pressure causes an increase in atriovenous reflux; initially the increased LA pressure is met with an increased tonus of the LA wall (= absence of atrial enlargement in acute left heart failure); eventually LA enlarges inciting a protective atrial-pulmonary-vascular reflex vasospasm, which narrows the lower lobe vessels and decreases atriovenous reflux
    • basal oligemia
    • hyperemia of upper lobes
    N.B.: flow inversion is never seen in pulmonary edema of renal failure/overhydration/low oncotic pressure
  • Generalized oligemia
    Cause: aortic valvular disease
Extrathoracic Manifestations of CHF
  • @ Hepatobiliary
    • GB wall edema
    • periportal edema
    • enlarged IVC
Constrictive Pericarditis
  • = PERICARDIAL CONSTRICTION
  • = fibrous thickening of pericardium interfering with filling of ventricular chambers through restriction of heart motion
Age: 30–50 years; M:F = 3:1
  • • elevation + equilibration of end-diastolic ventricular pressures
Etiology:
  • IDIOPATHIC (2nd most common today)
  • INFECTIOUS/INFLAMMATORY
    • Viral (Coxsackie B)
    • Tuberculosis (formerly most common etiology)
    • Rheumatoid arthritis
  • TRAUMATIC
    • Cardiac surgery (most common)
    • Radiotherapy to mediastinum
  • UREMIA = chronic renal failure
  • NEOPLASTIC = tumor invasion
Causes of acute pericarditis:
mnemonic: MUSIC
  • Myocardial infarction (acute)
  • Uremia
  • Surgery (cardiac)
  • Infection
  • Cancer
  • • dyspnea + weakness
  • • abdominal enlargement (ascites + hepatomegaly)
  • • peripheral edema
  • • pericardial knock sound = loud early-diastolic sound
  • • neck vein distension
  • Kussmaul sign = failure of venous pressure to fall (= elevation of jugular venous pressure) with inspiration
  • • prominent X and Y descent on venous pressure curve
  • linear/plaquelike pericardial calcifications (50–70%): predominantly over RV, posterior surface of LV, in atrioventricular groove
  • dilatation of SVC (77%), azygos vein (69%)
  • small atria; occasionally compensatory dilatation of nonconstricted portions, eg, LA enlargement (20%)
  • normal/small-sized heart (enlargement only due to preexisting disease)
  • normal pulmonary vascularity/pulmonary venous hypertension (43%)
  • straightening of heart borders:
    • straight/concave on right side
    • squared on left side
    • pericardial tenting
  • increase in ejection fraction (small EDV)
  • pleural effusion (34% bilateral, 26% right PE)
CT:
  • epicardium = visceral pericardium >2 mm thick
  • dilatation of SVC + IVC
  • reflux of contrast into coronary sinus
  • flattening of right ventricle + curvature of interventricular septum toward left
  • pleural effusion + ascites
MR:
  • pericardium thickened to ≥4 mm
  • pericardium of intermediate signal intensity similar to myocardium sandwiched between high-signal epicardial and mediastinal fat (on T1WI); most easily identified anterior to RV > RA > free wall of LV
  • often small LV + tubular appearing RV
  • dilatation of RA + IVC reflecting high RV filling pressure (DDx: restrictive cardiomyopathy)
  • flattened/sigmoid-shaped septum
ECHO (nonspecific features):
  • thickening of pericardium (not reliably demonstrated unless pericardial fluid present)
  • immobile pericardium
  • rapid early filling of LV
  • rapid early filling motion followed by flat posterior wall motion during diastasis period (= period between early rapid filling and atrial contraction)
  • premature opening of pulmonic valve
Cx: protein-losing enteropathy (increased pressure in IVC + portal vein)
Rx: surgical stripping of pericardium
DDx: (a) restrictive physiology:
1. Cardiac tamponade
2. Restrictive cardiomyopathy (eg, amyloid)
(b) nonrestrictive physiology:
1. S/P recent cardiac surgery
2. Organized intrapericardial hematoma
Coronary Artery Fistula
  • = single/multiple fistulous connections between a coronary artery (R > L) and other heart structures
Abnormal communication with (>90 % right heart):
  • RV > RA > pulmonary trunk > coronary sinus > SVC
P.633

Hemodynamics: L-to-R shunt; pulmonary:systemic blood flow = <1.5:1 (usually)
  • may have normal CXR (in small shunts)
  • cardiomegaly + shunt vascularity (in large shunts)
Angio:
  • dilated tortuous coronary a. with anomalous connection
Cor Triatriatum
  • = rare congenital anomaly in which a fibromuscular septum with a single stenotic/fenestrated/large opening separates the embryologic common pulmonary vein from the left atrium:
    • proximal/accessory chamber lies posteriorly receiving pulmonary veins
    • distal/true atrial chamber lies anteriorly connected to left atrial appendage + emptying into LV through mitral valve
Etiology: failure of common pulmonary vein to incorporate normally into left atrium
Associated with: ASD, PDA, anomalous pulmonary venous drainage, left SVC, VSD, tetralogy of Fallot, atrioventricular canal
  • • dyspnea, heart failure, failure to thrive
  • • clinically similar to mitral valve stenosis
  • pulmonary venous distention + interstitial edema + dilatation of pulmonary trunk and pulmonary arteries (in severe obstruction)
  • enlarged RA + RV
  • mild enlargement of LA
Angio:
  • dividing membrane on levophase of pulmonary arteriogram
Prognosis (if untreated):
  • usually fatal within first 2 years of life; 50% 2-year survival; 20% 20-year survival
Rx: surgical excision of obstructing membrane
Deep Vein Thrombosis
  • =DVT
Incidence: 140,000–250,000 new cases per year in United States with an estimated sole/major cause of 50,000–200,000 deaths per year (15% of in-hospital deaths); 6–7 million stasis skin changes; in 0.5% cause of skin ulcers
Pathogenetic factors:
  • Hypercoagulability
  • Decreased blood flow/stasis
  • Intimal injury
  • Decreased fibrinolytic potential of veins
  • Platelet aggregation
Risk factors:
  • Surgery, esp. on legs/pelvis: orthopedic (45–50%) especially total hip replacement >50%), gynecologic (7–35%), neurosurgery (18–20%), urologic (15–35%), general surgery (20–25%)
  • Severe trauma
  • Prolonged immobilization: hemiplegic extremity, paraplegia + quadriplegia, casting/orthopedic appliances
  • Malignancy (risk factor 2.5) = Trousseau syndrome
  • Obesity (risk factor 1.5)
  • Diabetes
  • Pregnancy (risk factor 5.5) and for 8–12 weeks postpartum
  • Medication: birth control pills, estrogen replacement, tamoxifen (risk factor 3.2)
  • Decreased cardiac function: congestive heart failure, myocardial infarction (20–50%; risk factor 3.5)
  • Age >40 years (risk factor 2.2)
  • Varicose veins
  • Previous DVT (risk factor 2.5)
  • Patients with blood group A > blood group 0
  • Polycythemia
  • Smoking
Pathologic terminology:
  • “organized thrombus” = transition to a vascularized lesion of connective tissue adherent to vessel wall
  • “recanalized thrombus” = vascular channel network within an organized clot reducing it to septations of collagen and elastic fibers often lined by endothelium
Location:
  • Dorsal veins of calf (± ascending thrombosis)
  • Iliofemoral veins (± descending thrombosis)
  • Peripheral + iliofemoral veins simultaneously
  • rare: internal iliac v., ovarian v., ascending lumbar vv.
Side: L:R = 7:3 due to compression of left common iliac v. by left common iliac a. (arterial pulsations lead to chronic endothelial injury with formation of intraluminal spur, which is present in 22% of autopsies + in 90% of patients with DVT)
  • • Local symptoms due to obstruction/phlebitis usually only when (a) thrombus occlusive, (b) clot extends into popliteal/more proximal vein (14–78% sensitivity, 4–21% specificity):
    • • warmth
    • • swelling (measurement of circumference)
    • • blanching of skin (phlegmasia dolens alba)/blue leg with complete obstruction (phlegmasia cerulea dolens)
    • • deep crampy pain in affected extremity, worse in erect position, improved while walking
    • • tenderness along course of affected vein
    • • Homans sign = calf pain with dorsal flexion of foot
    • • Payr sign = pain upon compression of sole of foot
    • 2/3 of deep vein thromboses are clinically silent:
      • DVT diagnosed ante mortem in <30%
      • Only 10–33% of patients with fatal PE are symptomatic for DVT
    • Clinically suspected DVT accurate in only 26–45%:
      • DVt symptomatology due to other causes in 15-35% of patients
    • Negative bilateral venograms in 30% of patients with angiographically detected pulmonary emboli (big bang theory = clot embolizes in toto to the lung leaving no residual in vein)
Venography (89% sensitivity, 97% specificity):
  • false negative in 11%, false positive in 5%; study aborted/nondiagnostic in 5%
Risk: postvenography phlebitis (1–2%), contrast reaction, contrast material-induced skin slough, nephropathy
  • intraluminal filling defect constant on all images
  • nonfilling of calf veins
  • inadequate filling of common femoral vein + external + common iliac veins
P.634

B-Mode US (88-100% sensitivity, 92-100% specificity, >90% accuracy for DVt in thigh and popliteal veins):
  • lack of complete luminal collapse with venous compression (DDx: deformity + scarring from prior DVt; technical difficulties in adductor canal + distal deep femoral vein)
  • visualization of clot within vein (DDx: slow flowing blood; machine noise)
  • <75% increase in diameter of common femoral vein during Valsalva
  • venous diameter at least twice that of adjacent artery suggests thrombus <10 days old
Doppler US:
  • absence of spontaneity (= any waveform recording), not reliable in peripheral veins
  • continuous venous signal = absence of phasicity (= no cyclic variation in flow velocity with respiration, ie, decrease in expiration + increase in inspiration) is suspicious for proximal obstruction
  • attenuation/absence of augmentation (= no increase in flow velocity with distal compression) indicates venous occlusion/compression in intervening venous segments
  • pulsatile venous flow is a sign of congestive heart failure/pericardial effusion/cardiac tamponade/pulmonary embolism with pulmonary hypertension
Venous Occlusion Plethysmography:
  • = temporary obstruction of venous outflow by pneumatic cuff around mid-thigh inflated above venous pressure leads to progressive increase in blood volume in lower leg; upon release of cuff limb quickly returns to resting volume with prompt venous runoff; limb blood volume changes are measured by impedance plethysmography in which a weak alternating current is passed through the leg; the electrical resistance varies inversely with blood volume; the current strength is held constant and voltage changes directly reflect blood volume changes
  • — 87-95-100% sensitivity, 92-100% specificity for aboveknee DVt
  • — 17-33% sensitivity for below-knee DVt
  • initial rise in venous volume (= venous capacitance) diminished
  • delay in venous outflow = “fall” measured at 3 seconds
False positives (6%): severe cardiopulmonary disease, pelvic mass, reduced arterial inflow
False negatives: calf vein thrombosis, small thrombus
I-125-Labeled fibrinogen:
  • 90% sensitive for calf vein thrombus
  • 60–80% sensitive for femoral vein thrombus
  • insensitive for thrombus in upper thigh/pelvis
Risk: results not available for several days, transmission of viral infection
False positives: hematoma, inflammation, wound, old small thrombus isolated in common femoral/iliac vein
Cx:
  • Pulmonary embolism (50%): in 90% from lower extremity/pelvis; in 60% with proximal “free-floating”/“widow-maker” thrombus; occurs usually between 2nd to 4th (7th) day of thrombosis
    • Source of pulmonary emboli:
      • multiple sites (1/3), cryptogenic in 50%;
      (a) lower extremity (46%)
      (b) inferior vena cava (19%)
      (c) pelvic veins (16%)
      (d) mural heart thrombus (4.5%)
      (e) upper extremity (2%)
    • Likelihood of pulmonary embolism:
      • 77% for iliac veins, 35–67% for femoropopliteal vein, 0–46% for calf veins
  • Postphlebitic syndrome (PPS) in 20% of cases with DVT (= recanalization to a smaller lumen, focal wall changes) due to valvular incompetence
  • Phlegmasia cerulea/alba dolens (= severely impaired venous drainage resulting in gangrene)
Prognosis: tibial/peroneal venous thrombi resolve spontaneously in 40%, stabilize in 40%, propagate into popliteal vein in 20%
Prophylaxis: intermittent compression of legs, heparin, warfarin
Rx:
  • Heparin IV
  • Systemic anticoagulation (warfarin) for ≥3 months decreases risk of recurrent DVT in initial 3 months from 50% to 3% + fatal pulmonary embolism from 30% to 8%; necessity for anticoagulation in DVT of calf veins is controversial
  • Caval filter (10–15%) in patients with contraindication/complication from anticoagulation or progression of DVT/PE despite adequate anticoagulation
DDx: pseudothrombophlebitis (= signs + symptoms of DVT produced by popliteal cyst/traumatic hematoma)
Double-Outlet Right Ventricle
  • = DORV = TAUSSIG-BING HEART
  • = most of the aorta + pulmonary artery arise from the RV secondary to maldevelopment of conotruncus
Type 1 = aorta posterior to pulmonary artery + spiraling course (most frequent)
Type 2 = Taussig-Bing heart = aorta posterior to pulmonary artery + parallel course
Type 3 = aorta anterior to pulmonary artery + parallel course
Hemodynamics:
fetus : no CHF in utero (in absence of obstructing other anomalies)
neonate : ventricular work overload leads to CHF
Associated with: VSD (100%), pulmonary stenosis (50%), PDA
  • aorta overriding the interventricular septum with predominant connection to RV
  • aorta posterior/parallel/anterior to pulmonary artery
  • LV enlargement (volume overload)
Ductus Arteriosus Aneurysm
  • = fusiform aneurysm of ductus arteriosus, usually patent toward aorta + completely/incompletely occluded toward pulmonary artery
Incidence: <100 cases
Classification:
  • according to age: infantile, childhood, adult type
  • according to cause: congenital, infectious, traumatic
P.635

Pathogenesis: ? delay in closure,? myxoid degeneration of ductus wall,? abnormal elastic fibers
Age: most <2 months of age
  • • dyspnea, tachypnea, hoarseness
  • pulmonary artery displaced anteromedially
  • distal aortic arch displaced laterally
CXR:
  • left-sided upper mediastinal mass in aorticopulmonary window
  • tracheal displacement to right + anteriorly/posteriorly
  • consolidation of adjacent lung (compression, fibrosis, hemorrhage)
CT:
  • contrast-enhancing mass in classic location
ECHO:
  • cystic mass with pulsatile flow
Cx: rupture, dissection, infection, thromboembolic disease, phrenic nerve compression
Prognosis: usually fatal (without prompt surgery)
Ebstein Anomaly
  • = downward displacement of septal + posterior leaflets of dysplastic tricuspid valve with ventricular division into
    • a large superior atrialized inflow portion with thin ventricular wall (incorporating part of the RV into the RA), and
    • a small inferior functional chamber with shortened chordae tendineae
Valve morphology:
  • inappropriately low attachment of posterior ± septal malformed defective valve leaflets; only anterior leaflet attaches normal to tricuspid annulus, but it can be dysplastic/sail-like
Etiology: chronic maternal lithium intake (10%)
Hemodynamics:
  • tricuspid valve insufficiency leads to tricuspid regurgitation (“ping-pong” volume) + severely dilated RA; RA dilatation stretches interatrial septum causing incompetence of foramen ovale (R-to-L shunt) in 75%
image
  • ± cyanosis in neonatal period (depending on degree of R-to-L shunt): may improve/disappear postnatally with decrease in pulmonary arterial pressure
  • CHF in utero/in neonate (in 50%)
  • systolic murmur (tricuspid insufficiency)
  • Wolff-Parkinson-White syndrome (10%) = paroxysmal supraventricular tachycardia/right bundle branch block (responsible for sudden death)
Cause: conduction system develops during formation of tricuspid valve adjacent to it
  • “boxlike/funnel-like” cardiomegaly (enlargement of RA + RV)
  • extreme RA enlargement (secondary to insufficient tricuspid valve)
  • IVC + azygos dilatation (secondary to tricuspid regurgitation)
  • hypoplastic aorta + pulmonary trunk (the ONLY cyanotic CHD to have this feature)
  • normal LA
  • calcification of tricuspid valve may occur
MR:
  • marked right atrial enlargement
  • small right ventricle ± dilatation of RV infundibulum
ECHO:
  • large “sail-like” tricuspid valve structure within dilated right heart
  • tricuspid regurgitation identified by Doppler ultrasound
Prognosis: 50% infant mortality; 13% operative mortality
Survival rate: 70% at 2 years, 50% at 13 years; survival into adulthood if valve functions normally
Rx: 1. Digitalis + diuretics
2. Tricuspid valve prosthesis
Eisenmenger Complex
  • = EISENMENGER DEFECT
  • (1) high VSD ± overriding aorta with hypoplastic crista supraventricularis
  • (2) RV hypertrophy
    • and as consequence of increased pulmonary blood flow:
  • (3) dilatation of pulmonary artery + branches
  • (4) intimal thickening + sclerosis of small pulmonary arteries + arterioles
  • • cyanosis appears in 2nd + 3rd decade with shunt reversal
Eisenmenger Syndrome
  • = EISENMENGER REACTION
  • = development of high pulmonary vascular resistance after many years of increased pulmonary blood flow secondary to L-to-R shunt (ASD, PDA, VSD), which leads to a bidirectional (= balanced) shunt and ultimately to R-to-L shunt
Etiology:
  • pulmonary microscopic vessels undergo reactive muscular hypertrophy, endothelial thickening, in situ thrombosis, tortuosity + obliteration; once initiated, pulmonary hypertension accelerates the vascular reaction, thus increasing pulmonary hypertension in a vicious cycle with RV failure + death
Path: adaptive anastomotic pathways connect plexiform lesions of pulmonary arterial vessels to bronchial arteries supplying terminal bronchioles + vasa vasorum of pulmonary arteries
Pathologic classification of severity (Heath & Edwards):
Grade I = medial hypertrophy of muscular pulmonary arteries and arterioles
•potentially reversible
Grade II = grade I + intimal proliferation in small muscular arteries and arterioles
• potentially reversible
Grade III = grade II + intimal laminar fibrosis + progressive vessel obliteration
•borderline for reversibility
Grade IV = occlusion of vessels with progressive aneurysmal dilatation of small arteries nearby
• irreversible
Grade V = tortuous “glomeruloid” channels within proliferation of endothelial cells (= plexiform + angiomatoid lesions)
• irreversible
Grade VI = thrombosis + necrotizing arteritis
•irreversible
P.636

