Cardiac

Front 1

Cyanosis with Decreased Vascularity

Back 1

-4 T's and E
-Tetralogy
-Truncus (type 4)
-Tricuspid atresia
-Transposition (but can also have increased vascularity)
-Ebstein

Front 2

Cyanosis with INCREASED vascularity

Back 2

--4 T's and S
--Truncus (1-3)
--TAPVR (total anomalous venous return)
--Tricuspid atresia (also decr)
--Transposition (also decr)
--Single Ventricle
--

Front 3

Peds--
Cardiomegaly with NORMAL vasculature

Back 3

-Endocardial fibroelastosis
-aberrant left coronary artery
-cystic medial necrosis
-viral myocarditis
-diabetic mother

Front 4

Pulmonic Stenosis
CXR

Back 4

--Enlarged main pulmonary artery
• Enlarged left pulmonary artery (jet stream effect)
• Normal to decreased peripheral pulmonary vasculature

Front 5

Lipomatous hypertrophy of the interatrial septum

Back 5

on-enhancing, dumbbell-shaped mass of fat density
Confined to interatrial septum
Spares fossa ovalis producing dumbbell shape
Cephalad portion thicker than caudal portion of mass
--Associated with older age and obesity
--usually asympt, but sometimes assoc with atrial arrythmias
--not a lipoma. Lipomas in heart very rare

Front 6

Pulmonary Sling

Back 6

Pulmonary sling occurs because of failure of formation of Left 6th aortic arch so there is absence of Left pulmonary artery
The blood to the Left lung comes from an aberrant Left pulmonary artery which arises from Right pulmonary artery and crosses between esophagus and trachea
Bronchial cyst may produce same finding on esophagus/trachea

Front 7

Double Aortic Arch

Back 7

--Double aortic arch is most common vascular ring (55%)
--Caused by persistence of Right and Left IV branchial arches
--Rarely associated with Congenital Heart Disease
--Symptoms (of tracheal compression or difficulty swallowing) may begin at birth
Right arch is higher, left arch is lower producing reverse S on esophagram in AP
Right arch supplies Right common carotid and Right subclavian arteries
Left arch supplies Left common carotid and Left subclavian arteries
On lateral, arches are posterior to esophagus and anterior to trachea
Congenital aortic arch anomaly related to persistence of both the left and right fourth aortic arches

**Each arch gives rise to a ventral carotid and a dorsal subclavian artery (symmetric "four artery sign")
--Right arch commonly larger and more superior and posterior extending than left
--Trachea is deviated from dominant arch, or may be in abnormal midline position (normally trachea is slightly deviated to right by left arch)
--TRUE complete vascular ring with trachea and esophagus encircled
--Dominant right arch, left descending aorta: 75%; Dominant left arch, right descending aorta: 20%
--Inspiratory stridor, worsening with feeding
--Determination of which arch is smaller on cross-sectional imaging will determine on which side thoracotomy is performed
**Look for signs of atretic segments of the double arch anomaly (will not be opacified on CTA or MRA, and show no flow voids on spin echo MRI)
**On cross-sectional imaging, both left and right arches are identified arising from ascending aorta and joining to form descending aorta
--Right arch typically runs behind esophagus to join left arch, to form left-sided descending aorta
--Part of left arch may be atretic but patent portions remain connected by fibrous band, completing the compressive ring around trachea and esophagus
--CXR: Prominent soft tissue on either side of the trachea, Bilateral tracheal indentations, mid-tracheal stenosis, Trachea is deviated from dominant arch, or may be in abnormal midline position (normally trachea is slightly deviated to right by left arch), Right arch indentation commonly somewhat higher and more prominent than left
On lateral view, anterior and posterior compression of trachea at level of arch, Symmetric aeration, no unilateral air trapping (as Pulmonary sling only vascular ring to be associated with asymmetric aeration)
--ESOPHAGRAM: Anteroposterior (AP) view: Bilateral indentations on contrast-filled upper esophagus, often at different levels; Lateral view: Prominent oblique or nearly horizontal posterior indentation
**CTA:Four artery sign: Symmetric take-off of four aortic branches on axial image at thoracic inlet: 2 ventral carotids and 2 dorsal subclavians,
Two arches are seen, completely encircling trachea and esophagus, leading to severe mid-tracheal compression, Smaller of two arches may be partially atretic
Severe tracheal compression at level of double arch, Coronal 3D images are very helpful to demonstrate arch anatomy in relation to trachea
**Radiography remains primary diagnostic test-->If radiography demonstrates lack of tracheal compression, vascular ring excluded
--Barium swallow rarely obviates need for cross-sectional imaging and therefore many feel is not useful in workup of suspected arch anomaly
However many asymptomatic arch anomalies are first diagnosed by barium swallow
Cross-sectional imaging (CT or MR) performed to confirm diagnosis and define anatomic variations for pre-surgical planning

