Congenital Heart disease and

vascular abnormality
Jeetendra

Normal Chest X-ray(PA view)

Index
RA right atrium
RDPA right descending
pulmonary artery
RPA right main pulmonary
artery
SVC superior vena cava
AA aortic arch
DA proximal descending
thoracic aorta
LPA left pulmonary artery
RV right ventricle

Normal chest X-ray(Lateral View)

Index:

RV right ventricle
RSS retrosternal clear space
AA ascending aorta
LPA left pulmonary artery
RPA right pulmonary artery
en face
IVC inferior vena cava
LA left atrium
LV left ventricle

Congenital Heart Disease

Transposition of the Great Vessels

Most common cyanotic congenital heart lesion

5%7% of congenital cardiac malformations
isolated in 90%
Produced by a ventriculoarterial discordance in which the aorta arises from the morphologic
right ventricle and the pulmonary artery arises from the morphologic left ventricle
Pulmonary artery is situated to the right of its normal location
Results in the apparent narrowing of the superior mediastinum on radiographs
Patent ASD, VSD, Foramen ovale, systemic collaterals to sustain life
Atrial border is abnormally convex, and the left atrium commonly is enlarged because of
increased pulmonary blood flow.

Transposition of great vessels

EGG ON STRING SIGN

Total Anomalous Pulmonary Venous Return

Occurs when the pulmonary veins fail to drain into the left atrium and
instead form an aberrant connection with some other cardiovascular
structure
2% of cardiac malformations

Total Anomalous Pulmonary Venous Return

SNOWMAN SIGN

Partial Anomalous Pulmonary Venous Return

Anomalous pulmonary vein drains any or all of the lobes of the right lung
Vein curves outward along the right cardiac border, usually from the
middle of the lung to the cardiophrenic angle, and usually empties into
the inferior vena cava but also may drain into the portal vein, hepatic
vein, or right atrium
Size of the vein generally increases as it descends.
Characteristic appearance of the vein has led to its comparison to a
scimitar

Partial Anomalous Pulmonary Venous Return

SCIMITAR SIGN

Endocardial Cushion Defects

Interruption of the normal development of the endocardial tissues
during gestation
Endocardial cushion forms the lower portion of the atrial septum, the
upper portion of the interventricular septum, and the septal leaflets
of the mitral valve and the tricuspid valve
4% of all cases of congenital heart disease
Gooseneck-shaped deformity
Caused by a deficiency of both the conus and sinus portions of the interventricular
septum, with narrowing of the left ventricular outflow tract.
Characteristic shape by concavity of the interventricular septum below the mitral
valve, along with the elongation and narrowing of the left ventricular outflow tract

Endocardial cushion defect GOOSENECK SIGN

Tetralogy of Fallot
10%11% of cases of congenital heart disease
As a result of single defect, an anterior malalignment of the conal
septum
Components:
Ventricular septal defect
Infundibular pulmonary stenosis
Overriding aorta
Right ventricular hypertrophy.
Heart has the shape of a wooden shoe or boot
Blood flow to the lungs is usually reduced

Tetralogy of Fallot
BOOT SHAPED SIGN

Aortic Coarctation
5%10% of congenital cardiac lesions
Produced by a deformity of the aortic media and intima, which causes a prominent
posterior infolding of the aortic lumen
Occurs at or near the junction of the aortic arch and the descending thoracic aorta
Infolding cause eccentric narrowing of the lumen at the level where the ductus or
ligamentum arteriosus inserts anteromedially
Resultant luminal narrowing in turn obstructs the flow of blood from the left
ventricle
Classic radiologic signs
Figure-of-three sign
Reverse figure-of-three sign
Rib notching on CXR pathognomonic

Aortic Coarctation
Figure of Three, and Reverse Figure of Three

Ebstein Anomaly
0.5%0.7% of cases of congenital heart disease.

Characterized by the downward displacement of the septal leaflets and posterior leaflets
of the tricuspid valve into the inflow portion of the right ventricle.
Results in the formation of a common right ventriculoatrial chamber and causes tricuspid
regurgitation.

Insufficiency of the tricuspid valve leads to dilatation of the right ventricular outflow tract
and all proximal right heart structures,
Most consistent imaging feature is right atrial enlargement

Ebstein Anomaly
Box Shaped Heart

References
Michael Y. M. Chen, Thomas L. Pope, David J. Ott. Basic Radiology.
2nd ed. Mc. Grow hill. P-86-9.
Cochard, Larry R.,Netter, Frank H. Netter's Introduction to Imaging.
Elseiver. P-54-9.

Thank you