CXR:
  • pronounced dilatation of central pulmonary arteries (pulmonary trunk, main pulmonary artery, intermediate branches)
  • pruning of peripheral pulmonary arteries
  • enlargement of RV + RA (proportionate to volume overload)
  • LA + LV return to normal size (with decrease of L-to-R shunt due to markedly elevated pulmonary vascular resistance)
  • normal pulmonary veins (unless superimposed cardiac volume overload):
    • pulmonary veins NOT distended (NO increase in pulmonary blood flow)
    • NO redistribution of pulmonary veins (normal venous pressure)
CT:
  • linear calcification + thrombus in central pulmonary arteries
  • mural calcification/aneurysmal dilatation of ductus arteriosus (in cases of patent ductus arteriosus)
Dx: measurement of pulmonary artery pressure + flow via catheter
Endocardial Cushion Defect
  • =ECD = ATRIOVENTRICULAR SEPTAL DEFECT
    • = PERSISTENT OSTIUM atrioventriculare COMMUNE = PERSISTENT COMMON ATRIOVENTRICULAR CANAL
  • = persistence of primitive atrioventricular canal + anomalies of AV valves
  • INCOMPLETE/PARTIAL ECD
    • = (1) Ostium primum ASD
    • (2) Cleft in anterior mitral valve leaflet/trileaflet
    • (3) Accessory short chordae tendineae arising from anterior MV leaflet insert directly into crest of deficient ventricular septum
    • left atrioventricular valve usually has 3 leaflets with a wide cleft between anterior + septal leaflet
    • “gooseneck” deformity secondary to downward attachment of anterior MV leaflet close to inter-ventricular septum by accessory chordae tendineae
    • communication between LA–RA or LV–RA, occasionally LV–RV
    • right atrioventricular valve usually normal
  • TRANSITIONAL/INTERMEDIATE ATRIOVENTRICULAR CANAL (uncommon)
    • = (1) Ostium primum ASD
    • (2) High membranous VSD
    • (3) Wide clefts in septal leaflets of both AV valves
    • (4) Bridging tissue between anterior + posterior common leaflet of both AV valves
  • COMPLETE ECD = AV COMMUNIS = COMMON AV CANAL
    • = (1) Ostium primum ASD above
    • (2) Posterior inlet VSD below
    • (3) One AV valve common to RV + LV with 5–6 leaflets
      • anterior common “bridging” leaflet
      • two lateral leaflets
      • posterior common “bridging” leaflet
Type 1 = chordae tendineae of anterior bridging leaflet attached to both sides of ventricular septum
Type 2 = chordae tendineae of anterior leaflet attached medially to anomalous papillary muscle within RV, but unattached to septum
Type 3 = free-floating anterior leaflet with chordae attachments to septum; only type becoming symptomatic in infancy!
Associated with:
  • Down syndrome:
    • in 25% of trisomy 21 an ECD is present;
    • in 45% of ECD trisomy 21 is present
  • Asplenia, polysplenia
    • common atrioventricular orifice
    • oval septal defect consisting of a low ASD + high VSD
    • atrial septum secundum usually spared (“common atrium” if absent)
    • frequently associated with mesocardia/dextrocardia
Hemodynamics:
fetus: atrioventricular valves frequently incompetent leading to regurgitation + CHF
neonate: L-to-R shunt after decrease of pulmonary vascular resistance resulting in pulmonary hypertension
  • incomplete right bundle branch block (distortion of conduction tissue)
  • left-anterior hemiblock
CXR:
  • Radiographic findings similar to ASD, but more marked
  • increased pulmonary vascularity (= shunt vascularity)
  • redistribution of pulmonary blood flow (mitral regurgitation)
  • enlarged pulmonary artery
  • diminutive aorta (secondary to L-to-R shunt)
  • cardiac enlargement out of proportion to pulmonary vascularity (L-to-R shunt + mitral insufficiency)
  • enlarged RV + LV
  • enlarged RA (LV blood shunted to RA)
  • normal-sized LA (secondary to ASD)
ECHO:
  • visualization of ASD + VSD + valve + site of insertion of chordae tendineae
  • paradoxical anterior septal motion (secondary to ASD)
  • atrioventricular insufficiency + shunts identified by Doppler ultrasound
Angio:
  • AP projection:
    • gooseneck deformity of LVOT (in diastole)
    • cleft in anterior leaflet of mitral valve (in systole)
    • mitral regurgitation
  • Hepatoclavicular projection in 45° LAO + C-C 45°(= 4-chamber view):
    • best view to demonstrate LV-RA shunt
    • best view to demonstrate VSD (inflow tract + posterior portion of interventricular septum in profile)
  • LAT projection:
    • irregular appearance of superior segment of anterior mitral valve leaflet over LVOT
Prognosis: 54% survival rate at 6 months, 35% at 12 months, 15% at 24 months, 4% at 5 years; 91% long-term survival with primary intracardiac repair, 4–17% operative mortality
P.637

Endocardial Fibroelastosis
  • = diffuse endocardial thickening of LV + LA from deposition of collagen + elastic tissue
Etiology:
  • ? viral infection
  • Secondary endocardial fibroelastosis
    • = subendocardial ischemia in critical LVOT obstruction: aortic stenosis, coarctation, hypoplastic left heart syndrome
  • • sudden onset of CHF during first 6 months of life
  • mitral insufficiency:
    • involvement of valve leaflets
    • shortening + thickening of chordae tendineae
    • distortion + fixation of papillary muscles
  • enlarged LV = dilatation of hypertrophied LV from mitral regurgitation
  • restricted LV motion
  • enlarged LA
  • pulmonary venous congestion + pulmonary edema
  • LLL atelectasis (= compression of left lower lobe bronchus by enlarged LA)
Prognosis: mortality almost 100% by 2 years of age
Fibromuscular Dysplasia
  • = nonatherosclerotic angiopathy of unknown pathogenesis caused by proliferation of muscular + fibrous elements in middle- and large-caliber arteries
Incidence: <1% of cerebral angiographies; 1,100 patients reported (by 1982)
Age: children + young adults <30–40 years; 2/3 >50 years; M:F = 1:3 to 1:4
  • • hypertension
  • • progressive renal insufficiency
  • • decreased peripheral pulses, bruit
  • • asymmetric limb pressures
  • • neurologic deficits
Location:
  • @ Cephalic arteries:
    • cervical + intracranial ICA (85%), extracranial carotid artery (30%), vertebral artery (7%); both anterior + posterior circulation (8%); bilateral (60–65%)
    Associated with: brain ischemia (up to 50%), intracranial aneurysms (up to 30%), intracranial tumors (30%), bruits, trauma
  • @ Abdominal aorta:
    • renal artery (60%), other aortic branches (in 1–2%: celiac a., hepatic a., splenic a., mesenteric a., iliac a.)
    • Simultaneous involvement of renal/muscular arteries in 3%
  • 1. INTIMAL FIBROPLASIA (1–2%)
    • = intimal hyperplasia
    • • progressive
    Path: circumferential/eccentric fibrous tissue between intima + internal elastic lamina
    Age: children + young adults; M:F = 1:1
    Site: main renal artery + major segmental branches; often bilateral
    • focal narrow annular radiolucent band
    • smooth tubular stenosis
    • poststenotic fusiform dilatation
    Cx: spontaneous dissection
    DDx: atherosclerosis, Takayasu arteritis
  • 2. MEDIAL FIBROPLASIA (60–85%)
    • = fibromuscular hyperplasia = medial fibroplasia with microaneurysm
    Age: 20–50 years; typically affects women; common cause of renal artery stenosis in children
    Path: multiple fibromuscular ridges + severe mural thinning with loss of smooth muscle + internal elastic lamina
    Site: mid + distal renal artery + branches; bilateral in 50%
    • “string-of-beads” sign = alternating areas of weblike stenoses + aneurysms (which exceed the normal diameter of the artery)
    • single tubular focal stenosis
    Cx: dissection
  • 3. perimedial FIBROPLASIA (rare)
    • = subadventitial fibroplasia
    Age: young females
    Path: fibroplasia of outer 1/2 of media replacing external elastic lamina
    Site: distal (mostly right) main renal artery
    • long irregular stenosis
    • beading = NO aneurysm formation (diameter of beads not wider than normal diameter of artery)
  • 4. MEDIAL HYPERPLASIA (5–15%)
    • = FIBROMUSCULAR HYPERPLASIA
    Path: smooth muscle + fibrous tissue hyperplasia within arterial media
    Site: main renal artery and branches
    • long smooth concentric tubular narrowing
    DDx: Takayasu arteritis, sclerosing arteritis, vessel spasm, arterial hypoplasia
  • 5. ADVENTITIAL FIBROPLASIA (<1%)
    • = subadventitial/Periadventitial hyperplasia
    Path: adventitial + periarterial proliferation in fibrofatty tissue
    Site: main renal artery, large branches
    • long segmental stenosis
    • added to original 5 types:
  • 6. MEDIAL DISSECTION (5–10%)
    Path: new channel in outer 1/3 of media within external elastic lamina
    Site: main renal artery + branches
    • false channel, aneurysm
  • 7. ATYPICAL FIBROMUSCULAR DYSPLASIA
    • (=? variant of intimal fibroplasia)
    • web = smooth/corrugated mass involving only one wall of vessel + projecting into lumen
    DDx: atherosclerotic disease, posttraumatic aneurysm
    VARIANT: segmental mediolytic arteriopathy
    • = rare noninflammatory disease of small + medium arteries
    Histo: focal segmental disruption of medial smooth muscle cells with mediolysis
    • string-of-beads appearance
    • irregular stenoses + aneurysms
Cx: dissection (in 3%), macroaneurysm formation, intramural hemorrhage
P.638

Prognosis: tends to remain stable/minimal progression of lesions in 20% causing decline in renal function
Rx: (1) Resection of diseased segment with end-to-end anastomosis
(2) Replacement by autogenous vein graft, excision + repair by patch angioplasty
(3) transluminal balloon angioplasty (90% success rate with very low restenosis rate)
Flail Mitral Valve
Cause:
  • ruptured chordae tendineae in rheumatic heart disease, ischemic heart disease, bacterial endocarditis
  • rupture of head of papillary muscle in acute myocardial infarction, chest trauma
Location: chordae to leaflet from posteromedial papillary muscle (single vessel blood supply)
  • deep holosystolic posterior movement
  • random anarchic motion pattern of flail parts in diastole
  • excessively large amplitude of opening of aML
HeterOtaxy Syndrome
[hetero, Greek = different; taxis, Greek = arrangement]
  • = CARDIOSPLENIC SYNDROMES
  • = situs ambiguus with a spectrum of various congenital truncal abnormalities + frequently cardiac malformations from asplenia to polysplenia
Embryology:
  • primary defect in lateralization with disruption of complete separation of cardiac chambers during 20–30 days of gestation
Inheritance: multifactorial (autosomal dominant, autosomal recessive, X-linked recessive)
Individualized approach of classification:
  • describes all critical structures by analyzing
  • position of atria
  • position of venous drainage below diaphragm relative to midline
  • position of aorta relative to midline
  • position of the stomach + presence of malrotation
  • position of liver + gallbladder
  • position of cardiac apex
  • presence, appearance, and number of spleens
  • presence of bi-/trilobed lungs
Asplenia syndrome
  • = BILATERAL RIGHT-SIDEDNESS = RIGHT ISOMERISM = IVEMARK SYNDROME
Incidence: 1:1,750–1:40,000 livebirths; M >
Associated with:
  • CHD (in 50%):
    • TAPVR (almost 100%), endocardial cushion defect (85%), single ventricle (51%), TGA (58%), pulmonary stenosis/atresia (70%), dextrocardia (42%), mesocardia, VSD, ASD, absent coronary sinus, common atrium, common hepatic vein
  • GI anomalies:
    • Partial/total situs inversus, annular pancreas, agenesis of gallbladder, ectopic liver, esophageal varices, duplication + hypoplasia of stomach, Hirschsprung disease, hindgut duplication, imperforate anus
  • GU anomalies (15%):
    • Horseshoe kidney, double collecting system, hydroureter, cystic kidney, fused/horseshoe adrenal, absent left adrenal, bilobed urinary bladder, bicornuate uterus
  • Cleft lip/palate, scoliosis, single umbilical artery, lumbar myelomeningocele
  • • cyanosis in neonatal period/infancy (if severe cyanotic CHD)
  • • severe respiratory distress
  • • Howell-Jolly bodies = RBC inclusions in patients with absent spleen
  • cardiac apex discordant from stomach + liver
  • absent spleen (risk of sepsis)
  • @ Lung
    • bilateral trilobed lungs = bilateral minor fissures (SPECIFIC)
    • bilateral eparterial bronchi (MR/tomogram) = pulmonary arteries inferior to bronchi on PA view + projecting anterior to trachea on LAT view
    • diminished pulmonary vascularity/pulmonary venous hypertension (TAPVR below diaphragm)
  • @ Heart & great vessels
    • bilateral systemic/right atria with broad-based appendages
    • ipsilaterality of abdominal aorta + IVC
      • = juxtaposed “piggybacked” IVC (aorta usually posterior) (MOST RELIABLE INDICATOR)
    • bilateral SVC
  • @ Abdomen
    • absent spleen
    • centrally located “bridging” liver = hepatic symmetry
    • stomach on right/left side/in central position and small (microgastria)
Prognosis: up to 80% mortality by end of 1st year of life
Polysplenia Syndrome
  • = BILATERAL LEFT-SIDEDNESS = LEFT ISOMERISM
Age: presentation in infancy/adulthood; M < F
Associated with:
  • CHD (>50%):
    • APVR (70%), dextrocardia (37%), ASD (37%), ECCD (43–65%), pulmonic valvular stenosis (23%), TGA (13–17%), DORV (13–20%)
    • • no/mild CHD in most patients
  • GI abnormalities:
    • esophageal atresia, TE fistula, gastric duplication, preduodenal portal vein, duodenal webs + atresia, short bowel, mobile cecum, malrotation, semiannular pancreas, biliary atresia, absent gallbladder
  • GU anomalies (15%):
    • renal agenesis, renal cysts, ovarian cysts
  • Vertebral anomalies, common celiac trunk–SMA
  • • CHF (due to L-to-R shunt)
  • • heart murmur, occasional cyanosis
  • • leftward/superiorly directed P-wave vector
  • • heart block (due to ECCD)
  • • extrahepatic biliary obstruction
  • absence of IVC (on LAT CXR)
  • large azygos vein (on AP CXR) may mimic aortic arch
P.639

  • @ Lung
    • bilateral morphologic left lungs (55-C68%), normal (18%), bilateral R-sided lungs (7%)
    • bilateral hyparterial bronchi (= arteries projecting superior to bronchi on PA view + posterior to tracheobronchial tree on LAt view)
    • normal/increased pulmonary vascularity
    • absence of middle lobe fissure
  • @ Heart & great vessels
    • bilateral pulmonary/left atria + pointed, tubular, narrow-based appendages
    • cardiac apex on R/in midline
    • bilateral SVC (50%)
    • interruption of hepatic segment of IVC with azygos/hemiazygos continuation in 65–70% (MOST CONSISTENT FINDING)
  • @ Abdominal heterotaxy (56%)
    • presence of ≥2 spleens (usually two major + indefinite number of splenules) located on both sides of the mesogastrium (esp. greater curvature of stomach)
    • centrally located liver = hepatic symmetry
    • absence of gallbladder (50%)
    • stomach always on same side of spleen(s)
    • malrotation of bowel (80%)
    • preduodenal portal vein
OB-US:
  • absence of intrahepatic IVC
  • aorta anterior to spine in midline
  • “double vessel” sign = 2 vessels of similar size in paraspinous location posterior to heart = aorta + azygos vein on left/right side of spine
Prognosis: 50% mortality by 4 months;
75% mortality by 5 years;
90% mortality by midadolescence
Heterotaxy syndromes
  Asplenia
= bilateral right sidedness
Polysplenia
= bilateral left sidedness
Clinical
Presenting age newborn/infant infant/adult
Sex predominance male female
Cyanosis severe usually absent
Heart disease severe moderate/none (5–10%)
Howell-jolly/Heinz bodies present absent
Spleen scan no spleen multiple small spleens
Characteristic eCG none abnormal P-wave vector
Prognosis poor good
Mortality high low
Plain radiograph
Lung vascularity decreased normal/increased
Aortic arch right/left right/left
Cardiac apex right/left/midline right/left
Bronchi bilateral eparterial bilateral hyparterial
Minor fissure possibly bilateral none/normal
Stomach midline/right/left right/left
Liver symmetrical/R/L in various positions
Malrotation of bowel yes (microgastria) yes
Cardiography
Coronary sinus usually absent sometimes absent
Atrial septum common atrium (100%) ASD (84%)
AV valve atresia/common valve normal/abnormal MV
Single ventricle 44% infrequent
IVS VSD VSD common
Great vessels d-/l-transposition (72%) normal relationship
Pulmonary stenosis the rule frequent
Pulmonary veins tAPVR PAPVR (42%) tAPVR (6%)
Single coronary artery 19%  
SVC bilateral (53%) bilateral (33%)
IVC-aorta relationship same side of spine normal
IVC normal interrupted (84%)/normal
Azygos vein inapparent continuation R/L
Hypoplastic Left Heart Syndrome
  • = SHONE SYNDROME = AORTIC ATRESIA
  • = underdevelopment of left side of heart characterized by
    • hypoplastic/atretic aortic valve
    • hypoplastic/atretic mitral valve
    • hypoplastic LV (due to endocardial fibroelastosis)
    • hypoplastic ascending aorta
    • normally related great vessels
    Prevalence: 0.2/1,000 live births; M:F = 2:1
    • 4th most common cardiac malformation manifesting in 1st year of life (after VSD, TGV, tetralogy of Fallot)
    • Most common cause of CHF in neonate
    • Responsible for 25% of all cardiac deaths in 1st week of life
Hemodynamics: pulmonary venous blood in LA faces an atretic/stenotic MV (= pulmonary venous outflow obstruction) and is diverted to RA through herniated foramen ovale/ASD (L-to-R shunt); RV supplies (a) pulmonary artery, (b) ductus arteriosus, (c) descending aorta (antegrade flow), (d) aortic arch + ascending aorta + coronary circulation (retrograde flow) leading to RV work overload + CHF
P.640