Front 8

LA Stenosis
CXR

Back 8

--Small aortic knob from decreased cardiac output
-- Double density of left atrial enlargement (four bumps on left--from aorta, pulm a, LA, and LV)

Front 9

Vascular rings

Back 9

The 2 most common types of complete vascular rings are double aortic arch and right aortic arch with left ligamentum arteriosum. These make up 85-95% of the cases

Front 10

Tetralogy of Fallot

Back 10

Surgery, a

Front 11

Transposition

Back 11

a

Front 12

Epstein

Back 12

a

Front 13

Coronary a aneurysm

Back 13

=Coronary artery diameter > 1.5 times normal adjacent segments
**Best imaging tool: Gated coronary CTA
--Invasive angiography may underestimate luminal diameter if mural thrombus present
--Atherosclerosis most common in USA
--Kawasaki disease most common worldwide
--Other: Congenital, trauma, procedural (angioplasty, stent, laser, atherectomy), mycotic emboli, dissection, connective tissue disease (SLE, Marfan, Behcet)
--Can result in thrombosis and myocardial infarction
--Morphology: Fusiform or saccular dilatation, Can be thrombosed or dissected
--Calcification frequently present in atherosclerosis
--CTA: Detects mural thrombus that may be occult on invasive angiography
--MR Findings:Can use coronary angiography sequence, Lumen dark on double IR FSE, Bright on GRE or b-SSFP unless thrombus, Difficult to detect calcification
--TXs: Anticoagulants, antiplatelet therapy, Bypass and exclusion of aneurysm, Covered stent graft

Front 14

Myocardial Bridge

Back 14

--Congenital coronary anatomic variant in which a segment of epicardial coronary artery takes an intramyocardial course
--Location: Most common = mid segment of left anterior descending coronary artery, left circumflex artery (~ 40%), right coronary artery (~ 20%)
--Most patients have single bridge
--Cause of ischemia currently uncertain
**Clinical Issues--Most patients asymptomatic; small subgroup develop ischemia
--On cardiac CT, use multiplanar reformats to depict bridge
--DDx=Hypertrophic Cardiomyopathy (Asymmetric myocardial hypertrophy may mimic a bridge), Cardiac Tumor (May invade epicardial fat to engulf coronary arteries), Coronary Anomaly (Classically a left main arising from the RCA and passing between the great vessels may bridge)
--Prevalence of 1.5-16% in angiography studies (an underestimate)
Autopsy studies = up to 80%

Front 15

Aortic intramural hematoma

Back 15

=Rupture of vasa vasorum resulting in hematoma within aortic media
--CT is imaging modality of choice
--Crescentic hyperdense aortic wall on NECT
--Hypodense wall with elliptical aortic lumen on CECT
--Smooth well-defined margin, no intimal flap

MRA:
High signal aortic wall thickening on pre-contrast T1WI
IMH appears as low signal on post-contrast T1WI
TE MRA shows only aortic lumen filling
CE MRA shows lack of contrast in IMH

DDX=Saccular Aortic Aneurysm with Mural Thrombus, Dissection, Traumatic Aortic Pseudoaneurysm, Aortitis, Penetrating Atherosclerotic Ulcer
--Types A (ascending is involved) and B (only descending is involved) aortic IMH
--50-80% involve the descending aorta
--Hemorrhage confined to media, with no intimal tear
--Typically hypertensive
--Acute chest pain: Severe, tearing, or migratory
--Treated similar to typical aortic dissection
--Medical or surgical (open, EVAR) management
--May have complete resorption of hematoma (< 30%)
--CTA within 48 hrs of initial scan to check for progression to dissection

Front 16

Takayasu

Back 16

=Granulomatous inflammatory vasculitis affects walls of medium and large vessels, especially aorta and branches
**Best diagnostic clue: Smooth narrowing of aorta and major vessels
--Occasional pulmonary artery involvement
--Distribution usually patchy, symmetric great vessel distribution common
--Size: Essentially only vasculitis to involve aorta and major vessels
--Most common in Asian countries
--Thickening of the large and medium size vessels
--Early phase inflammatory or prepulseless phase, Late phase occlusive or pulseless
--Age < 30 in 90% of patients
--Gender: M:F = 1:8, but may be less female predominant in non-Asian countries
--Morbidity and mortality are due to hypertension and stroke
--Corticosteroids are mainstay of therapy
--Diagnosis may be problematic and delayed due to smoldering nature of disease
--Left subclavian most common

Front 17

Scimitar Syndrome

Back 17

=Congenital hypoplasia of the right lung and anomalous pulmonary venous drainage to the inferior vena cava (IVC)
--Vertical vein: Gently curved vein in the right mid-lung towards the right costovertebral angle, vein enlarges as it descends towards the diaphragm
**Anomalous vein shaped like a Turkish SWORD (scimitar sign)
--Anomalous vein usually drains into subdiaphragmatic inferior vena cava
--Oxygenated blood drains into right atrium producing a left-to-right SHUNT
--Associated cardiac anomalies (25%)
--50% asymptomatic
--Recurrent infections common
--Without cardiac anomalies, usually normal life span

