Prepared by Medical student of Nepal. This is for educational purpose.

1. Congenital Heart disease and
vascular abnormality
Jeetendra

2. 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

3. 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

4. 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.

5. Transposition of great vessels
EGG ON STRING SIGN

6. 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

7. Total Anomalous Pulmonary Venous Return
SNOWMAN SIGN

8. 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

9. Partial Anomalous Pulmonary Venous Return
SCIMITAR SIGN

10. 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

11. Endocardial cushion defect -
GOOSENECK SIGN

12. 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

13. Tetralogy of Fallot
BOOT SHAPED SIGN

14. 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

15. Aortic Coarctation
Figure of Three, and Reverse Figure of Three

16. 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

17. Ebstein Anomaly –
Box Shaped Heart

18. 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.

19. Thank you