  • Associated malformations:
    • coarctation of aorta, PDA, patent foramen ovale, dilated pulmonary artery, VSD, dilated RA, enlarged RV, double-outlet right ventricle, endocardial fibroelastosis
  • • severe CHF (RV volume + pressure overload):
    • • characteristically presents within first few hours of life
  • • ashen gray color/dusky complexion (systemic underperfusion due to inadequate atrial L-to-R shunt)
  • • myocardial ischemia (decreased perfusion of aorta [= “common coronary artery”] + coronary arteries):
    • • cardiogenic shock, metabolic acidosis (when ductus arteriosus closes)
CXR:
  • hypoplastic/normal/enlarged cardiac silhouette:
    • prominent right atrial border
    • ±absence of left ventricular silhouette
    • ±thymic atrophy
  • interstitial + alveolar pulmonary edema (due to pulmonary venous hypertension with severely restrictive interatrial communication in 80%)
  • normal pulmonary vasculature (with wide nonrestrictive interatrial communication in 20%)
OB-US (may be missed <22 weeks GA):
  • small left ventricular cavity (apex of LV and RV should be at same level)
  • hypoplastic ascending aorta
  • aortic coarctation (in 80%)
  • diastolic flow reversal in narrow ascending aorta is DIAGNOSTIC
ECHO:
  • normal/enlarged LA
  • slitlike/small/normal LV
  • enlarged RA
  • herniation + prolapse of foramen ovale flap into RA
  • hypoplastic ascending aorta (<5 mm = aortic atresia)
  • absent/grossly distorted mitral valve echoes
Angio:
  • retrograde flow in ascending aorta + aortic arch + coronary arteries via PDA
  • stringlike ascending aorta <6 mm in diameter
  • massive enlargement of RV + RVOT
Prognosis: almost 100% fatal by 6 weeks
Time of diagnosis: 32% pre-, 65% 1–4 days postnatally
Rx: (1) Prostaglandin E1 (patency of ductus arteriosus)
(2) Hypoventilation (increase in CO2 maintains high pulmonary vascular resistance)
(3) Nitroprusside IV (decreases systemic vascular resistance)
(4) Norwood procedure = palliative attempt
(5) Cardiac transplant
Hypoplastic Right Ventricle
  • = PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM
  • = underdeveloped right ventricle due to pulmonary atresia in the presence of an intact interventricular septum
Type I = small RV secondary to competent tricuspid valve (more common)
Type II = normal/large RV secondary to incompetent tricuspid valve
Hemodynamics:
fetus: L-to-R atrial shunt through foramen ovale; retrograde flow through ductus arteriosus into pulmonary vascular bed
neonate: closure of ductus results in cyanosis, acidosis, death
  • small right ventricular cavity (apex of RV + LV should be at same level)
  • atresia of pulmonary valve
  • hypoplastic proximal pulmonary artery
  • secundum atrial septal defect (frequently associated)
Rx: prostaglandin E1 infusion + valvotomy + systemic-pulmonary artery shunt
Idiopathic Dilatation of Pulmonary Trunk
= CONGENITAL ANEURYSM OF PULMONARY ARTERY
Age: adolescence; M < F
  • • systolic ejection murmur (in most cases)
  • • asymptomatic
  • dilated main pulmonary artery causing a round bulge at the mediastinal border simulating a mass
  • normal peripheral pulmonary vascularity
  • normal pulmonary arterial pulsations
  • NO lateralization of pulmonary flow
Dx per exclusion:
  • Absence of shunts, CHD, acquired disease
  • Normal RV pressure
  • No significant pressure gradient across pulmonic valve
Prognosis: nonprogressive
DDx: (1) Marfan syndrome
(2) Takayasu arteritis
Interruption of Aortic Arch
= rare congenital anomaly as a common cause of death in the neonatal period
Trilogy: (1) Interrupted aortic arch
(2) VSD
(3) PDA (pulmonary blood supplies lower part of body)
In 1/3 associated with:
  • Bicuspid aortic valve
  • Muscular subaortic stenosis
  • ASD
  • Truncus arteriosus
  • Transposition
  • Complete anomalous pulmonary venous return
  • presents with CHF
Location:
Type A: distal to left subclavian artery (42%)
Type B: between left CCA and subclavian artery (53%) associated with: DiGeorge syndrome
Type C: between innominate and left CCA (4%)
  • dilatation of right atrium + ventricle
  • P.641

  • dilatation of pulmonary artery
  • ascending aorta much smaller than pulmonary artery
  • arch formed by pulmonary artery + ductus arteriosus gives the appearance of a low aortic arch
  • aortic knob absent
  • trachea in midline
  • NO esophageal impression
  • retrosternal clear space increased (small size of ascending aorta)
  • increased pulmonary vascularity (L-to-R shunt)
Prognosis: 76% dead at end of 1st month
image
Interruption of Aortic Arch
Interruption Of Pulmonary Artery
= pulmonary trunk continues only as one large artery to one lung while systemic aortic collaterals supply the other side
Associated with: CHD (particularly if interruption on left side):
1. Tetralogy of Fallot
2. Scimitar syndrome = congenital pulmonary venolobar syndrome
3. PDA, VSD
4. Pulmonary hypertension
Collateral supply:
  • Arteries arising from arch + ascending aorta
  • Bronchial vessels
  • Intercostal vessels
  • Branches from subclavian artery
Location: usually opposite from aortic arch; R > L pulmonary artery
CXR:
  • hypoplastic ipsilateral lung
  • lung opacity similar to normal lung/slightly increased
  • volume loss of affected hemithorax:
    • mediastinal shift toward involved lung
    • hemidiaphragm may be elevated
    • small hyperlucent ipsilateral chest with narrowed intercostal spaces
  • hyperinflated contralateral lung with herniation into smaller hemithorax
  • “comma-shaped” small distorted hilar shadow
  • asymmetry of pulmonary vascularity
  • normal respiratory motion (normal aeration of hypoplastic lung)
CECT:
  • affected pulmonary artery completely absent/terminates within 1 cm of its origin
  • serrated pleural thickening (= enlarged intercostal + transpleural arteries)
  • multiple linear opacities perpendicular to pleural surface (= transpleural systemic vessels)
NUC:
  • absent perfusion with normal aeration
Angio:
  • absent pulmonary artery
Cx: recurrent pulmonary infection, hemorrhage, hemoptysis (10%), mild exertional dyspnea, pulmonary hypertension (19–25%)
Rx: surgical anastomosis between proximal + distal pulmonary artery (to prevent progressive pulmonary hypertension with dyspnea, cyanosis, hemoptysis, death)
DDx: (1) Hemitruncus
(2) Swyer-James syndrome (ipsilateral air trapping, reduced ventilation + perfusion)
(3) Chronic thromboembolic occlusion
(4) Takayasu arteritis
(5) Mediastinal fibrosis
(6) Hypogenetic lung syndrome (abnormal bronchial brancing pattern
Intravenous Drug Abuse
Complications secondary to:
  • direct toxic effects of drugs or drug combinations (eg, heroin + cocaine/talwin)
  • direct toxic effects of adulterants [eg, heroin is mixed (“cut”) with quinine, baking soda, sawdust]
  • septic preparation
  • injection technique
  • choice of injection site (eg, “groin hit” into femoral vein; “pocket shot” into jugular, subclavian, brachiocephalic vein)
  • Cardiovascular complications
    • Arterial pseudoaneurysm may be followed by rupture with exsanguination/loss of limb
    • Arteriovenous fistula
    • Arterial occlusion
      • at injection site due to intimal damage, thrombosis, spasm
      • distal to injection site due to embolization, spasm
    • Venous thrombosis
    • Intravenous migration of needle to heart/lungs
    • embolization of infectious agent/foreign body/air through inadvertent arterial injection (“hit the pink”)
    • endocarditis (most commonly S. aureus)
  • Soft-tissue complications
    • Hematoma/abscess
    • foreign bodies
    • Lymphadenopathy
    • Cellulitis
  • Skeletal complications
    • Osteomyelitis
      • direct contamination: eg, pubic bone (“groin hit”)/clavicle (“pocket shot”)
      • hematogenous: spine most commonly affected
    • P.642

    • Septic arthritis:
      • spine, sacroiliac, sternoclavicular, symphysis pubis, acromioclavicular, hip, knee, wrist
  • Pleuropulmonary complications
    • Pneumothorax (“pocket shot”)
    • Hemo-/pyothorax
    • Septic pulmonary emboli
  • Gastrointestinal complications
    • Severe colonic ileus
    • Colonic pseudoobstruction
    • Necrotizing enterocolitis
    • Liver abscess
  • Genitourinary complications
    • focal/segmental glomerulosclerosis (heroin abuser)
    • Amyloidosis
  • CNS complications
    • Spinal epidural abscess in 5–18%(from vertebral osteomyelitis)
    • Cord compression (from collapsed vertebral body)
    • Cerebral infarction from subacute bacterial endocarditis, toxic effect of drug, spasm, intimal damage from “pocket shot”
    • Intracranial hemorrhage from trauma, hypertension, vasculitis, injection of anticholinergic drugs, rupture of mycotic aneurysm
    • Meningitis, cerebral abscess
Ischemic Heart Disease
= CORONARY ARTERY DISEASE (CAD)
Incidence: 1.5 million/year; leading cause of death in industrial nations
Morbidity: 28.7 cases per 1,000 men per year
Mortality: 3.1 deaths per 1,000 men per year
Noninvasive testing:
  • Noninvasive testing is of marginal benefit when disease prevalence is <0.2/>0.7
  • Concordant thallium-201 and stress ECG are greater predictors of disease probability than either one used alone and/or when discordant
  • Sequential thallium-201 and stress ECG are most useful to establish the diagnosis of CAD when pretest prevalence is intermediate + test results are concordant
CXR:
  • often normal
  • coronary artery calcification
  • pulmonary venous hypertension following acute infarction (40%)
  • LV aneurysm
ECHO:
  • region of dilatation with disturbance of wall movement
(1) Akinesis = no wall motion
(2) Hypokinesis = reduced wall motion
(3) Dyskinesis = paradoxical systolic expansion
(4) Asynchrony = disturbed temporal sequence of contraction
Coronary angiography: 1.2 million procedures per year
Kawasaki Syndrome
  • = MUCOCUTANEOUS LYMPH NODE SYNDROME
  • = acute febrile multisystem vasculitis of unknown cause involving large + medium-sized + small arteries with a predilection for the coronary arteries
Incidence: average of 1.1:100,000 population per year
Histo: panvasculitis
Age: <5 years of age (in 85%); peak age of 1–2 years;
M:F = 1.5:1
Associated with: polyarthritis (30–50%), aseptic meningitis (25%), hepatitis (5–10%), pneumonitis (5–10%)
  • • fever >5 days
  • • mucosal reddening (injected fissured lips, injected pharynx, strawberry tongue) in 99%
  • • nonpurulent cervical lymphadenopathy (82%)
  • • maculopapular rash on extensor surfaces (99%)
  • • bilateral nonpurulent conjunctivitis (96%)
  • • erythema of palms + soles with desquamation (88%)
  • @ Cardiovascular system (1/3)
    • Coronary artery abnormality (15–25%)
      • coronary artery aneurysm: LCA (2/3), RCA (1/3); proximal segment in 70%; 48% regress, 37% diminish in size
      • coronary artery stenosis (39%) due to thrombus formation in aneurysm + intimal thickening
      • coronary artery occlusion (8%) in aneurysms >9 mm
    • Myocarditis (25%)
    • Pericarditis
    • Valvulitis
    • Atrioventricular conduction disturbance
  • intestinal pseudoobstruction
  • transient gallbladder hydrops
Prognosis: 0.4–3% mortality (from myocardial infarction/myocarditis with congestive heart failure/rupture of coronary artery aneurysm)
Rx: aspirin (100 mg/kg per day) + gamma globulin
DDx: infantile polyarteritis
Leiomyosarcoma of Ivc
most common intravascular venous tumor
Path: growth patterns: extravascular, intravascular, combined
  • • shortness of breath (decreased cardiac return)
  • • elevated liver functions, jaundice
  • • lower-extremity edema
  • collateral pathways bypassing IVC: hemiazygos, azygos
  • tumor extension from IVC into right atrium
US:
  • blood flow in IVC/hepatic veins may be absent/reversed/turbulent (depending on degree of obstruction)
  • tumor vascularity (DDx from thrombus)
CT:
  • contrast enhancement of tumor
Cx: Budd-Chiari syndrome (from sudden/gradual occlusion of hepatic veins/IVC/both)
DDx of tumor extension into right atrium:
  • renal cell carcinoma, hepatocellular carcinoma
Lymphoma of heart
Secondary Cardiac Lymphoma
P.643

Incidence: in 16–28% on autopsy; pericardial involvement more frequent; more common in immunocompromised patients
Primary Cardiac Lymphoma
  • = lymphoma that involves only heart/pericardium at time of diagnosis (extremely rare)
Age: 13–90 (mean, 60) years
Predisposed: immunocompromised patients, esp. AIDS
Path: multiple firm nodules; contiguous invasion of pericardium
Histo: typically NHL: well-differentiated B-cell lymphoma, follicular center cell lymphoma, diffuse large cell lymphoma, undifferentiated Burkitt-like lymphoma
  • unresponsive rapidly progressive heart failure
  • arrhythmia, cardiac tamponade, SVC syndrome
  • chest pain
Location: RA > RV > LV > LA > atrial septum > ventricular septum; >1 chamber (75%)
CXR:
  • cardiomegaly
  • signs of CHF
  • massive pericardial effusion
ECHO:
  • hypoechoic myocardial masses in RA/RV
  • pericardial effusion
CT:
  • hypo-/isoattenuating masses relative to myocardium
  • heterogeneous enhancement of masses
MR:
  • poorly marginated heterogeneous lesions of iso- to hypointensity relative to myocardium on T1WI
  • lesions isointense to myocardium on T2WI
  • heterogeneous enhancement with gadolinium
Dx: positive cytology in pericardial fluid (in 67%); exploratory thoracotomy with biopsy of cardiac tissue
Prognosis: very poor
Microscopic Polyangitis
  • MICROSCOPIC POLYARTERITIS = HYPERSENSITIVITY VASCULITIS = LEUKOCYTOCLASTIC VASCULITIS
  • pauci-immune necrotizing small-vessel angitis without granulomatous inflammation
Path: necrotizing arteritis identical to polyarteritis nodosa but in vessels smaller than arteries (= arterioles, venules and capillaries)
Trigger: drugs (eg, penicillin), microorganisms, heterologous proteins, tumor antigens
  • hemoptysis, hematuria, proteinuria
  • abdominal pain, GI bleeding, muscle pain + weakness
  • ANCA (antineutrophil cytoplasmic autoantibodies) in >80%
  • negative serologic tests for hepatitis B
Location: skin, mucous membranes, lung, brain, heart, GI tract, kidney, muscle
  • Most common cause of the pulmonary-renal syndrome!
  • pulmonary infiltrates (due to capillaritis)
  • glomerulonephritis (90%)
Rx: removal of offending agent
Mitral Regurgitation
= MITRAL INSUFFICIENCY
Cause:
  • ACUTE
    • Spontaneous rupture of chordae tendineae
    • Myocardial infarction with involvement of papillary muscle (posteromedial > anterolateral papillary muscle)
    • Bacterial endocarditis
    • Periprosthetic valve leak
  • CHRONIC
    • Acute rheumatic fever
      • isolated: frequently seen in children
      • uncommon in adults (mostly combined with stenosis)
    • Mitral valve prolapse syndrome
    • Atrial myxoma
    • Coronary artery disease
    • Idiopathic hypertrophic subaortic stenosis (IHSS)
    • Myxomatous degeneration of mitral valve: eg, Marfan syndrome
    • Mitral annulus calcification
    • Functional/secondary (from dilatation of mitral ring in any condition with severe dilatation of LV)
    • Congenital heart disease: short/abnormally inserted chordae tendineae; persistent ostium primum ASD with cleft mitral valve, corrected transposition with Ebstein-like anomaly
Hemodynamics:
  • backward flow of blood from LV into LA during LV systole; increased volume of blood under elevated pressure causes dilatation of LA; marked increase in LV diastolic volume with little increase in LV diastolic pressure (= increase in preload without increase in afterload = elevated ejection fraction)
  • ACUTE MITRAL REGURGITATION
    • pulmonary venous hypertension with engorged pulmonary vessels and cephalization (less than with mitral stenosis)
    • symmetric interstitial/alveolar pulmonary edema:
      • asymmetric right upper lobe edema (9%) due to preferential flow of regurgitant jet into pulmonary vein of RUL (PATHOGNOMONIC)
    • limited cardiac enlargement
  • CHRONIC MITRAL REGURGITATION
    • enlarged heart
    • massive LA:
      • LA posterior wall calcification (McCallum patch)
      • enlarged LA appendage (with history of previous rheumatic heart disease)
    • mitral annular calcification (frequent)
    • marked LV enlargement (cardiothoracic ratio >0.55) + LV failure
ECHO:
  • LV volume overload:
    • normal-sized/enlarged LV
    • increased septal + posterior wall motion
  • increased EF slope
  • early closure of aortic valve (LV stroke volume partially lost to LA)
  • LA enlargement (in chronic MV insufficiency)
  • bulging of interatrial septum to the right during systole
  • P.644