**CXR**
--Anomalous vein (75%)
Gently curved tubular shadow descending from the right mid-lung towards the R CVA
--Vein gets larger as it descends towards the diaphragm
Shaped like a Turkish sword (scimitar syndrome)
**Small right hemithorax with mediastinal shift to R, due to R lung hypoplasia
Hypoplasia may be mild or marked
--Cardiac shift may be mistaken for dextrocardia (actually DEXTROPOSITION)
Left lung is hyperinflated with large left pulmonary artery (depends on degree of hypoplasia right pulmonary artery)
**Increased vascularity from left-to-right shunt, seen by enlarged L pulm a.
--Small right hilum

Associated anomalies
Cardiomegaly: Secondary to pulmonary artery hypertension, left-to-right shunt, or cardiac anomalies
Vertebral anomalies
Bronchogenic cyst sometimes associated
Extremely rare accessory diaphragm
Diaphragmatic hernia

CECT
Bronchial anomalies, associated
Absent minor fissure, mirror image branching, bilobed right lung
Horseshoe lung: Bridge of lung fusing the two lungs across the posterior mediastinum
HORSESHOE lung: Ominous finding often associated with lethal cardiac anomalies
Bronchial diverticula
Cardiovascular anomalies, associated
Dilated right ventricle in left-to-right shunts and pulmonary hypertension
Enlarged main pulmonary artery
Anomalous vein
Precisely define drainage into systemic vein
Occasional systemic arterialization of the lung from the descending thoracic aorta or upper abdominal aorta
HRCT
Hypoplastic lung: Mosaic perfusion with hypoattenuation of the hypoplastic lung
Secondary bronchiectasis from recurrent infections
Infections may be secondary to seeding of lung from bronchial diverticula

**DDx=
Congenital Interruption Pulmonary Artery
Pulmonary Sequestration
Wandering Vein
Fibrosing Mediastinitis
Chronic Thromboembolic Occlusion
Swyer-James Syndrome
Pathology

Front 18

Pulmonary varix

Back 18

--Acquired or development dilatation pulmonary vein at its entrance to left atrium
--Lobulated mass or nodule posterior to the heart

Front 19

approach to congenital heart disease

Back 19

This case demonstrates the steps of the segmental approach to congenital heart disease:

Define the visceroatrial situs (solitus, inversus, ambiguous)
Discern the orientation of the ventricular loop (D, L)
Delineate the relative positions of the great vessels (S, I, D-TGA, L-TGA)
Determine the atrioventricular (AV) and ventriculoarterial (VA) relationships (concordance, discordance)
Search for additional anomalies of the cardiac chambers, septae, outflow tracts, and great vessels

It is important to note at the outset of this discussion that the morphologic “sidedness” of an atrium or ventricle refers to its position after normal embryologic development, and, is thus, independent from its actual position in the chest in the setting of congenital heart disease.

Visceroatrial situs can be inferred on chest radiography from the major bronchial anatomy. The side of the epiarterial bronchus (and trilobed lung) will be on the same side as the morphologic right atrium. Correspondingly, the side of the hypoarterial bronchus (and bilobed lung) will be on the same side as the morphologic left atrium. On cross sectional imaging, the most reliable indicator of the morphologic right atrium is that it receives the inferior vena cava, although the morphologic left atrium does not. Atrial appendage morphology can be used on gated, cross sectional studies. Our patient had normal visceroatrial situs.

Orientation of the ventricular loop requires differentiating the features of the morphologic right ventricle (trabeculated septal wall, moderator band, infundibulum, and apical displacement of the septal leaflet of its AV valve) from that of the left (smooth septal wall and lack of infundibulum resulting in fibrous continuity of its AV and semilunar valves). The morphologic left ventricle on the left side is consistent with a D-ventricular loop; however, in our case, the morphologic left ventricle was on the right side, consistent with an L-ventricular loop.

The relative position of the aorta in relation to the main pulmonary artery is posterior and to the right in solitus, and posterior and to the left in inversus. Anterior and to the right is D-TGA (complete transposition of the great arteries), although anterior and to the left, as in our case, is L-TGA (congenitally corrected transposition of the great arteries).

AV concordance is determined by whether the morphologic atrium connects its corresponding morphologic ventricle, and VA concordance is determined by whether that morphologic ventricle, in turn, connects to its corresponding great vessel. A normal connection is concordant, whereas an abnormal connection is discordant. Our patient had both AV and VA discordance.

In segmental anatomy terms, our patient had {S, L, L-TGA} ({visceroatrial situs, ventricular loop orientation, relative arterial positions}), consistent with L-TGA, also known as a “ventricular switch” as the pulmonary and systemic circuits remain separate, but with the wrong ventricle. On chest radiography, a straightened left heart border can be appreciated, and on CT, the great vessels will appear parallel in almost the same coronal plane.