Cardiovascular Disorders
Aberrant Left Pulmonary Artery
| 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:
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
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
Total Anomalous Pulmonary Venous Return
| 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 |
Hemodynamics:
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 |
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
| 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%)
| Age: |
presentation in neonatal period |
| 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%)
Partial Anomalous Pulmonary Venous Return
| 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:
• 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
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:
Abnormal volume load: severe aortic regurgitation
True Aneurysm
False Aneurysm
Fusiform Aneurysm (80%)
Saccular aneurysm
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:
| 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:
• 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):
Cx:
| 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 |
Postoperative Cx:
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:
• 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
| 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 |
| 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
| 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 |
| 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 |
| 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
| 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
| 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 |
| 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%)
| 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
| 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
 |
| 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 |
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
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):
| 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) |
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:
| 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%)
| Cause: |
hemorrhage of vasa vasorum |
| Path: |
clotted intramural blood within aortic media; NO/(?) slow flow within the false channel |
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)
Aortic motion artifact simulating type A IMH
Aortic Prosthetic Graft Infection
| Incidence: |
1.3–6% of prosthetic graft procedures |
Classification:
• 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
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:
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
Cause:
| 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/sec
2 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 Stenosis
| 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)
ECHO:
| 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
| |
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
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
| Degree: |
mild: >0.7 cm2; moderate: 0.5–0.7 cm2; severe: <0.5 cm2
|
Congenital types:
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
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
| 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:
Effect of hyperlipidemia:
| 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) |
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%)
| 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%)
| Location: |
lower end of septum inferior to fossa ovalis (at outlet portion of atrial septum) adjacent to atrioventricular valves |
Almost always associated with:
-
SINUS VENOSUS ASD (10%)
| 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
-
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%)
 |
• 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
-
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 |
 |
| Atrial Septal Defects |
 |
| Normal Newborn Heart |
 |
| Ostium Secundum Defect |
 |
| Sinus Venosus Defect |
 |
| Ostium Primum Defect |
Azygos Continuation of IVC
| 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) |
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:
-
Bicuspid aortic valve:
-
Prosthetic valve:
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
| 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%) |
| 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
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
| 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 |
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:
| 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
| 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
| 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:
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
| Incidence: |
<50 cases |
| Age: |
18-85 (mean, 40) years |
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 |
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 |
| 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%) |
| Prognosis: |
12–30 months survival |
Undifferentiated Sarcoma
| Age: |
45 years (neonates to elderly) |
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
• 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
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
| DDx: |
myxoma (heterogeneous texture on CT) |
Cardiomyopathy
Dilated and Ischemic 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
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%)
-
SYMMETRIC/CONCENTRIC HYPERTROPHY (2–20%)
-
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%)
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)
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
| 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
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:
| 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 |
 |
| Coarctation of the Aorta |
Coarctation Of Aorta
-
LOCALIZED COARCTATION
| Location: |
most frequent in juxtaductal portion of arch |
-
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: |
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
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:
Postsurgical Cx:
Residual coarctation (in 32%)
Subsequent obstruction (rare)
-
Mesenteric arteritis: 2-3 days after surgery secondary to paradoxical hypertension from increased plasma renin
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 |
Asymptomatic CoA
| 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
| 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
Extrathoracic Manifestations of CHF
-
@ Hepatobiliary
GB wall edema
periportal edema
enlarged IVC
Constrictive Pericarditis
| Age: |
30–50 years; M:F = 3:1 |
Etiology:
IDIOPATHIC (2nd most common today)
-
INFECTIOUS/INFLAMMATORY
-
TRAUMATIC
UREMIA = chronic renal failure
NEOPLASTIC = tumor invasion
Causes of acute pericarditis:
• 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
Abnormal communication with (>90 % right heart):
| 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
| 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):
| Rx: |
surgical excision of obstructing membrane |
Deep Vein Thrombosis
| 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:
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) |
Venography (89% sensitivity, 97% specificity):
| 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
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
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
| 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
Classification:
according to age: infantile, childhood, adult type
according to cause: congenital, infectious, traumatic
| Pathogenesis: |
? delay in closure,? myxoid degeneration of ductus wall,? abnormal elastic fibers |
| Age: |
most <2 months of age |
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
Valve morphology:
| Etiology: |
chronic maternal lithium intake (10%) |
Hemodynamics:
± 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
(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 |
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
| 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:
-
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 |
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:
| 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 |
Endocardial Fibroelastosis
Etiology:
| Prognosis: |
mortality almost 100% by 2 years of age |
Fibromuscular Dysplasia
| 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:
| 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%)
| 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
-
3. perimedial FIBROPLASIA (rare)
| 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%)
| 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%)
| Path: |
adventitial + periarterial proliferation in fibrofatty tissue |
| Site: |
main renal artery, large branches |
-
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
| DDx: |
atherosclerotic disease, posttraumatic aneurysm |
| VARIANT: |
segmental mediolytic arteriopathy |
| 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 |
| 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]
Embryology:
| Inheritance: |
multifactorial (autosomal dominant, autosomal recessive, X-linked recessive) |
Individualized approach of classification:
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
| Incidence: |
1:1,750–1:40,000 livebirths; M > |
Associated with:
• 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