  • Doppler is only diagnostic tool + allows assessment of severity
Mitral Stenosis
Cause: rheumatic heart disease (5–15 years after initial episode of rheumatic fever); carcinoid syndrome; eosinophilic endocarditis; rheumatoid arthritis; SLE; mass obstructing LV inflow (tumor, atrial myxoma, thrombus); congenital
  • M:F = 1:8
Hemodynamics:
  • rise in left atrial + pulmonary vascular pressure throughout systole and into diastole; compensatory dilation of LA + pulmonary venous hypertension; development of medial hypertrophy + intimal sclerosis in pulmonary arterioles leads to postcapillary pulmonary arterial hypertension; RV hypertrophy; tricuspid regurgitation; RV dilatation; right heart failure
May be associated with: ASD = Lutembacher syndrome (in 0.6%) causing L-to-R shunt
  • history of rheumatic fever (in 50%)
  • dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea
  • atrial fibrillation
  • systemic embolization from thrombosis of atrial appendage
Stages (according to degree of pulmonary venous hypertension):
Stage 1 : loss of hilar angle, redistribution
Stage 2 : interstitial edema
Stage 3 : alveolar edema
Stage 4 : hemosiderin deposits + ossification
  • @ Left heart
    • enlarged LA ±wall calcification:
      • “double density” seen through right upper cardiac border (AP view)
      • bulge of superior posterior cardiac border below carina (lateral view)
      • splaying of mainstem bronchi
      • esophagus displaced toward right + posteriorly
      • dilated left atrial appendage (not present with retracting clot), in 90% associated with rheumatic heart disease
        • Dilatation of left atrial appendage + calcification = rheumatic heart disease!
    • calcification of valve leaflets in 60% of severe MS, usually >50 years of age (DDx: calcification of mitral annulus)
    • normal/undersized LV
    • small aorta (due to decrease of forward cardiac output)
  • @ Right heart
    • prominent pulmonary artery segment (precapillary hypertension)
    • hypertrophy of RV
    • dilatation of RV (tricuspid insufficiency/pulmonary hypertension)
    • increase in cardiothoracic ratio
    • diminution of retrosternal clear space
    • IVC pushed backward (lateral view)
  • @ Lung
    • pulmonary vascular cephalization = redistribution of pulmonary blood flow to upper lobes (postcapillary pressure 16–19 mm Hg)
    • interstitial pulmonary edema (postcapillary pressure 20–25 mm Hg)
      DDx: interstitial fibrosis/deposition of hemosiderin-laden macrophages (= “brown induration”) of chronic mitral valve stenosis
    • alveolar edema (postcapillary pressure 25–30 mm Hg)
      DDx: diffuse alveolar hemorrhage = diffuse confluent acinar/ground-glass areas of increased opacity sparing the lung periphery (= “window frame” effect)
    • pulmonary hemosiderosis:
      • 1–3 mm ill-defined nodules
      • fine/coarse reticular areas of increased opacity with bias for middle and lower lungs
    • pulmonary ossification (3–13%) = densely calcified 1–3–5 mm nodules (±trabeculae) mainly in middle and lower lungs
ECHO:
  • thickening of leaflets toward free edge (fibrosis, calcification)
  • flattening of EF slope = MV remains open throughout diastole due to persistently high LA pressure (crude index of severity of MV stenosis)
  • diastolic anterior tracking of pML in 80% (secondary to diastolic anterior pull by larger + more mobile aML)
  • diastolic doming of MV leaflets
  • commissure fusion = increased echodensity + decreased leaflet motion at level of commissure
  • area reduction of MV orifice: normal within 4–6 cm2; mild narrowing with <2 cm2; severe narrowing with <1 cm2 (reproducible to within 0.3 cm2)
  • shortening + fibrosis of chordae tendineae
  • abnormal septal motion = early diastolic dip of IVS due to rapid filling of RV (in severe MV stenosis)
  • slowed LV filling pattern of small LV
  • dilatation of LA (>5 cm increases risk of atrial fibrillation + left atrial thrombus)
  • De opening amplitude reduced to <20 mm indicating loss of valve pliability (DDx: low cardiac output state)
  • absent A-wave common (atrial fibrillation)
  • increase in valve gradient + pressure halftime on Doppler
Rx: (1) Commissurotomy if valves pliable + calcium absent + MV regurgitation absent
(2) Valve replacement for symptomatic patients with severely stenotic valves
DDx: (1) Pseudomitral stenosis in decreased LV compliance (decreased ef slope, normal leaflet thickness + motion)
(2) Rheumatic mitral insufficiency (indistinguishable findings + evidence of LV volume overload)
(3) LA myxoma (mass behind MV + in LA)
(4) Low cardiac output (apparent small valve orifice)
Lutembacher Syndrome
  • = rheumatic mitral valve stenosis + ASD
Mitral Valve Prolapse
Incidence: 2-6%of general population; 5-20%of young women;? autosomal dominant inheritance
Age: commonly 14-30 years
P.645

Cause:
  • “floppy mitral valve” = elongation of cusps + chordae leading to redundant valve tissue, which prolapses into LA during systole
    • Associated with:
      • Skeletal abnormalities: scoliosis, straightening of thoracic spine, narrow anteroposterior chest dimension, pectus excavatum deformity of sternum
      • Barlow syndrome = straight back syndrome
      • Marfan syndrome
      • tricuspid valve prolapse
      • Long-standing ASD
      • Autosomal dominant polycystic kidney disease
  • Secondary MV prolapse:
    • papillary muscle dysfunction, rupture of chordae tendineae, rheumatic mitral insufficiency, primary pulmonary hypertension, ostium secundum ASD
  • •arrhythmias, palpitation, chest pain, light-headedness, syncope
  • •responsible for midsystolic click + late systolic murmur (when associated with mitral regurgitation)
  • LA not enlarged (unless associated with significant mitral regurgitation)
ECHO:
  • interruption of CD line with bulge toward left atrium:
    • abrupt midsystolic posterior buckling of both leaflets (classic pattern)
    • “hammocklike” pansystolic posterior bowing of both leaflets
  • multiple scallops on mitral valve leaflets (short-axis parasternal view)
  • valve leaflets may appear thickened (myxomatous degeneration + valve redundancy)
  • mitral valve leaflets passing >2 mm posterior to plane of mitral annulus (apical 4-chamber view)
  • hyperactive atrioventricular groove
  • mitral annulus may be dilated >4.7 cm2
DDx: (1) Pericardial effusion (systolic posterior displacement of MV leaflets + entire heart)
(2) Bacterial endocarditis (mimicked by locally thickened + redundant leaflets)
Myocardial Infarction
Incidence: 1,500,000 per year in USA resulting in 500,000 deaths (50% occur in asymptomatic individuals)
  • • atrioventricular block (common with inferior wall infarction as AV nodal branch originates from RCA); complete heart block has worse prognosis because it indicates a large area of infarction
CXR:
  • normal-sized heart (84–95%) in acute phase if previously normal
  • cardiomegaly: high incidence of congestive heart failure with anterior wall infarction, multiple myocardial infarctions, double- and triple-vessel CAD, LV aneurysm
CECT:
  • perfusion defect within 60–90 seconds after bolus injection
  • delayed enhancement of infarcted tissue peaking at 10–15 minutes (due to accumulation of iodine in ischemic cells), size of enhanced area correlates well with size of infarct
MR:
  • no change/mildly decreased signal intensity of myocardium on T1WI (= edema of myocardium)
  • high-intensity focus in region of acute infarction on T2WI
  • thinning of LV wall + decreased signal intensity on T2WI (in remote infarction)
  • increased intracavitary signal (from slow flow caused by stasis of blood adjacent to infarcted myocardium)
CEMR:
  • pooling of gadolinium within region of infarction (disproportionately slow washout) on delayed images 20–30 minutes post injection
  • highlighting of infarcted tissue with contrast using magnetic susceptibility to selectively suppress signals from normal myocardium
Cx: (myocardium is prone to rupture during 3rd–14th day post infarction)
Left Ventricular Failure (60–70%)
  • • “cardiac shock” = systolic pressure <90 mm Hg
  • Signs of pulmonary venous hypertension are a good predictor of mortality (>30% if present, <10% if absent)
  • progressive enlargement of heart
  • haziness + indistinctness of pulmonary arteries
  • increase in size of right descending pulmonary artery >17 mm
  • pleural effusion
  • septal lines
  • perihilar ±peripheral parenchymal clouding
  • alveolar pulmonary edema
Mortality: 30–50% with mild LV failure; 44% with pulmonary edema; 80–100% with cardiogenic shock; 8% in absence of LV failure
Ventricular Aneurysm (12–15% of survivors)
 
Myocardial Rupture (3.3%)
  • • occurs usually on 3rd–5th day post MI
  • enlargement of heart (slow leakage of blood into pericardium)
Prognosis: cause of death in 13% of all infarctions; almost 100% mortality
Rupture of Papillary Muscle (1%)
Cause: infarction of posteromedial papillary muscle in inferior MI (common)/anterolateral papillary muscle in anterolateral MI (uncommon)
  • • sudden onset of massive mitral insufficiency
  • •unresponsive to medical management
  • abrupt onset of severe persistent pulmonary edema
  • asymmetric PVH in right upper lobe
  • minimal LV enlargement/normal-sized heart
  • NO dilatation of LA (immediate decompression into pulmonary veins)
Prognosis: 70% mortality within 24 hours; 80–90% within 2 weeks
Rupture of Interventricular Septum (0.5–2%)
  • • occurs usually within 4–21 days with rapid onset of L-to-R shunt
  • P.646

  • •Swan-Ganz catheterization: increase in oxygen content of RV, capillary wedge pressure may be within normal limits
  • right-sided cardiac enlargement
  • engorgement of pulmonary vasculature:
  • asymmetric PVH of right upper lobe
  • NO pulmonary edema (DDx to ruptured papillary muscle)
Prognosis: 24% mortality within 24 hours; 87% within 2 months; >90% in 1 year
Dressler Syndrome (<4%)
  • = POSTMYOCARDIAL INFARCTION SYNDROME
Etiology: autoimmune reaction to myocardial infarction
Onset: 2–3 weeks (range 1 week–several months) following infarction
  • • relapses occur as late as 2 years after initial episode
  • • pleuritic chest pain
  • •fever
  • thickening of pericardium
  • pericardial effusion
  • pleural effusion
  • pneumonitis
Right Ventricular Infarction
  • Right ventricle involved in 33% of left inferior myocardial infarction
  • decreased RV ejection fraction
  • accumulation of Tc-99m pyrophosphate
Prognosis: in 50% RV ejection fraction returns to normal within 10 days
Cx: (1) cardiogenic shock (unusual)
(2) elevation of RA pressure
(3) decrease of pulmonary artery pressure
Myxoma
= most common benign primary cardiac tumor (true neoplasm) in adults, 40–50% of all cardiac tumors
Age: 11–82 (mean 50) years; 90% of patients are between ages 30 and 60 years; M:F = 1:1.7 to 1:4
Classification: sporadic (most frequent); familial type (mean age of 24 years); complex type = Carney syndrome
Path: (a) gelatinous, friable, papillary/villous pedunculated tumor
(b) round/lobular smooth sessile tumor (25%) with firm surface
♢ No infiltration of underlying tissues!
Histo: composed of myxoma cells (= ovoid nucleus with inconspicuous/large nucleoli + abundant eosinophilic cytoplasm)) forming rings/syncytia/cords; hypocellular amorphous acid mucopolysaccharide matrix in areas without fibrosis; covered by a mono-layer of endothelial cells (= endocardial tumor)
Size: 0.6–12 (mean, 5.7) cm
  • short history + rapid progression
  • dyspnea, chest pain
  • constitutional symptoms (30%):
    • fever, myalgia, arthralgia, weight loss, lethargy
    • leukocytosis, anemia, elevated ESR, petechiae
    • hypergammaglobulinemia
  • positional symptoms (ie, change with position) due to hemodynamic obstruction:
    • arrhythmia (20%), heart murmur
    • congestive heart failure (valve obstruction)
    • syncope
  • embolization (30–40%) to CNS, coronary artery, aorta, kidney, spleen, extremities, pulmonary artery (caused by tumor fragments/accumulated thrombus)
Location: left atrium (75–80%); right atrium (10–20%); ventricle (5%); biatrial (with growth through fossa ovalis)
Site: attached to interatrial septum by small stalk in fossa ovalis (75%)/to wall of cardiac chambers/to valve surfaces; may protrude into ventricle causing partial obstruction of atrioventricular valve
  • small myxomas produce no CXR findings
  • cardiomegaly
  • atrial obstruction (mimicking valvular stenosis)
  • persistent defect in atrium/diastolic defect in ventricle
  • LEFT ATRIAL MYXOMA (75–80%) with obstruction of mitral valve:
    • pulmonary venous hypertension:
      • pulmonary vascular redistribution
      • interstitial edema
    • enlargement of LA
    • NO enlargement of atrial appendage
    • ossific lung nodules
    Cx: systemic emboli (27%) in 50% to CNS (stroke/“mycotic” aneurysm)
  • RIGHT ATRIAL MYXOMA (10–20%) with obstruction of tricuspid valve:
    • tumor calcification: R > L
    • enlargement of RA
    • prominent SVC, IVC, azygos vein
    • decreased pulmonary vascularity
    • pleural effusion (occasionally)
    Cx: pulmonary emboli
ECHO: (2D-ECHO is study of choice)
  • tumor attached by narrow stalk
  • tumor mobility:
    • prolapse across AV valve during diastole
  • tumor distensibility
  • hyperechoic spherical mass:
    • internal hypoechoic areas (= hemorrhage, necrosis)
    • speckled echogenic foci (= calcifications)
    • frondlike surface projections
  • Doppler:
    • valvular regurgitation
  • M-mode findings of only historical interest:
    • dense echoes appearing posterior to aML soon after onset of diastole
    • pML obscured
    • tumor echoes can be traced into LA
    • dilated LA
    • reduced E-F slope
CT:
  • well-defined spherical/ovoid intraluminal filling defect
  • lobular/smooth surface contour
  • tumor attenuation lower than unopacified blood (due to gelatinous component)
  • P.647

  • heterogeneous texture (due to hemorrhage, necrosis, cyst formation, fibrosis, calcification [16%], ossification)
MR:
  • iso-/hypointense on T1WI relative to myocardium
  • heterogeneous contrast enhancement (secondary to necrotic areas)
  • markedly hyperintense on T2WI
  • areas of decreased signal intensity (calcifications, hemosiderin deposits)
Rx: urgent surgical excision ±valvuloplasty/valve replacement
Prognosis: 5–14% recurrence rate (multifocal myxomas)
DDx: (1) Thrombus (most commonly in LA + LV)
(2) Other cardiac tumors: sarcoma, malignant mesenchymoma, metastasis, papillary fibroelastoma (also arises from narrow stalk)
Carney Complex
  • = CARNEY SYNDROME = COMPLEX MYOMA
  • = autosomal-dominant inherited disorder
Prevalence: 7% of all myxomas; 150 patients identified since 1985 worldwide
Age: younger than patients with sporadic myxoma
  • endocrine overactivity:
    • Cushing syndrome
    • sexual precocity
    • acromegaly
  • Cardiac myxomas: multifocal (66%), outside left atrium, recurring at an increased rate after resection
  • Hyperpigmented skin lesions: lentigines, ephelides, blue nevi
  • Myxoid fibroadenoma of the breast
  • Psammomatous melanotic schwannoma
  • Pituitary adenoma
  • Testicular tumor: large-cell calcifying Sertoli cell tumor
  • Primary pigmented nodular adrenocortical hyperplasia
N.B.: not related to Carney triad (pulmonary hamartomas, extraadrenal paragangliomas, gastric leiomyosarcoma)
Papillary Fibroelastoma
  • = FIBROELASTIC PAPILLOMA = PAPILLOMA/MYXOMA/FIBROMA OF VALVES = GIANT LAMBL EXCRESCENCE = MYXOFIBROMA = HYALINE FIBROMA
  • = benign endocardial papilloma predominantly affecting cardiac valves
Prevalence: 25% of all cardiac valvular tumors (most common valvular tumor); 10% of all primary cardiac tumors (2nd most common primary benign cardiac neoplasm after myxoma)
Mean age: 60 years; M:F = 1:1
Cause: ? reactive process,? hamartoma
Path: gelatinous mass with “sea anemone” appearance due to multiple delicate branching papillary fronds attached to endocardium by short pedicle
Histo: avascular papilloma composed of fibrous core + lined by a single layer of endothelium; scattered smooth muscle cells within papillary projections
  • mostly asymptomatic (incidental finding at autopsy, surgery, echocardiography, cardiac catheterization)
  • chest pain, dyspnea, embolic events (TIA/stroke from thrombi collecting on tumor)
  • NO valvular dysfunction
Location: aortic (29%) > mitral (25%) > tricuspid (17%) > pulmonary valve (13%); nonvalvular endocardial surface of atrium/ventricle (16%)
Size: <1 cm in diameter (may be as large as 5 cm)
ECHO:
  • <1.5 cm homogeneous mobile pedunculated mass:
    • elongated strandlike projection/well-defined head
    • CHARACTERISTIC stippled edge with a “shimmer/vibration” at interface between tumor and surrounding blood (DDx: amorphous thrombus)
    • flutters/prolapses with cardiac motion
  • turbulent blood flow
Rx: surgical excision ±leaflet repair/valve replacement
Patent Ductus Arteriosus
PDA = persistence of left 6th aortic arch, which connects the left pulmonary artery with the descending aorta beyond the origin of the left subclavian artery
Incidence: 9% of all CHD; M:F = 1:2
Associated with: prematurity, birth asphyxia, high-altitude births, rubella syndrome, coarctation, VSD, trisomy 18 + 21
Normal ductus physiology in mature infant:
  • increase in arterial oxygen pressure leads to constriction + closure of duct
  • Functional closure due to muscular contraction within 10–15–48 hours
  • Anatomic closure due to subintimal fibrosis + thrombosis: in 35% by 2 weeks; in 90% by 2 months; in 99% by 1 year
Hemodynamics of PDA:
  • increased volume of blood flows from aorta through PDA + pulmonary artery into lungs and then to left side of heart
image
  • mostly asymptomatic
  • congestive heart failure (rare) usually by 3 months of age if L-to-R shunt large
  • continuous murmur
  • bounding peripheral pulses (intraaortic pressure runoff through PDA)
CXR (mimics VSD):
  • enlarged pulmonary artery segment
  • increase of pulmonary vasculature; less flow directed to LUL
  • enlarged ascending aorta + aortic arch (thymus may obscure this)
  • LA + LV enlargement
  • enlarged RV (only with pulmonary hypertension)
  • prominent ductus infundibulum (diverticulum) = prominence between aortic knob + pulmonary artery segment
  • obscured aortopulmonary window
  • “railroad track” = calcified ductus arteriosus
ECHO:
  • LA:Ao ratio≥1.2:1 (signalizes significant L-to-R shunt)
P.648

Angio:
  • catheter course from RA to RV, main pulmonary artery, PDA, descending aorta
  • communication from aorta (distal to left subclavian artery) to left pulmonary artery on AP/LAT/LAO aortogram
PDA in Premature Infant
  • premature infant not subject to medial muscular hypertrophy of small pulmonary artery branches (which occurs in normal infants subsequent to progressive hypoxia in 3rd trimester)
  • • CHF
    • Cause:
      • pulmonary artery pressure remains low without opposing any L-to-R shunts (PDA/VSD)
      • ductus arteriosus remains open secondary to hypoxia in RDS
  • recurrence of alveolar airspace filling after resolution of RDS
  • granular pattern of hyaline membrane disease becomes more opaque
  • enlargement of heart (masked by positive pressure ventilation)
Rx:
  • Medical therapy:
    • supportive oxygen, diuretics, digitalis
    • avoid fluid overload (not to increase shunt volume)
    • antiprostaglandins = indomethacin opposes prostaglandins, which are potent duct dilators
  • Surgical ligation
Beneficial PDA
  • = compensatory effect of PDA in:
  • Tetralogy of Fallot
    • cyanosis usually occurs during closure of duct shortly after birth
  • Eisenmenger pulmonary hypertension
    • PDA acts as escape valve shunting blood to descending aorta
  • Interrupted aortic arch
    • supply of lower extremity via PDA
Nonbeneficial PDA
  • in L-to-R shunts (VSD, aortopulmonic window) a PDA increases shunt volume
Penetrating Aortic Ulcer
  • = PENETRATING ATHEROSCLEROTIC ULCER OF THE AORTA
  • = atheromatous intimal plaque characterized by ulceration that penetrates deep into the internal elastic lamina + reaches the media
Incidence: uncommon
Age: elderly with hypertension, hyperlipidemia and severe atherosclerosis
  • often asymptomatic
  • chest pain that may radiate to back (WITHOUT pulse difference, aortic regurgitation, CNS symptoms)
  • occasional distal ischemia due to embolic event
Location: middle/distal third of descending thoracic aorta (31%); occasionally abdominal aorta; may occur anywhere in aorta
  • extensive atherosclerotic disease + ectasia
  • lack of compression of the aortic lumen
  • intramural hematoma
  • NO intimal flap
CECT:
  • focally ulcerated plaque
  • adjacent subintimal hematoma (differentiation from intraluminal thrombus/atherosclerotic plaque not possible):
    • inward displacement of calcified intima (common)
  • thickening/enhancement of adjacent aortic wall
MR (valuable for patients in renal failure):
  • focal excavation of aortic wall
  • subacute hematoma in aortic wall of high signal intensity on T1WI + T2WI (methemoglobin) either localized or mimicking type 3 dissection
Angio:
  • ulcerated atherosclerotic plaque
  • aortic wall thickening
Cx:
  • Aortic dissection (2° to hemorrhage within media; controversial):
    • localized/extensive
    • communicating “double-barreled”/thrombosed
  • Saccular/fusiform aortic aneurysm (2° to stretching of aortic wall)
  • Aortic rupture (40% risk compared with 7% risk in aortic dissection)
Rx: (1) antihypertensive medication, analgesics
(2) excision of ulcer + aortic interposition graft (for patients with recurrent symptoms/pseudoaneurysm formation)
DDx: (1) Aortic dissection (intimal flap, patent false lumen)
(2) Atheroma/chronic intramural thrombus (low signal intensity on T1WI + T2WI)
Pericardial Defect
  • failure of pericardial development secondary to premature atrophy of the left duct of Cuvier (cardinal vein), which fails to nourish the left pleuropericardial membrane
Frequency: 1:13,000; M:F = 3:1
Age at detection: newborn to 81 years (mean 21 years)
Location:
  • PARTIAL ABSENCE (91%)
    (a) complete absence on left side (35%)
    (b) foraminal defect on left side (35%)
    (c) diaphragmatic pericardial aplasia (17%)
    (d) foraminal defect on right side (4%)
  • TOTAL BILATERAL ABSENCE (9%)
In 30% associated with:
  • Bronchogenic cyst (30%)
  • VSD, PDA, mitral stenosis
  • Diaphragmatic hernia, sequestration
  • • mostly asymptomatic
  • • palpitations, tachycardia, dyspnea, dizziness, syncope
  • • positional discomfort while lying on left side
  • • nonspecific intermittent chest pain (lack of pericardial cushioning, torsion of great vessels, tension on pleuropericardial adhesions, pressure on coronary arteries by rim of pericardial defect)
  • P.649

  • • ECG: right axis deviation, right bundle branch block
  • size:
    — small foraminal defect = no abnormality
    — large defect = herniation of cardiac structures/lung
    — complete absence = levoposition of heart
  • absence of left pericardial fat-pad
  • levoposition of heart with lack of visualization of right heart border
  • prominence/focal bulge in the area of RVOT, main pulmonary artery, left atrial appendage
  • sharp margination + elongation of left heart border
  • insinuation of lung between heart + left hemidiaphragm
  • insinuation of lung between aortic knob + pulmonary a.
  • increased distance between heart + sternum secondary to absence of sternopericardial ligament (cross-table lateral projection)
  • pneumopericardium following pneumothorax
  • NO tracheal deviation
Cx: cardiac strangulation
Rx: foraminal defect requires surgery because of
(a) herniation + strangulation of left atrial appendage,
(b) herniation of LA/LV
(1) Closure of defect with pleural flap
(2) Resection of pericardium
Pericardial Mesothelioma
= malignant primary neoplasm arising from mesothelial cells of the pericardium
Incidence: <1% of all mesotheliomas; 50% of all primary pericardial tumors
Age: 2–78 (mean, 46) years; M:F = 2:1
Path: multiple coalescing pericardial masses with obliteration of pericardial space; myocardial invasion is rare
Histo: biphasic tumor composed of epithelial areas forming tubulopapillary structures (resembling carcinoma) and spindled areas (resembling sarcoma)
  • • chest pain, cough, dyspnea, palpitations
  • • signs of pericarditis, cardiac tamponade
  • irregular diffuse pericardial thickening
  • cardiac encasement by soft-tissue masses
  • pericardial effusion
CXR:
  • cardiac enlargement with irregular contour
  • diffuse mediastinal enlargement
Rx: palliative surgery + radiation therapy
Prognosis: 6–12-month survival after diagnosis
Pericardial Teratoma
= benign germ cell neoplasm
Age: infants + children
Histo: derivatives of all 3 germ cell layers (neuroglia, cartilage, skeletal muscle, liver, intestine, pancreas, glandular tissue)
Location: within pericardial sac connected to a great vessel via a pedicle; intramyocardial (rare)
  • • respiratory distress, cyanosis (due to pericardial tamponade + compression of SVC, RA, aortic root, PA)
CXR:
  • enlarged cardiomediastinal silhouette
  • formed calcified teeth
US:
  • intrapericardial heterogeneous complex multilocular cystic mass:
    • intrinsic echogenic foci (= calcifications)
  • pericardial effusion
  • fetal hydrops (ascites, pleural effusion, subcutaneous edema, polyhydramnios)
MR:
  • large mass of heterogeneous signal intensity
Rx: emergent pericardiocentesis (life-threatening lesion); urgent surgical excision
Prognosis: good
Persistent Fetal Circulation
  • = PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN
  • = delay in transition from intra- to extrauterine pulmonary circulation
Cause: primary disorder related to birth asphyxia, concurrent parenchymal lung disease (meconium aspiration, pneumonia, pulmonary hemorrhage, hyaline membrane disease, pulmonary hypoplasia), concurrent cardiovascular disease, hypoxic myocardial injury, hyperviscosity syndromes)
  • • labile PO2
  • structurally normal heart
Polyarteritis Nodosa
  • = PERIARTERITIS NODOSA = PAN
  • = systemic fibrinoid necrotizing inflammation of medium-sized + small muscular arteries without glomerulo-nephritis or vasculitis in arterioles, capillaries, venules
Frequency: 4–9 cases/million/year (rare); 70 per million/year in patients with hepatitis B; M:F = 2:1
Etiology: ? deposition of immune complexes
Age: 18–81 (mean age, 55) years
Path: focal panmural necrotizing vasculitis; mucoid degeneration + fibrinoid necrosis begins within media; absence of vasculitis in vessels other than arteries (DDx: necrotizing angitis, mycotic aneurysm)
Histo: polymorphonuclear cell infiltrate in all layers of arterial wall + perivascular tissue (acute phase), mononuclear cell infiltrate, intimal proliferation, thrombosis, perivascular inflammation (chronic stage)
Associated with: hepatitis B + HIV antigenemia
  • low-grade fever, malaise, abdominal pain, weight loss
  • elevated ESR, thrombocytosis, anemia
  • positive for hepatitis B surface antigen (up to 30%)
  • positive perinuclear ANCA titers
Location: all organs may be involved, kidney (70–90%), heart (65%), liver (50–60%), spleen (45%), pancreas (25–35%), GI tract, CNS (cerebrovascular accident, seizure), skin
  • @ Kidney (involved in 70–80–90%)
    • • painless hematuria
    • irregular nephrogram
    • radiolucent cortical areas
    • prolonged washout of contrast material
    • P.650

    • multiple small intrarenal microaneurysms (at bifurcation of interlobar/arcuate arteries)
    • aneurysms may disappear (thrombosis) or appear in new locations
    • arterial narrowing + thrombosis (chronic stage/healing stage)
    • multiple small cortical infarcts
    • CECT:
      • lobulated renal contour + irregular thinning (due to prior cortical infarcts)
      • multiple hypoattenuating bands (arterial occlusion)
    Cx: intrarenal/subcapsular/perinephric hemorrhage (rupture of aneurysm)
  • @ Chest (involved in 70%)
    • • CHF, myocardial infarction
    • cardiac enlargement/pericardial effusion (14%)
    • pleural effusion (14%)
    • pulmonary venous engorgement (21%)
    • massive pulmonary edema (4%)
    • linear densities/platelike atelectasis (10%)
    • wedge-shaped/round peripheral infiltrates of nonsegmental distribution (14%) (simulating thromboembolic disease with infarction)
    • cavitation may occur
    • interstitial lower lung field pneumonitis
  • @ Liver (50–66%)
    • prolonged washout of contrast material (due to increase in peripheral hepatic arterial resistance + hepatic infarcts
  • @ GI tract (50–70%)
    Location: small intestine > mesentery > colon
    • • abdominal pain, nausea, vomiting (66%)
    • ulcer formation, GI bleeding (6%)
    • bowel perforation (5%), intestinal infarction (1.4%)
  • @ Skeletal muscle (39%)
    • • myalgia, arthralgia (50%), limb claudication
    • aneurysms of lumbar + intercostal arteries (19%)
    • lower extremity ischemia (16%)
  • @ Skin (20%)
    • palpable purpura, infection, ischemic ulcer
    • tender subcutaneous nodules (15%)
    • peripheral neuropathy (= mononeuritis multiplex)
Angiography (61–89% sensitive, 90% specific, 55% PPV, 98% NPV, 80% true-positive rate):
  • multiple (>10) aneurysms of small + medium-sized arteries typically at branching points as a result of pannecrosis of the internal elastic lamina in 50–60% (HALLMARK):
    • 1–5 mm saccular aneurysms in 60–75%
    • fusiform aneurysms/arterial ectasia
    • Aneurysms are found in 12–94% of patients with polyarteritis nodosa
  • luminal irregularities (in up to 90%)
  • stenoses of arteries
  • arterial occlusions + organ infarcts (98%)
DDx: rheumatoid vasculitis, drug abuse, systemic lupus erythematosus, Churg-Strauss syndrome
Dx: angiography, tissue biopsy
Cx: renin-mediated hypertension, renal failure, hemorrhage secondary to aneurysm rupture (9%), organ infarction due to vessel thrombosis, gangrene of fingers/toes
Prognosis: clinical course lasts several months to >1 year; relapse in 40% with median interval of 33 months; 13% 5-year survival rate if untreated
Rx: immunosuppression with corticosteroids + cyclophosphamide (increases 5-year survival rate to 48–90%)
Popliteal Artery Entrapment Syndrome
  • = popliteal artery classically winding medially and then inferiorly to the tendinous insertion of the medial head of the gastrocnemius
Incidence: 35 cases in American surgical literature; bilateral in up to 66%
Cause: anomalous development and course of medial head of gastrocnemius muscle, which attaches to medial femoral condyle after development of primitive popliteal artery in 20-mm embryo slinging around lateral aspect of popliteal a.
Pathophysiology:
  • flow unimpeded when muscle relaxed; increased arterial angulation with muscle contraction (early); progressive intimal hyperplasia (“atheroma” = misnomer) due to microtrauma in area of repeated arterial compression; ultimately occlusion/thrombosis within aneurysm (late)
Age: <35 years in 68%; age peaks at 17 and 47 years; M:f = 9:1
  • • slowly progressive intermittent unilateral calf claudication (early) esp. during periods of prolonged standing
  • • acute ischemia of leg with permanent occlusion of popliteal a. (late)
  • posterior tibial pulse obliterated during active plantar flexion against resistance
  • PVR has 40% false-positive results
  • ankle-arm index reduced during active muscle contraction
  • Doppler waveforms of posterior tibial a. diminished during muscle contractions
Angio (biplanar views with hyperextended knee):
  • medial deviation of artery (29%), popliteal stenosis (11%), poststenotic dilatation (8%)
Dx:
  • arteriography with typical medial deviation of popliteal a. before + after gastrocnemius contraction
  • popliteal a. thrombosis/occlusion
Cx: popliteal a. aneurysm
DDx: cystic adventitial disease of popliteal a., arterial embolism, premature arteriosclerosis, popliteal aneurysm with thrombosis, popliteal a. trauma, popliteal a. thrombosis, Buerger disease, spinal cord stenosis (= neurogenic claudication)
Primary Pulmonary Hypertension
  • = PLEXOGENIC PULMONARY ARTERIOPATHY
Diagnosis per exclusion:
  • clinically unexplained progressive pulmonary arterial hypertension without evidence for thromboembolic disease/pulmonary venoocclusive disease
P.651

At risk: portal hypertension (with/without liver disease), collagen vascular disease, HIV infection, aminorex fumarate (appetite suppressant) ingestion
Histo: plexiform + angiomatoid lesions = tortuous channels within “glomeruloid” proliferation of endothelial cells (75%); acute + organizing thrombi (50%)
Age: 3rd decade; M:F = 1:3
  • • gradual onset of progressive dyspnea (60%)
  • • easy fatigability, syncope, angina
  • • hyperventilation, hemoptysis
  • • Raynaud phenomenon
  • right ventricular enlargement (hypertrophy + dilatation)
  • dilatation of central pulmonary arteries
CXR:
  • prominent central pulmonary arteries:
    • enlarged pulmonary trunk
    • right descending pulmonary artery >25 mm wide
  • pulmonary vascularity:
    • oligemia + rapidly tapering vessels
    • overcirculation + vascular distension
CT:
  • enlargement of central pulmonary arteries:
    • diameter of main pulmonary artery > 29 mm (87% sensitive, 89% specific) measured at scan plane of bifurcation at right angle to its long axis just lateral to ascending aorta
    • segmental artery-to-bronchus ratio >1:1
    • pulmonary artery-to-aorta ratio (rPA) >1
  • abruptly diminished caliber of peripheral pulmonary vessels (at outer to medial third of lung mantle)
HRCT:
  • mosaic pattern of lung attenuation (due to regional variations in lung perfusion):
    • hyperdense areas contain large caliber vessels
    • hypodense areas contain small caliber vessels
MR:
  • reversal of interventricular septal curvature
  • direct linear correlation between mean pulmonary artery pressure (PAP) and ratio of main pulmonary artery caliber to descending aorta (MPA/AO)
  • abnormal intravascular signal (due to slow arterial flow caused by pulmonary vascular resistance) in 92% on gated T1WI
NUC:
  • normal/low-probability V/Q scans
Angio:
  • symmetrically enlarged central arteries
  • diffuse pattern of abruptly tapering + pruned subsegmental vessels
  • filamentous/“corkscrew” peripheral arteries
  • subpleural collaterals (occasionally)
Prognosis: death in 2–5 years
Rx: vasodilators, calcium channel blockers, diuretics, anticoagulants; lung/heart-lung transplantation
Pseudocoarctat
  • AORTIC KINKING
  • elongated redundant thoracic aorta with acute kink/anterior buckling just distal to origin of left subclavian artery at lig. arteriosum
  • variant of coarctation without a pressure gradient
Age: 12–64 years
Associated with:
  • hypertension, bicuspid aortic valve, PDA, VSD, aortic/subaortic stenosis, single ventricle, ASD, anomalies of aortic arch branches
  • • asymptomatic
  • • ejection murmur
  • • NO pressure gradient across the buckled segment
  • anteromedial deviation of aorta
  • “chimney-shaped” high aortic arch (in children)
  • rounded/oval soft-tissue mass in left paratracheal region + superior to presumed normally positioned aortic arch [secondary to elongation of ascending aorta + aortic arch] (in adults)
  • anterior displacement of esophagus
  • NO rib notching/dilatation of brachiocephalic arteries/LV enlargement/poststenotic dilatation
Angio:
  • high position of aortic arch
  • “figure 3 sign” = notch in descending aorta at attachment of short ligamentum arteriosum
DDx: true coarctation, aneurysm, mediastinal mass
Pulmonary Artery Pseudoaneurysm
tear/disruption of layers of vessel wall with extravasation of blood contained by adventitia/clot/compressed surrounding tissue
Incidence: rare
Cause:
  • TRAUMA
    • Improper placement of Swan-Ganz catheter
    • Penetrating/blunt (rare) trauma
  • INFECTION:
    • Mycotic aneurysm (endovascular seeding from endocarditis, direct extension from necrotizing pneumonia)
    • Mycobacterial aneurysm (Rasmussen aneurysm)
    • Syphilitic aneurysm
  • VASCULAR ABNORMALITY: cystic medial necrosis, Behçet disease, Marfan syndrome, Takayasu disease
  • OTHER: septic emboli, neoplasm
Associated with: CHD (patent ductus arteriosus)
  • • hemoptysis (= leakage of blood into bronchial tree)
CXR:
  • stable/increasing focal lung mass
CT:
  • enhancing round lung mass isointense to central pulmonary artery
Cx: 100% mortality with rupture
Pulmonary Atresia
  • = CONGENITAL ABSENCE OF PULMONARY ARTERY
  • = atretic pulmonary valve with underdeveloped pulmonary artery distally
May be associated with: hypogenetic lung
CXR:
  • small hemithorax of normal radiodensity
  • mediastinal shift to affected side
  • elevation of ipsilateral diaphragm
  • reticular network of vessels on affected side (due to systemic collateral circulation from bronchial arteries)
  • P.652

  • rib notching from prominence of intercostal arteries (due to large transpleural collateral vessels)
OB-US:
  • small/enlarged/normal right ventricle
  • progressive atrial enlargement (tricuspid regurgitation)
  • flow reversal in ductus arteriosus + main pulmonary artery (most reliable)
Pulmonary Atresia with Intact Interventricular Septum
Associated with: ASD (R-to-L shunt)
Type I: no remaining RV, no tricuspid regurgitation
√ moderately enlarged RA (depending on size of ASD)
Type II: normal RV with tricuspid regurgitation
√ massive enlargement of RA
  • cardiomegaly (LV, RA)
  • concave/small pulmonary artery segment
  • diminished pulmonary vascularity
Pulmonary Venoocclusive Disease
= fibrous narrowing of intrapulmonary veins; the postcapillary counterpart of primary pulmonary hypertension
Cause: idiopathic (rare condition); venous thrombosis initiated by infection/toxic exposure/immune complex deposition
May be associated with:
  • pregnancy, transplantation, drug toxicity (carmustine, bleomycin, mitomycin)
Hemodynamics:
  • elevated pressure in right atrium + pulmonary artery
  • decreased cardiac output
  • normal/variably elevated capillary wedge pressures
  • normal pressure in left atrium + left ventricle (excludes cardiac disease as the cause for venous hypertension)
Age: children (33%), adolescents; M:F = 1:1
Histo:
  • specific changes: webs, recanalized thrombus (in up to 95%), intimal fibrosis of pulmonary veins; “capillary hemangiomatosis” = sheets and nodular collections of thin-walled capillaries invading pulmonary arteries + veins + bronchioles + pleura
  • nonspecific changes of venous hypertension: venous medial hypertrophy, septal edema + fibrosis, paraseptal venous infarction, interstitial + pleural lymphatic dilatation, intraalveolar hemosiderin-laden macrophages
  • progressive dyspnea, hemoptysis
  • antecedent flulike symptoms
CXR:
  • pulmonary arterial hypertension
  • diffuse interstitial pulmonary edema
  • normal-sized left atrium
  • mediastinal lymphadenopathy
CT:
  • markedly small central pulmonary veins
  • central and gravity-dependent parenchymal ground-glass attenuation
  • smoothly thickened interlobular septa
  • pleural effusions
  • normal-sized left atrium
  • centrilobular nodules
NUC:
  • patchy distribution of Tc-99m MAA (of “upstream” pulmonary arterial hypertension)
Angio:
  • enlarged right ventricle + central pulmonary arteries
  • prolonged parenchymal phase enhancement
  • delayed filling of normal pulmonary veins
  • normal to small left atrium
Prognosis: death within 3 years (no effective therapy)
Cx: potentially fatal pulmonary edema following administration of vasodilators for presumed precapillary pulmonary hypertension
Dx: often missed initially (clinical presentation + radiographic findings mimic interstitial lung disease)
Pulmonic Stenosis
Frequency: pulmonary artery stenosis without VSD in 8% of all CHD
Embryology: infundibulum formed from proximal portion of bulbis cordis; pulmonary valves develop in 6–9th week from outgrowth of 3 tubercles
  • • mostly asymptomatic
  • • cyanosis/heart failure
  • • loud systolic ejection murmur
  • systolic doming of pulmonary valve (= incomplete opening)
  • normal/diminished/increased pulmonary vascularity (depending on presence + nature of associated malformations)
  • enlarged pulmonary trunk + left pulmonary artery (poststenotic dilatation)
  • prominent left pulmonary artery + normal right pulmonary artery
  • hypertrophy of RV with reduced size of RV chamber:
    • elevation of cardiac apex
    • increased convexity of anterior cardiac border on LAO
    • diminution of retrosternal clear space
  • cor pulmonale
  • mild enlargement of LA (reason unknown)
  • calcification of pulmonary valves in older adults (rare)
Prognosis: death at mean age of 21 years if untreated
Subvalvular Pulmonic Stenosis
  • INFUNDIBULAR PULMONIC STENOSIS
    • typically in tetralogy of Fallot
  • SUBINFUNDIBULAR PULMONIC STENOSIS
    • = hypertrophied anomalous muscle bundles crossing portions of RV
Associated with: VSD (73–85%)
(a) low type: courses diagonally from low anterior septal side to crista posteriorly
(b) high type: horizontal defect across RV below infundibulum
  • no dilatation of PA because of dissipation of RV force through elongated area of obstruction
Valvular Pulmonic Stenosis
  • CLASSIC/TYPICAL PULMONIC VALVE STENOSIS (95%)
    • = commissural fusion of pulmonary cusps
      Age of presentation: childhood
    • P.653