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
| Age: |
presentation in infancy/adulthood; M < F |
Associated with:
• 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
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
| 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 |
-
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):
• 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):
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
| 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
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:
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
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 |
 |
| 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:
| 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
-
Septic arthritis:
spine, sacroiliac, sternoclavicular, symphysis pubis, acromioclavicular, hip, knee, wrist
-
Pleuropulmonary complications
-
Gastrointestinal complications
-
Genitourinary complications
-
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
| 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)
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 |
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:
Lymphoma of heart
Secondary Cardiac Lymphoma
| Incidence: |
in 16–28% on autopsy; pericardial involvement more frequent; more common in immunocompromised patients |
Primary Cardiac Lymphoma
| 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
| 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
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:
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
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 |
Hemodynamics:
| 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 cm
2; mild narrowing with <2 cm
2; severe narrowing with <1 cm
2 (reproducible to within 0.3 cm
2)
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) |
Mitral Valve Prolapse
| Incidence: |
2-6%of general population; 5-20%of young women;? autosomal dominant inheritance |
| Age: |
commonly 14-30 years |
Cause:
•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 cm
2
| 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) |
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%)
| 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%)
| 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) |
| 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
•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%)
| Etiology: |
autoimmune reaction to myocardial infarction |
| Onset: |
2–3 weeks (range 1 week–several months) following infarction |
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:
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
ECHO: (2D-ECHO is study of choice)
CT:
well-defined spherical/ovoid intraluminal filling defect
lobular/smooth surface contour
tumor attenuation lower than unopacified blood (due to gelatinous component)
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
| 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
| 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:
Hemodynamics of 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)
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
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
Penetrating Aortic Ulcer
| 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):
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
| Frequency: |
1:13,000; M:F = 3:1 |
| Age at detection: |
newborn to 81 years (mean 21 years) |
Location:
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)
• 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
| 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 |
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
| 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:
| Age: |
<35 years in 68%; age peaks at 17 and 47 years; M:f = 9:1 |
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
Diagnosis per exclusion:
| 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
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
Cause:
-
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) |
CXR:
stable/increasing focal lung mass
CT:
enhancing round lung mass isointense to central pulmonary artery
| Cx: |
100% mortality with rupture |
Pulmonary Atresia
| 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)
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
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 |
| Prognosis: |
death at mean age of 21 years if untreated |
Subvalvular Pulmonic Stenosis
| 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
Supravalvular Pulmonic Stenosis
| Site of narrowing: |
pulmonary trunk, pulmonary bifurcation, one/both main pulmonary arteries, lobar pulmonary artery, segmental pulmonary artery |
Shape of narrowing:
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
Raynaud Disease
| Cause: |
? acquired adrenoreceptor hypersensitivity |
| May be associated with: |
reflex sympathetic dystrophy, early stages of autoimmune disorders |
| Age: |
most common in young women |
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)
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:
Rhabdomyoma Of Heart
= benign myocardial hamartoma
| 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
| Associated with: |
TGV or DORV |
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 |
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
fingertip necrosis
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
Occult Subclavian Steal Syndrome
Superior Vena Cava Syndrome
= obstruction of SVC with development of collateral pathways
-
Etiology:
-
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
= 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
• 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:
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
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 |
| 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
Overriding aorta straddling the VSD and receiving blood from both ventricles
Embryology:
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 |
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
| Operative mortality: |
3–10% |
Trilogy of Fallot (infantile presentation)
Thoracic Outlet Syndrome
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
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%)
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:
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
Embryology:
| 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:
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 |
 |
| 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
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
| 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)
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
| 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:
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
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
-
left/right “apical pleural cap” sign in 37%
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%)
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:
| 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:
False negative:
| 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:
| Rx: |
(1) Antihypertensive medication
(2) Surgical repair (20–54% mortality, 5–10% morbidity from paraplegia) |
Prognosis:
| Cx: |
postoperative paraplegia (9%) due to aortic cross clamping >30 minutes |
Chronic Posttraumatic Aortic Pseudoaneurysm
| 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
| Frequency: |
1.5% of all CHD |
| Embryology: |
imbalanced tissue proliferation + resorption results in absence of valvular tissue |
Usually small VSD + PS (75%) restrict pulmonary blood flow
Hemodynamics:
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)
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
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:
Hemodynamics:
| 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 |
 |
| Variation In Truncus Arteriosus |
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
| Associated with: |
PDA (80%), VSD, tetralogy (usually isolated to left PA) |
Pseudotruncus Arteriosus
| 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
True Ventricular Aneurysm
| Cause: |
sequelae of transmural myocardial infarction |
Location:
| 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
| 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
| Cx: |
high risk of delayed rupture (infrequent in true aneurysms) |
Ventricular Septal Defect
Embryology:
-
MEMBRANOUS = PERIMEMBRANOUS VSD (75–80%)
| Location: |
posterior + inferior to crista supraventricularis near commissure between right and posterior (= noncoronary) aortic valve cusps |
-
SUPRACRISTAL = CONAL VSD (5–8%)
Crista supraventricularis = inverted U-shaped muscular ridge posterior + inferior to pulmonary valve
| Cx: |
right aortic valve cusp may herniate into VSD (= aortic insufficiency) |
-
MUSCULAR VSD (5–10%)
| Location: |
(a) inlet portion
(b) trabecular portion
(c) infundibular/outlet portion |
-
ATRIOVENTRICULAR CANAL TYPE
| Location: |
adjacent to septal + anterior leaflet of mitral valve; rare as isolated defect |
-
Hemodynamics:
-
Classification:
NATURAL HISTORY OF VSD causing reduction in pulmonary blood flow:
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)
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
small VSDs without increase in pulmonary arterial pressure are followed