    • •pulmonic click
    • •ECG: hypertrophy of RV
    • thin mobile dome-shaped valve
    • jet of contrast through small central orifice
    • dilated main + left pulmonary artery
    Rx: balloon valvuloplasty
  • DYSPLASTIC PULMONIC VALVE STENOSIS (5%)
    • = thickened redundant distorted cusps, immobile secondary to myxomatous tissue
    • • NO click
    • NO poststenotic dilatation
      Rx: surgical resection of redundant valve tissue
      Hemodynamics: obstruction of RV systolic ejection with pressure burden on RV
      image
    • CXR:
    • normal pulmonary vascularity
    • normal-sized heart
    Angio:
    • increase in trabecular pattern of RV
    • hypertrophied crista supraventricularis (lateral projection)
Supravalvular Pulmonic Stenosis
  • 60% of all pulmonic valve stenoses
Site of narrowing: pulmonary trunk, pulmonary bifurcation, one/both main pulmonary arteries, lobar pulmonary artery, segmental pulmonary artery
Shape of narrowing:
  • localized with poststenotic dilatation
  • long tubular hypoplasia
May be associated with:
  • Valvular pulmonic stenosis, supravalvular aortic stenosis, VSD, PDA, systemic arterial stenoses
  • Familial peripheral pulmonic stenoses + supravalvular aortic stenosis
  • Williams-Beuren syndrome: PS, supravalvular AS, peculiar facies
  • Ehlers-Danlos syndrome
  • Postrubella syndrome: peripheral pulmonic stenoses, valvular pulmonic stenosis, PDA, low birth weight, deafness, cataract, mental retardation
  • Tetralogy of Fallot/critical valvular pulmonic stenosis
Peripheral Pulmonary Artery Stenosis
Frequency: 5% of all pulmonary artery stenoses with an intact ventricular septum
Raynaud syndrome
  • episodic digital ischemia in response to cold/emotional stimuli
  • Pathogenesis:
    • increase in vasoconstrictor tone
    • low blood pressure
    • slight increase in blood viscosity
    • immunologic factors (4–81%)
    • cold provocation
    • exaggerated response of digit to cold/emotional stress:
      • numbness + loss of tactile perception
      • demarcated pallor/cyanosis
    • hyperemic throbbing during rewarming
    • sclerodactyly
    • small painful ulcers at tip of digit
Raynaud Disease
  • = PRIMARY VASOSPASM = SPASTIC FORM OF RAYNAUD SYNDROME
  • = exaggerated cold-induced constriction of smooth muscle cells in otherwise normal artery
Cause: ? acquired adrenoreceptor hypersensitivity
May be associated with: reflex sympathetic dystrophy, early stages of autoimmune disorders
Age: most common in young women
  • • usually affects all fingers of both hands equally
  • normal segmental arm + digit pressures at room temperature
  • peaked digit volume pulse = rapid rise in systole, anacrotic notch just before the peak, dicrotic notch high on the downslope
PPG:
  • flat-line tracing at low temperatures (10°–22°C) with sudden reappearance of normal waveform at 24–26°C = “threshold phenomenon”
Raynaud Phenomenon
  • = SECONDARY VASOSPASM WITH OBSTRUCTION = OBSTRUCTIVE FORM OF RAYNAUD SYNDROME
  • = digital artery occlusion due to stenotic process in normally constricting artery/associated with an abnormally high blood viscosity
  • Cause:
    • Atherosclerosis (most frequent)
      • embolization from an upstream lesion
      • occlusion of major arteries supplying arm
    • Arterial trauma
    • End stage of many autoimmune disorders: eg, scleroderma, rheumatoid arthritis, systemic lupus erythematosus
    • Takayasu disease
    • Buerger disease
    • Drug intoxication (ergot, methysergide)
    • Dysproteinemia
    • Primary pulmonary hypertension
    • Myxedema
  • • normal vasoconstrictive response to cold
  • reduced segmental arm + digit pressures at room temperature
  • PPG (76% sensitivity, 92% specificity):
    • flat-line/barely detectable tracing at low temperature with gradual increase of amplitude upon rewarming
  • Hand magnification angiography:
    • Baseline angiogram with ambient temperature
    • Stress angiogram immediately following immersion of hand in ice water for 20 seconds
Rhabdomyoma Of Heart
= benign myocardial hamartoma
P.654

Prevalence: most common cardiac tumor in infancy + childhood (up to 90%)
Age: usually discovered <1 year of age
Path: well-circumscribed intramural lobulated nodule/multiple <1 mm nodules (= rhabdomyomatosis)
Histo: “spider cells” = enlarged vacuolated cells with high glycogen content + central nucleus surrounded by clear cytoplasm and radial extensions
Associated with: tuberous sclerosis (in 50–86%); congenital heart disease
  • asymptomatic (incidental detection at prenatal US/screening)
  • murmur, arrhythmia
  • heart failure (secondary to obstruction of outflow tract/reduction of enddiastolic volume/decreased contractility)
  • supraventricular tachycardia (accessory conductive pathways within tumor)
Location: usually multiple; ventricular wall with intramural growth + tendency to involve interventricular septum; atrial wall (rare)
Size: up to 10 cm in diameter (average 3–4 cm)
US (good for small intramural lesions):
  • fetal nonimmune hydrops
  • solid echogenic sessile mass ±intracavitary component bulging into ventricular outflow tract/atrioventricular valve
  • diffuse myocardial thickening (with multiple small lesions)
MR (complimentary to US):
  • tumor isointense on T1WI + hyperintense to myocardium on T2WI
Prognosis: may regress spontaneously in patients <4 years of age
Rx: surgical excision for life-threatening symptoms
DDx: fibroma (solitary centrally calcified + cystic tumor, in ventricular myocardium, associated with Gorlin syndrome), teratoma (single intrapericardial multicystic mass), hemangioma (arise from right atrium, pericardial effusion, skin hemangiomas)
Single Ventricle
  • univentricular HEART = DOUBLE INLET SINGLE VENTRICLE
  • failure of development of interventricular septum ±absence of one atrioventricular valve (mitral/tricuspid atresia) ±aortic/pulmonic stenosis
Associated with: TGV or DORV
  • • conduction defect (aberrant anatomy of conduction system)
  • two atrioventricular valves connected to a main ventricular chamber
  • the single ventricle may be a LV (85%)/RV/undetermined
  • a second rudimentary ventricular chamber may be present, which is located anteriorly (in left univentricle)/posteriorly (in right univentricle):
    • rudimentary chamber ±connection to one great artery
  • may be associated with tricuspid/mitral atresia
Sinus Of Valsalva Aneurysm
deficiency between aortic media + annulus fibrosis of aortic valve resulting in distension + eventual aneurysm formation
Age: puberty to 30 years of age
Site: right sinus/noncoronary sinus (>90%)
♢ Right sinus usually ruptures into RV, occasionally into RA
♢ Noncoronary sinus ruptures into RA
  • • sudden retrosternal pain, dyspnea, continuous murmur
  • shunt vascularity
  • cardiomegaly
  • prominent ascending aorta
Splenic Artery Aneurysm
= most frequent of visceral artery aneurysms
Etiology: medial degeneration with superimposed atherosclerosis, congenital, mycotic, pancreatitis, trauma, portal hypertension (7–10% of cases due to high flow rate)
Predisposed: women with ≥2 pregnancies (88%)
May be associated with: fibromuscular disease (in 20%)
M:F = 1:2
  • • usually asymptomatic
  • • pain, GI bleeding
Location: intra-/extrasplenic
  • calcified wall of aneurysm (2/3)
Cx: rupture of aneurysm (6–9%, higher during pregnancy) especially if >1.5 cm in diameter
Mortality: up to 76%
DDx: renal artery aneurysm, tortuous splenic artery
Subclavian Steal Syndrome
= stenosis/obstruction of subclavian artery near its origin with flow reversal in ipsilateral vertebral artery at the expense of the cerebral circulation
Incidence: 2.5% of all extracranial arterial occlusions
Etiology:
  • congenital: interruption of aortic arch, preductal infantile coarctation, hypoplasia of left aortic arch, hypoplasia/atresia/stenosis of an anomalous left subclavian artery with right aortic arch, coarctation with aberrant subclavian artery arising distal to the coarctation
  • acquired: atherosclerosis (94%), dissecting aneurysm, chest trauma, embolism, tumor thrombosis, inflammatory arteritis (Takayasu, syphilitic), ligation of subclavian artery in Blalock-Taussig shunt, complication of coarctation repair, radiation fibrosis
Age: average 59–61 years; M:F = 3:1; Whites:Blacks = 8:2
Associated with: additional lesions of extracranial arteries in 81%
  • lower systolic blood pressure by >20–40 mm Hg on affected side
  • delayed weak/absent pulse in ipsilateral extremity
  • signs of vertebrobasilar insufficiency (40%):
    • syncopal episodes initiated by exercising the ischemic arm
    • headaches, nausea, vertigo, ataxia
    • mono-, hemi-, para-, quadriparesis, paralysis
    • diplopia, dysphagia, dysarthria, paresthesias around mouth
    • uni-/bilateral homonymous hemianopia
  • signs of brachial insufficiency (3–10%):
    • intermittent/constant pain in affected arm precipitated by increased activity of that arm
    • paresthesia, weakness, coolness, numbness, burning in fingers + hand
    • P.655

    • fingertip necrosis
Location: L:R = 3:1
Color Doppler:
  • reversal of vertebral artery flow, augmented by reactive hyperemia (blood pressure cuff inflated above systolic pressure for 5 minutes)/arm exercise
Angio:
  • subclavian stenosis/occlusion (aortic arch injection)
  • reversal of vertebral artery flow (selective injection of contralateral subclavian/vertebral artery)
    CAVE: “false steal” = transient retrograde flow in contralateral vertebral artery caused by high-pressure injection
Rx: bypass surgery, PTA (good long-term results)
Partial Subclavian Steal Syndrome
  • = retrograde flow in systole + antegrade flow in diastole
Occult Subclavian Steal Syndrome
  • = reverse flow seen only after provocative maneuvers, ie, ipsilateral arm exercise of 5 minutes/5 minutes inflation of sphygmomanometer > systolic blood pressure levels
Superior Vena Cava Syndrome
  • = obstruction of SVC with development of collateral pathways
  • Etiology:
    • Malignant lesion (80–90%)
      • Bronchogenic carcinoma (>50%)
      • Lymphoma
    • Benign lesion
      • Granulomatous mediastinitis (usually histoplasmosis, sarcoidosis, TB)
      • Substernal goiter
      • Ascending aortic aneurysm
      • Pacer wires/central venous catheters (23%)
      • Constrictive pericarditis
  • Collateral routes:
    • Esophageal venous plexus = “downhill varices” (predominantly upper 2/3)
    • Azygos + hemiazygos veins
    • Accessory hemiazygos + superior intercostal veins = “aortic nipple” (visualization in normal population in 5%)
    • Lateral thoracic veins + umbilical vein
    • Vertebral veins
  • • head and neck edema (70%)
  • • cutaneous enlarged venous collaterals
  • • headache, dizziness, syncope
  • • with benign etiology: slower onset + progression, both sexes, 25–40 years of age
  • • with malignancy: rapid progression within weeks, mostly males, 40–60 years of age
  • • proptosis, tearing
  • • dyspnea, cyanosis, chest pain
  • • hematemesis (11%)
  • superior mediastinal widening (64%)
  • encasement/compression/occlusion of SVC
  • dilated cervical + superficial thoracic veins (80%)
  • SVC thrombus
  • NUC:
    • increased tracer uptake in quadrate lobe + posterior aspect of medial segment of left lobe (umbilical pathway toward liver when injected in upper extremity)
Syphilitic Aortitis
LUETIC AORTITIS
Incidence: in 10–15% of untreated patients (accounts for death in 1/3)
Path: periaortitis (via lymphatics), mesaortitis (via vasa vasorum) = primarily disease of media leading to secondary injury of intima, which predisposes the intima to premature calcific atherosclerosis
Age: between 40 and 65 years
Site: ascending aorta (36%), aortic arch (24%), descending aorta (5%), sinus of Valsalva (1%), pulmonary artery
  • thick aortic wall (fibrous + inflammatory tissue)
  • saccular (75%)/fusiform (25%) dilatation of ascending aorta
  • small saccular aneurysms often protrude from fusiform aneurysm
  • fine pencil-like calcifications of intima (15–20%) in ascending aorta, late in disease
Cx: (1) stenosis of coronary ostia (intimal thickening)
(2) aortic regurgitation (syphilitic valvulitis), rare
DDx: degenerative calcification of ascending aorta (older population, no aneurysm, no aortic regurgitation)
Takayasu Arteritis
  • = PULSELESS DISEASE = AORTITIS SYNDROME
    • = AORTOARTERITIS = IDIOPATHIC MEDIAL AORTOPATHY
    • = AORTIC ARCH SYNDROME
  • = granulomatous inflammation of unknown pathogenesis affecting mainly elastic arteries, i.e., segments of aorta + major aortic branches + pulmonary arteries limited to persons usually <50 years of age
  • the only form of aortitis that produces stenosis/occlusion of the aorta!
Etiology: probably cell-mediated inflammation
Incidence: 2.6 new cases/million/year; 2.2% (at autopsy)
Age: 12–66 years; M:f = 1:8; especially in Orientals
Histo: (a) Acute stage: granulomatous infiltrative process focused on elastic fibers of media of arterial wall consisting of multinucleated giant cells, lymphocytes, histiocytes, plasma cells
(b) fibrotic stage (weeks to years): progressive fibrosis of vessel wall resulting in constriction from intimal proliferation/thrombotic occlusion/aneurysm formation (from extensive destruction of elastic fibers in the media); ultimately leads to fibrosis of intima + adventitia
♢Morphologically indistinguishable from temporal arteritis!
  • • prepulseless/systemic phase of a few months to a year
    • = nonspecific systemic signs + symptoms of fever, night sweats, weakness, weight loss, myalgia, arthralgia
    • Mean interval of 8 years between onset of symptoms and diagnosis
  • • pulseless phase = signs + symptoms of ischemia of
    • limb (claudication, pulse deficit, bruits) + renovascular hypertension
  • P.656

  • • erythrocyte sedimentation rate (eSR) >20 mm/hour in 80%
Location:
Type I: classic pulseless type = brachiocephalic trunk + carotid arteries + subclavian arteries
Type II: combination of type I + III
Type III: atypical coarctation type = thoracic and abdominal aorta distal to arch + its major branches
Type IV: dilated type = extensive dilatation of the length of the aorta + its branches
  • Commonly involved: left subclavian artery (<50%), left common carotid artery (20%), brachiocephalic trunk, renal arteries, celiac trunk, superior mesenteric artery, pulmonary arteries (>50%)
  • Infrequently involved: axillary, brachial, vertebral, iliac arteries (usually bilaterally), coronary arteries
Angiography:
  • difficult catheterization/risk of ischemic complications (increase in coagulation)
  • arterial wall thickening + contrast enhancement
  • full-thickness calcification (chronic disease)
  • mural thrombi
CXR:
  • widened supracardiac shadow >3.0 cm
  • wavy/scalloped appearance of lateral margin of descending aorta
  • aortic calcification (15%) commonly in aortic arch + descending aorta
  • focal decrease of pulmonary vascularity
  • @ Aorta
    • long + diffuse/short + segmental irregular stenosis/occlusion of major branches of aorta near their origins
    • stenotic lesions of descending thoracic aorta > abdominal aorta
    • frequent skipped lesions
    • abundant collateralization (late phase)
    • aneurysmal dilatation of ascending aorta + arch
      • = diffusely dilated lumen with irregular contours
    • fusiform/saccular aortic aneurysms (10–15%) (common in descending thoracic + abdominal aorta)
  • @ Brachiocephalic arteries
    • multisegmented dilatation of carotid artery producing segmental septa
    • diffuse homogeneous circumferential thickening of vessel wall in proximal common carotid artery
    • increase in flow velocity + turbulence
    • distal CCA, ICA, ECA spared with dampened waveforms
  • @ Pulmonary arteries (50–80%)
    Location: segmental + subsegmental (common);
    lobar + main pulmonary arteries (uncommon)
    • often late manifestation of disease
    • dilatation of pulmonary trunk (19%)
    • nodular thrombi (3%)
    • “pruned tree” appearance of pulmonary arteries (66%)
    • systemic-pulmonary artery shunts
    • CT angio:
      • wall thickening with enhancement (acute)
      • luminal + mural calcium deposition (chronic)
      • pulmonary artery stenosis/occlusion (chronic)
    Cx: (1) Cerebrovascular accidents
    (2) Heart failure due to aortic regurgitation
    DDx: atherosclerosis, temporal arteritis (CCA not involved), fibromuscular dysplasia (in ICA not CCA), idiopathic carotid dissection (ICA), syphilitic aortitis (calcification of ascending aorta)
    Rx: steroids, angioplasty after decline of active inflammation
Temporal Arteritis
  • = CRANIAL/GRANULOMATOUS ARTERITIS
  • = POLYMYALGIA RHEUMATICA = GIANT CELL ARTERITIS (poor choice because Takayasu disease is also a giant cell arteritis)
  • = systemic granulomatous vasculitis limited to persons usually >50 years of age
Incidence: 1.7 new cases/million/year
Histo:
  • acute stage: granulomatous infiltrative process focused on elastic fibers of arterial wall consisting of multinucleated giant cells, lymphocytes, histiocytes, plasma cells
  • fibrotic stage (weeks to years): progressive fibrosis of vessel wall resulting in constriction from intimal proliferation/thrombotic occlusion/aneurysm formation
  • Morphologically indistinguishable from Takayasu arteritis!
Age peak: 65–75 years; M:F = 1:3
  • prodromal phase of flulike illness of 1–3 weeks:
    • malaise, low-grade fever, weight loss, myalgia
    • unilateral headache (50–90%)
  • chronic stage:
    • jaw claudication (while chewing + talking)
    • palpable tender temporal artery
    • neuroophthalmic manifestations: visual impairment /diplopia/blindness
    • polymyalgia rheumatica (50%) = intense myalgia of shoulder + hip girdles
  • erythrocyte sedimentation rate (ESR) of 40–140 mm/ hour (HALLMARK)
Location: any artery of the body; mainly medium-sized branches of aortic arch (10%), external carotid artery branches (particularly temporal artery); extracranial arteries below neck (9%): subclavian > axillary > brachial > profunda femoris > forearm > calf; commonly bilateral + symmetric
  • long smooth stenotic arterial segments with skip areas
  • smooth tapered occlusions with abundance of collateral supply
  • absence of atherosclerotic changes
  • aortic root dilatation + aortic valve insufficiency
Dx: biopsy of palpable temporal artery
Prognosis: disease may be self-limiting (1–2 years); 10% mortality within 2–3 years
Tetralogy Of Fallot
underdevelopment of pulmonary infundibulum secondary to unequal partitioning of the conotruncus
Frequency: 8% of all CHD; most common CHD with cyanosis after 1 year of life
TETRAD:
  • Obstruction of right ventricular outflow tract: usually of pulmonary infundibulum, occasionally of pulmonic valve
  • Large VSD immediately below aortic valve
  • Right ventricular hypertrophy secondary to elevated RV systolic pressure
  • P.657

  • Overriding aorta straddling the VSD and receiving blood from both ventricles
Embryology:
  • abnormal spiraling caudad growth of truncoconal ridges in 3rd–4th week causing unequal partitioning of the conotruncus into a small underdeveloped anteromedial pulmonary infundibulum + large posterolateral LV outflow tract
Hemodynamics:
fetus: pulmonary blood flow supplied by retrograde flow through ductus arteriosus with absence of RV hypertrophy/IUGR
neonate: R-to-L shunt bypassing pulmonary circulation with decrease in systemic oxygen saturation (cyanosis); pressure overload + hypertrophy of RV secondary to pulmonic-infundibular stenosis
image
Associated with:
  • Bicuspid pulmonic valve (40%)
  • Stenosis of left pulmonary artery (40%)
  • Right aortic arch (25%)
  • TE fistula
  • Down syndrome
  • Forked ribs, scoliosis
  • Anomalies of coronary arteries in 10% (single RCA/LAD from RCA)
  • • cyanosis by 3–4 months of age (concealed at birth by PDA)
  • • dyspnea on exertion, clubbing of fingers and toes
  • • “squatting position” when fatigued (increases pulmonary blood flow)
  • • “episodic spells” = loss of consciousness
  • • polycythemia, lowered PO2 values, systolic murmur in pulmonic area
  • coeur en sabot (boot-shaped heart) = enlargement of right ventricle
  • pronounced concavity in region of pulmonary artery trunk (small/absent PA)
  • marked reduction in caliber + number of pulmonary vessels:
    • asymmetric pulmonary vascularity
    • reticular pattern with horizontal course usually in periphery (= prominent collateral circulation of bronchial vessels + pleuropulmonary connections)
  • enlarged aorta
  • right-sided aortic arch in 25%
OB-US:
  • dilated aorta overriding the interventricular septum
  • usually perimembranous VSD
  • mildly stenotic RV outflow tract
  • NO RV hypertrophy in midtrimester
ECHO:
  • discontinuity between anterior aortic wall + interventricular septum (= overriding of the aorta)
  • small left atrium
  • RV hypertrophy with small right ventricular outflow tract
  • widening of the aorta
  • thickening of right ventricular wall + interventricular septum
Prognosis: spontaneous survival without surgical correction in 50% up to age 7; in 10% up to age 21
Rx: surgery in early childhood
  • palliative
    • Blalock-Taussig shunt = end-to-side anastomosis of subclavian to pulmonary artery opposite aortic arch (64% survival rate at 15 years, 55% at 20 years)
    • Pott operation on left = anastomosis of left PA with descending aorta
    • Waterston-Cooley procedure = anastomosis between ascending aorta + right pulmonary artery
    • Central shunt = Rastelli procedure = tubular synthetic graft between ascending aorta + pulmonary artery
  • corrective open cardiac surgery = VSD-closure + reconstruction of RV outflow tract by excision of obstructing tissue (82% survival rate at 15 years)
Operative mortality: 3–10%
Pink Tetralogy
  • = infundibular hypertrophy in VSD (3%)
Pentalogy of Fallot
  • = tetralogy + ASD
Trilogy of Fallot (infantile presentation)
  • Severe pulmonic valvular stenosis
  • Hypertrophy of RV
  • ASD with R-to-L shunt (increased pressure in RA forces foramen ovale open)
Thoracic Outlet Syndrome
  • = compression of nerves, veins, and arteries between chest and arm
Cause:
  • CONGENITAL
    • Cervical rib
    • Scalenus minimus muscle (rare) extending from transverse process of 7th cervical vertebra to 1st rib with insertion between brachial plexus + subclavian artery
    • Anterior scalene muscle = scalenus anticus syndrome (most common) = wide/abnormal insertion/hypertrophy of muscle
    • Anomalous 1st rib = unusually straight course with narrowing of costoclavicular space
  • ACQUIRED
    • Muscular body habitus
      • = arterial compression in pectoralis minor tunnel
    • Slender body habitus
      • with long neck, sagging shoulders
    • Fracture of clavicle/1st rib (34%)
      • with nonanatomic alignment/exuberant callus
    • Supraclavicular tumor/lymphadenopathy
    • pain in forearm + hand that increases upon elevation of arm
    • paresthesias of hand + fingers (numbness, “pins and needles”) in 95%
    • decreased skin temperature, discoloration of hand
    • intermittent claudication of fingers (from ischemia)
    • hyperabduction maneuver with obliteration of radial pulse (34%)
    • P.658

    • Raynaud phenomenon (40%): episodic constriction of small vessels
    • supraclavicular bruit (15–30%)
Bidirectional Doppler:
  • Adson maneuver (for scalenus anticus muscle) = hold deep inspiration while neck is fully extended + head turned toward ipsilateral and opposite side
  • Costoclavicular maneuver (compression between clavicle + 1st rib) = exaggerated military position with shoulders drawn back and downward
  • Hyperabduction maneuver (compression by humeral head/pectoralis minor muscle) = extremity monitored through range of 180° abduction
  • complete cessation of flow in one position
Photoplethysmography:
  • Photo pulse transducer secured to palmar surface of one fingertip of each hand
  • Arterial pulsations recorded with arm in
    • neutral position
    • extended 90° to side
    • 180° over the head
    • in “military” position with arms at 90° + shoulders pressed back
  • complete disappearance of pulse in one position
Angio:
  • abnormal course of distal subclavian artery
  • focal stenosis/occlusion
  • poststenotic dilatation of distal subclavian artery
  • aneurysm
  • stress test: bandlike/concentric constriction
  • mural thrombus ±distal embolization
  • venous thrombosis/obstruction
DDx: Cervical disk disease, radiculopathy, spinal cord tumor, trauma to brachial plexus, arthritis, carpal tunnel syndrome, Pancoast tumor, peripheral arterial occlusive disease, aneurysm, causalgia, thromboembolism, Raynaud disease, vasculitis
Transposition Of Great Arteries
Complete Transposition of Great Arteries
  • = TGA = D-TRANSPOSITION
  • = great vessels originate from inappropriate ventricle:
    • aorta originating from RV with an infundibulum
    • pulmonary artery originating from LV
    • normal position of atria + ventricles
Embryology:
  • failure of the aorticopulmonary septum (= truncoconal ridges) to follow a spiral course
Incidence: 10% of all CHD
VARIATIONS:
  • Complete TGA + intact interventricular septum
  • Complete TGA + VSD: CHF due to VSD
  • Complete TGA + VSD + PS: PS prevents CHF = longest survival
Hemodynamics:
fetus: no hemodynamic compromise with normal birth weight
neonate: mixing of the 2 independent circulations necessary for survival
Admixture of blood from both circulations via:
  • PDA (carries aortic blood into pulmonary artery) + patent foramen ovale (allows saturated blood to enter RA from LA)
    Prognosis: worst when PDA closes
  • VSD (in 50%)
image
  • cyanosis (most common cause for cyanosis in neonate) 2nd most common cause of cyanosis after tetralogy of Fallot
  • symptomatic 1–2 weeks following birth
CXR:
  • “egg-on-its-side” appearance of heart = narrow superior mediastinum secondary to hypoplastic thymus + hyperaeration + abnormal relationship of great vessels
  • cardiac enlargement beginning 2 weeks after birth
  • right heart enlargement
  • enlargement of LA (with VSD)
  • absent pulmonary trunk (99%) = PA located posteriorly in midline
  • increased pulmonary blood flow (if not associated with PS)
  • midline aorta (30%)/ascending aorta with convexity to the right
  • right aortic arch in 3% (difficult assessment due to midline position + small size)
OB-US:
  • great arteries arise from ventricles in a parallel fashion
  • aorta anterior + to right of pulmonary artery (in 60%; rarely side by side)
Prognosis: overall 70% survival rate at 1 week, 50% at 1 month, 11% at 1 year by natural history
image
Variation of Transposition
Rx:
  • Prostaglandin E1 administration to maintain ductal patency
  • Rashkind procedure = balloon septostomy to create ASD
  • Blalock-Hanlon procedure = surgical creation of ASD
  • P.659

  • Mustard operation (corrective) = removal of atrial septum + creation of intraatrial baffle directing the pulmonary venous return to RV + systemic venous return to LV; 79% 1-year survival rate; 64–89% 5-year survival
Corrected Transposition of Great Arteries
  • = CONGENITALLY CORRECTED TRANSPOSITION = L-TRANSPOSITION = VENTRICULAR INVERSION
  • = anomalous looping of the bulboventricular loop
    • (= primordial ventricles) associated with lack of spiral rotation of conotruncal septum characterized by
    • Transposition of great arteries (= aorta anterior + to right of PA)
    • Inversion of ventricles (LV on right side, RV on left side):
      • RA connected to morphologic LV
      • LA connected to morphologic RV
    • AV valves + coronary arteries follow their corresponding ventricles
Hemodynamics: functionally corrected abnormality
Associated with:
  • usually perimembranous VSD (in >50%)
  • pulmonic stenosis (in 50%)
  • anomaly of left (= tricuspid) atrioventricular valves (Ebstein-like) leading to insufficiency
  • dextrocardia (high incidence)
  • NO cyanosis
  • atrioventricular block (malalignment of atrial + ventricular septa)
CXR:
  • abnormal convexity/straightening in upper portion of left heart border (ascending aorta arising from inverted RV)
  • inapparent aortic knob + descending aorta (overlying spine)
  • inapparent pulmonary trunk (rightward posterior position) = PREMIER SIGN
  • humped contour of lower left heart border with elevation above diaphragm (anatomic RV)
  • apical notch (= septal notch)
  • increased pulmonary blood flow (if shunt present)
  • pulmonary venous hypertension (if left-sided AV valve incompetent)
  • LA enlargement
MR:
  • posterior cardiac chamber has moderator band + muscular infundibulum (morphologic RV)
Angio:
  • original LV on right side: smooth-walled, cylinder-/cone-shaped with high recess emptying into aorta (= venous ventricle)
  • original RV on left side: bulbous, triangular shape, trabeculated chamber with infundibular outflow tract into pulmonary trunk (= arterial ventricle)
OB-US:
  • great arteries arise from ventricles in a parallel fashion
  • aortic valve separated from tricuspid valve by a complete infundibulum
  • fibrous continuity between pulmonic valve + mitral valve
Prognosis: (unfavorable secondary to additional cardiac defects) 40% 1-year survival rate, 30% 10-year survival rate
Traumatic Aortic Injury
  • = AORTIC LACERATION = BLUNT TRAUMA TO THORACIC AORTA
  • = laceration that disrupts the physical integrity of >1 structural layers of the aorta
Incidence: >100,000 people in United States/year
Cause: rapid deceleration (high-speed MVA >48 km/h with unrestrained driver or ejected passenger, fall from height >3 m)/crushing chest injury
Pathomechanism: horizontal/vertical deceleration, hydrostatic force, osseous pinch
Length of tear: circumferential tear (in majority)
Site: (a) Aortic isthmus just distal to left subclavian artery (88–95%): brachiocephalic arteries + ligamentum arteriosum fix aorta in this region
(b) Aortic arch with avulsion of brachiocephalic trunk (4.5%)
(c) Ascending aorta immediately above aortic valve (5–9%)
      Cx: aortic valve rupture, coronary artery laceration, hemopericardium + cardiac tamponade; NO mediastinal hematoma
(d) Diaphragmatic hiatus (1–3%)
♢ Most often posteriorly (in noncircumferential tear)
Extent of laceration:
  • Incomplete rupture (15%)
    • Aorta goes on to rupture completely within 24 hours in 50% of patients!
    • — INTIMA
      • (a) intimal hemorrhage without tear
      • (b) transverse laceration of intima with hemorrhage (= intimal tear/flap = traumatic aortic dissection)
      • Minimal aortic injury (10%) = intimal flap of <10 mm without significant periaortic hematoma
    • — MEDIA
      • tear into media with subadventitial hematoma (40–60%)
    • — ADVENTITIA
      • periaortic hemorrhage (±aortic injury)
      • traumatic pseudoaneurysm = laceration of intima + media + adventitia with locally contained periadventitial hematoma
  • Complete rupture (85%) = transmural extension of laceration = aortic transection = traumatic aortic rupture
    • exsanguination before reaching a hospital
Acute Thoracic Aortic Injury
Prevalence: 10–16–20% of all fatalities in high-speed deceleration accidents
  • severe chest pain: precordial (ascending aorta), neck-jaw (aortic arch), interscapular (descending thoracic aorta)
  • anterior chest wall contusion, dyspnea, dysphagia
  • blood pressure changes:
    • unexplained hypotension
    • P.660

    • scapulothoracic syndrome = decreased/absent upper extremity pulses
    • acute coarctation syndrome = decreased/absent lower extremity + normal upper extremity pulses with upper extremity hypertension + systolic murmur in 2nd left parasternal interspace
CXR (53–100% sensitive, 1–60% specific, 4–20% PPV):
  • A normal anteroposterior upright CXR virtually excludes acute thoracic aortic injury (96–98% negative predictive value)!
N.B.: There are no plain CXR findings of aortic injury (since aortic integrity is maintained by intact adventitia)! The sources of mediastinal hematoma are frequently the azygos, hemiazygos, internal thoracic, paraspinal and intercostal vessels!
♢Aortic injury is the cause of mediastinal hematoma in only 12.5%!
  • normal admission CXR in 28% (radiographic signs may not develop until 6–36 hours): supine CXR is very inaccurate for mediastinal widening
  • Most specific signs:
    • deviation of nasogastric/endotracheal tube to the right of T3-T4 spinous process (12–100% sensitive, 80–95% specific)
    • depression of left mainstem bronchus anteroinferiorly >40° below the horizontal + toward right (53%)
  • mediastinal widening >8 cm at level of origin of left subclavian artery (present in 75–92%; 53–93–100% sensitive, 1–34–60% specific):
    • mediastinal width to chest width >0.25
  • indistinct aortic contour at arch/descending aorta (53–100% sensitive, 21–55% specific)
  • obscuration of aortopulmonary window (40–100% sensitive, 56–83% specific)
  • widened left paraspinal “stripe” >5 mm (12–83% sensitive, 89–97% specific)
  • thickening of right paratracheal stripe >4–5 mm
    • (= hematoma between pleura + trachea)
  • left/right “apical pleural cap” sign in 37%
    • (= extrapleural hematoma along brachiocephalic vessels)
  • tracheal compression + displacement toward right (61%)
  • rapidly accumulating commonly left-sided hemothorax without evident rib fracture (break in mediastinal pleura)
  • fractures of 1st + 2nd rib (17%)
mnemonic: BAD MEAT
  • Bronchus depression (left main)
  • Aortic silhouette shaggy
  • Death in 80–90%
  • Mediastinal widening
  • Enteric (nasogastric) tube displacement
  • Apical cap
  • Tracheal shift
NECT screening (90–100% sensitive, 19–45% specific, 0–50% PPV, 94–100% NPV):
  • obliteration of aorta-fat interface with increased attenuation (= mediastinal/periaortic hematoma)
  • A negative CT examination for mediastinal hemorrhage has an almost 100% NPV for aortic injury!
  • All patients with periaortic/middle/superior mediastinal hemorrhage require aortography! Save your contrast for that study!
  • If hematomas are seen only in anterior/posterior mediastinum traumatic aortic injury is very unlikely!
  • False positive:
    • residual thymic tissue, periaortic atelectasis, pericardial recess, patient motion, streak artifacts, volume averaging of pulmonary artery, pleural effusion adjacent to descending aorta, sternal + spinal fracture
CECT (100% sensitive; 92–99% specific; 0–39% false positive; 0.7% false negative):
Technique: 100–150 mL at 2 mL/sec with 20–30 second scanning delay
♢ Helical multislice CT has become a major screening tool!
Advantages:
  • negative findings will obviate invasive angio
  • unsuspected injuries are discovered (pulmonary contusion, pneumothorax, pericardial effusion, rib fracture)
Disadvantages:
  • CT delays the definitive aortography + surgery
  • few data exist on accuracy of CT for branch vessel injury
  • intraluminal low-density filling defect:
    • linear = intimal flap
    • polypoid = clot
  • contour deformity of outer aortic wall = pseudoaneurysm
  • contour deformity of inner aortic wall:
    • intramural hematoma
    • pseudocoarctation = abrupt tapering of the diameter of the descending aorta compared with the ascending aorta
  • extravasation of contrast material (rare)
  • False positive:
    • pulsation artifact (aortic valve leaflets, wall of ascending aorta, cardiac motion), streak artifact from high-density contrast in brachiocephalic vein, volume averaging, prominent periaortic bronchial/mediastinal vessels, atherosclerotic pseudoaneurysm, small ductus diverticulum
  • Transesophageal echocardiography:
    • (in 2–15% technically unsuccessful, 57–63% sensitive, 84–91% specific):
    • intimal flap
    • intraluminal thick stripes
    • pseudoaneurysm
    • aortic occlusion (= pseudocoarctation)
    • fusiform aneurysm
    • aortic wall hematoma
  • Aortography (92% sensitive, 98–100% specific):
Technique: LAO + RAO projection; high-flow pigtail catheter; 50 mL at 35 mL/sec
Morbidity: 1.7% (iatrogenic extension of flap, entry of guidewire into pseudoaneurysm)
Delay: 147 minutes between admission and angio
True positive:
  • in 17–20% of patients with mediastinal hematoma angio demonstrates acute traumatic aortic injury!
P.661

False negative:
  • small transverse intimal tears may be missed!
  • resistance in advancing guide wire
  • intimal irregularity, linear defect, filling defect = intimal flap = posttraumatic dissection (5–10%)
  • intramural injury:
    • thickening of aortic wall
    • posttraumatic coarctation
  • transmural laceration:
    • contained extravasation = traumatic false aneurysm
    • free extravasation = aortic rupture
DDx: ductus diverticulum (in 10% of normals), aortic spindle, infundibula of brachiocephalic arterial branches; volume averaging with left brachio-cephalic vein/left superior intercostal vein/right bronchial arteries (vs. intimal flap); artifact from physiologic streaming/mixing of contrast material; atherosclerotic aortic ulceration; atheromatous plaque; syphilitic aortic aneurysm
Recommendations for work-up:
  • Normal well-defined mediastinal contours on CXR: no further imaging
  • Unstable patient + unequivocally abnormal CXR/strong clinical evidence of aortic injury: angiography/emergency surgery
  • Stable patient
    • with unequivocally abnormal CXR:
      • CT of chest + head + abdomen (while waiting for angiographic examination)
    • with equivocal CXR: screening CT of chest
    • abnormal chest CT:
      • angiography for confirmation/surgery
Rx: (1) Antihypertensive medication
(2) Surgical repair (20–54% mortality, 5–10% morbidity from paraplegia)
Prognosis:
  • 80–90% fatal at scene of accident
  • 10–20% reach hospital (due to formation of periaortic hematoma + false aneurysm contained by adventitia ± surrounding connective tissue)
    • without intervention: 30% dead within 6 hours; 40–50% dead within 24 hours, 90% dead within 4 months; chronic false aneurysm may develop in 2–5% at isthmus/descending aorta
    • with surgical repair: 60–70% survive; surgical mortality rate of 9–44% varies with degree of hemodynamic instability + severity of associated injuries + magnitude of aortic laceration
Cx: postoperative paraplegia (9%) due to aortic cross clamping >30 minutes
Chronic Posttraumatic Aortic Pseudoaneurysm
  • = aneurysm existing for >3 months (amount of wall fibroplasia following rupture usually not sufficient to prevent subsequent rupture until at least 3 months after initial traumatic episode)
Incidence: 2–5% of patients surviving aortic transection >24–48 hours
  • symptom-free period of months to years (in 11% >10 years)
  • delayed clinical symptoms (42% within 5 years, 85% within 20 years): chest pain, back pain, dyspnea, cough, hoarseness, dysphagia, systolic murmur
Location: descending aorta at level of lig. arteriosum filling the aorticopulmonary window (most commonly)
  • well-defined rounded mass in left paramediastinal region
  • ±inferior displacement of left mainstem bronchus
Cx: CHF, partial obstruction of aortic lumen, bacterial endocarditis, aortoesophageal fistula, aortic dissection, obstruction of tracheobronchial tree, systemic emboli
Prognosis: enlargement + eventual rupture;
10-year survival rate: 85% with surgical repair, 66% without surgical repair
Tricuspid Atresia
2nd most common cause of pronounced neonatal cyanosis (after transposition) characterized by
  • absent tricuspid valve
  • ASD
  • small VSD (in most patients)
Frequency: 1.5% of all CHD
Embryology: imbalanced tissue proliferation + resorption results in absence of valvular tissue
  • TRICUSPID ATRESIA WITHOUT TRANSPOSITION (80%)
    • without PS, (b) with PS, (c) with pulmonary atresia
  • TRICUSPID ATRESIA WITH TRANSPOSITION
    • without PS, (b) with PS [most favorable combination], (c) with pulmonary atresia
  • Usually small VSD + PS (75%) restrict pulmonary blood flow
Hemodynamics:
  • absent tricuspid valve forces blood from an enlarged RA through an ASD into LA (R-to-L shunt); pulmonary blood flow limited by pulmonary valvular stenosis
image
  • progressive cyanosis from birth on, increasing with crying = OUTSTANDING FEATURE (inverse relationship between degree of cyanosis + volume of pulmonary blood flow)
  • pansystolic murmur (VSD)
  • ECG: left-axis deviation
CXR (typical cardiac contour):
  • heart size ranging from normal to moderately enlarged (depending on volume of pulmonary blood flow and size of RA)
  • left rounded contour = enlargement + hypertrophy of LV
  • right rounded contour = enlarged RA
  • flat/concave pulmonary segment
  • normal/decreased pulmonary vascularity
  • typical flattening of right heart border with transposition (in 15%)
Prognosis: may survive well into early adulthood
Rx:
  • Blalock-Taussig procedure (if pulmonary blood flow decreased in infancy)
  • Glenn procedure = shunt between IVC + right PA (if total correction not anticipated)
  • P.662

  • Fontan procedure = external conduit from RA to pulmonary trunk + closure of ASD (if pulmonary vascular disease has not developed)
Tricuspid Insufficiency
Cause:
  • Right ventricular failure (most common)
  • Carcinoid syndrome
  • Bacterial endocarditis
  • Rheumatic heart disease
  • Congenital heart disease: Ebstein anomaly, atrioventricular cushion defect
  • normal/reduced pulmonary vascularity
  • cardiomegaly
  • RA + RV enlargement
  • distension of IVC > SVC
Trousseau Syndrome
PARANEOPLASTIC THROMBOEMBOLISM
Incidence: 1–11%; higher in terminally ill cancer patients
Tumors: mucin-secreting adenocarcinoma of GI tract and pancreas (most common), lung, breast, ovary, prostate
Pathogenesis: (?)
(a) tumors activate coagulation + depress anticoagulant function
(b) cancer cells cause injury to endothelial lining, activate platelets + coagulation
Type of lesion: (1) Venous thrombosis
(2) Arterial thromboembolism
(3) Nonbacterial thrombotic endocarditis
  • Patients with thromboembolism have an increased incidence of occult malignancy!
Prevalent criteria:
  • absence of apparent cause for thromboembolism
  • age >50 years
  • multiple sites of venous thrombosis
  • simultaneous venous + arterial thromboembolism
  • resistance to oral anticoagulant therapy
  • associated other paraneoplastic syndromes
  • regression of thromboembolism with successful treatment of cancer
  • • disorders of consciousness (cerebral emboli)
  • • muscular pain + weakness (emboli to skeletal muscle)
  • • decompensated disseminated intravascular coagulation
  • deep vein thrombosis
  • pulmonary embolism
  • nonbacterial thrombotic endocarditis (echocardiography)
Rx: (1) Heparin (more successful than warfarin)
(2) Greenfield filter
Truncus Arteriosus
  • = PERSISTENT TRUNCUS ARTERIOSUS = SINGLE OUTLET OF THE HEART
  • = failure of septation of the conotruncus characterized by
    • one great artery arising from the heart giving rise to the coronary, pulmonary, and systemic arteries, and straddling a
    • large VSD
Incidence: 2% of all CHD
Types:
Type I (50%) = main PA + aorta arise from common truncal valve
Type II (25%) = both pulmonary arteries arise from back of trunk
Type III (10%) = both pulmonary arteries arise from side of trunk
Type IV = “pseudotruncus” = absence of pulmonary arteries; pulmonary supply from systemic collaterals arising from descending aorta
Subtype A = infundibular VSD present
Subtype B = VSD absent
Associated with:
  • Right aortic arch (in 35%)
    • right aortic arch + cyanosis + shunt vascularity = TRUNCUS
  • Forked ribs
Hemodynamics:
  • admixture lesion (R-to-L and L-to-R shunt across VSD) with volume of pulmonary blood flow inversely related to degree of pulmonary vascular resistance
fetus: CHF only with incompetent valve secondary to massive regurgitation from truncus to ventricles
neonate: L-to-R shunt after decrease in pulmonary resistance (massive diversion of flow to pulmonary district) leads to CHF (ventricular overload)/pulmonary hypertension with time
image
image
Variation In Truncus Arteriosus
P.663

  • moderate cyanosis (degree inversely related to volume of pulmonary blood flow), apparent with crying
  • severe CHF within first days/months of life (in large R-to-L shunt)
  • systolic murmur (similar to VSD)
  • early diastolic murmur (with truncal insufficiency)
  • wide pulse pressure
CXR:
  • cardiomegaly:
    • increased volume of both ventricles
    • enlarged LA (50%) secondary to increased pulmonary blood flow
  • wide mediastinum due to large “aortic shadow” = truncus arteriosus
  • “waterfall/hilar comma sign” = elevated right hilum (30%); elevated left hilum (10%)
  • concave pulmonary segment (50%) (type I has left convex pulmonary segment)
  • markedly increased pulmonary blood flow, may be asymmetric
ECHO:
  • single arterial vessel overriding the interventricular septum (DDx: tetralogy of Fallot)
  • frequently dysplastic single semilunar valve with 3–6 leaflets (most commonly 3 leaflets)
    • truncal valve may be stenotic
    • truncal valve insufficiency with age (in 25%)
Prognosis: 40% 6-months survival rate,
20% 1-year survival rate
Rx: Rastelli procedure (30% no longer operable at 4 years of age) = (a) artificial valve placed high in RVOT and attached via a Dacron graft to main pulmonary artery (b) closure of VSD
Hemitruncus
  • = rare anomaly characterized by
    • one pulmonary artery (commonly right PA) arising from trunk
    • one pulmonary artery arising from RV/supplied by systemic collaterals
Associated with: PDA (80%), VSD, tetralogy (usually isolated to left PA)
  • acyanotic
Pseudotruncus Arteriosus
  • = TRUNCUS TYPE IV
  • = severe form of tetralogy of Fallot with atresia of the pulmonary trunk; entire pulmonary circulation through bronchial collateral arteries (NOT a form of truncus arteriosus in its true sense); characterized by
    • pulmonary atresia
    • VSD with R-to-L shunt
    • RV hypertrophy
Associated with: right aortic arch in 50%
  • • cyanosis
  • concavity in area of pulmonary segment
  • commalike abnormal appearance of pulmonary artery
  • absent normal right and left pulmonary artery (lateral chest film)
  • esophageal indentation posteriorly (due to large systemic collaterals)
  • prominent hilar + intrapulmonary vessels (= systemic collaterals)
  • “coeur en sabot” = RV enlargement
  • prominent ascending aorta with hyperpulsations
Ventricular Aneurysm
  • CONGENITAL LEFT VENTRICULAR ANEURYSM
    • rare, young Black adult
    • Submitral type:
      • bulge at left middle/upper cardiac border
    • Subaortic type:
      • small + not visualized
      • heart greatly enlarged (from aortic insufficiency)
  • ACQUIRED LEFT VENTRICULAR ANEURYSM
    • = complication of myocardial infarction, Chagas disease
    • • may be asymptomatic + well tolerated for years
    • • occasionally associated with persistent heart failure, arrhythmia, peripheral embolization
True Ventricular Aneurysm
  • = circumscribed noncontractile outpouching of ventricular cavity with broad mouth + localized dyskinesis
Cause: sequelae of transmural myocardial infarction
Location:
  • left anterior + anteroapical: readily detected (anterior + LAO views)
  • inferior + inferoposterior: less readily detected (steep LAO + LPO views)
Detection rate: 50% by fluoroscopy; 96% by radionuclide ventriculography; frequently not visible on CXR
  • localized bulge of heart contour = “squared-off” appearance of mid left lateral margin of heart border
  • localized paradoxical expansion during systole (CHARACTERISTIC)
  • rim of calcium in fibrotic wall (chronic), rare
  • akinetic/severely hypokinetic segment
  • left ventriculography in LAO, RAO is diagnostic
  • wide communication with heart chamber (no neck)
Cx: wall thrombus with embolization
Prognosis: rarely ruptures
Pseudoaneurysm of Ventricle
  • = FALSE ANEURYSM
  • = left ventricular rupture contained by fused layers of visceral + parietal pericardium/extracardiac tissue
    • cardiac rupture with localized hematoma contained by adherent pericardium; typically in the presence of pericarditis
    • subacute rupture with gradual/episodic bleeding
Etiology: trauma, myocardial infarction
Location: typically at posterolateral/diaphragmatic wall of LV
  • left retrocardiac double density
  • diameter of mouth smaller than the largest diameter of the globular aneurysm
  • delayed filling
P.664

Cx: high risk of delayed rupture (infrequent in true aneurysms)
Ventricular Septal Defect
  • Most common CHD (25–30%):
    • isolated in 20%
    • with other cardiac anomalies in 5% (PDA, CoA)
  • Acyanotic L-to-R shunt + right aortic arch (in 2–5%) = VSD
Embryology:
  • single ventricular chamber divides into two by fusion of membranous portion of ventricular septum + endocardial cushions + bulbis cordis (= proximal part of truncus arteriosus) between 4–8th week
  • MEMBRANOUS = PERIMEMBRANOUS VSD (75–80%)
    Location: posterior + inferior to crista supraventricularis near commissure between right and posterior (= noncoronary) aortic valve cusps
    • May be associated with:
      • small aneurysms of membranous septum commonly leading to decrease in size of membranous VSD (their presence does not necessarily predict eventual complete closure)
  • SUPRACRISTAL = CONAL VSD (5–8%)
    • Crista supraventricularis = inverted U-shaped muscular ridge posterior + inferior to pulmonary valve
    • RV view = VSD just beneath pulmonary valve with valve forming part of superior margin of defect
    • LV view = VSD just below commissure between R + L aortic valve cusps
    Cx: right aortic valve cusp may herniate into VSD (= aortic insufficiency)
  • MUSCULAR VSD (5–10%)
    • May consist of multiple VSDs; bordered entirely by myocardium
    Location: (a) inlet portion
    (b) trabecular portion
    (c) infundibular/outlet portion
  • ATRIOVENTRICULAR CANAL TYPE
    • = ENDOCARDIAL CUSHION TYPE = POSTERIOR VSD (5–10%)
    Location: adjacent to septal + anterior leaflet of mitral valve; rare as isolated defect
  • Hemodynamics:
    • small bidirectional shunt during fetal life (similar pressures in RV + LV); after birth pulmonary arterial pressure decreases + systemic arterial pressure increases with development of L-to-R shunt
  • Classification:
    • “Maladie de Roger”
      • = small restrictive VSD with defect <1 cm; little/no hemodynamic significance with normal pulmonary artery pressure, normal pulmonary vascular resistance
      • • asymptomatic
      • • holosystolic heart murmur at 4th left rib interspace
      • normal plain film
      Prognosis: spontaneous closure
    • Moderate Shunt
      • VSD defect <75% of aortic diameter (1–1.5 cm); systolic LV pressure > systolic RV pressure; intermediate pulmonary artery pressure; normal pulmonary vascular resistance
        image
      • • respiratory infections, mild dyspnea
      • slight prominence of pulmonary vessels (45% shunt)
      • slight enlargement of LA
      Prognosis: spontaneous closure in large percentage
    • Nonrestrictive Large Shunt
      • VSD defect >75% of aortic diameter; systolic LV pressure = systolic RV pressure (pulmonary vascular disease + hypertension increases RV pressure); pulmonary artery pressure approaching systemic levels; slightly increased pulmonary vascular resistance; pulmonary blood flow 2–4 × systemic flow;
        image
      • • bouts of respiratory infections
      • • feeding problems, failure to thrive
      • • CHF soon after birth (due to RV overload)
      • prominent pulmonary segment + vessels (= shunt vascularity)
      • calcification of pulmonary arteries
        • = PATHOGNOMONIC for pulmonary arterial hypertension
      • enlargement of LA + LV
      • normal/small thoracic aorta
    • Eisenmenger syndrome
      • large VSD eventually leads to shunt reversal (R-to-L shunt) due to irreversible increase in pulmonary vascular resistance (= intima + medial hyperplasia) when pulmonary vascular resistance >0.75 of systemic vascular resistance
        image
        Frequency: 10% of large VSDs by 2 years of age
      • • cyanotic, but less symptomatic; CHF rare
      • eventual decrease of pulmonary vessel caliber
      • eventual decrease in size of LA + LV
NATURAL HISTORY OF VSD causing reduction in pulmonary blood flow:
  • Spontaneous closure
    • in 40% within first 2 years of life; 60% by 5 years (65% with muscular VSD, 25% with membranous VSD); with large VSD in 10%; with small VSD in 50%
  • RVOT obstruction
    • infundibular hypertrophy in 3% = pink tetrad
  • Prolapse of right aortic valve cusp
    • = aortic valve insufficiency
CXR (with increase in size of VSD):
  • variable appearance due to variations in defect size
  • enlargement of LA
  • enlargement of pulmonary artery segment
  • enlargement of LV
  • RV hypertrophy
  • increase in pulmonary blood flow (if >45% of pulmonary blood flow from systemic circulation)
  • Eisenmenger reaction
ECHO:
  • prolapse of aortic valve cusp (in supracristal VSD)
  • deformity of aortic cusp (in membranous VSD)
  • P.665

  • lack of echoes in region of interventricular septum with sharp edges (DDx: artifactual dropout with sound beam parallel to septum); muscular VSD difficult to see
  • LA enlargement
Angio:
  • Projections:
    • LAO 60° C-C 20° for membranous + anterior muscular VSD
    • LAO 45° C-C 45° (hepatoclavicular) for posterior endocardial cushion + posterior muscular VSD
    • RAO for supracristal VSD + assessment of RVOT
    • RVOT/pulmonary valve fill without filling of RV chamber (in supracristal VSD)
  • Rx:
    • large VSD + left heart failure at 3 months of age: aim is to delay closure until child is 18 months of age; pulmonary-to-systemic blood flow >2:1 requires surgery before pulmonary hypertension becomes manifest
      • Digitalis + diuretics
      • Pulmonary artery banding
      • Patching of VSD: surgical approach through RA/through RV for supracristal VSD
    • small VSDs without increase in pulmonary arterial pressure are followed