SOME CLASSICAL SIGN IN CONGENITAL HEART DISEASE

1. Classic Imaging Signs of Congenital
Cardiovascular Abnormalities
DR.RIYADH W. AL ESAWI
DMRD, MSc, PhD
Diagnostic radiology
Assist. Prof. faculty of medicine/kufa university

2. The field of cardiovascular imaging is rapidly
expanding, and it is important that
radiologists &physicians be familiar with the
classic signs of congenital cardiovascular
abnormalities in both pediatric and adult
patients. An understanding of the origin and
pathophysiologic significance of each of these
signs is essential in daily radiologic practice.

3. 1-TPGA
In transposition of the great arteries (TPGA), the pulmonary
artery is situated to the right of its normal location
and is obscured by the aorta on frontal chest radiographs.
This malposition, in association with stress induced thymic
atrophy and hyperinflated lungs, results in the apparent
narrowing of the superior mediastinum on radiographs,
the most consistent sign of transposition of the great
arteries. The cardiovascular silhouette varies from normal
in the first few days after birth to enlarged and globular,
with the classic appearance described as an egg on a string.

4. 1-Egg on string
Chest radiograph obtained in a
neonate shows narrowing of
the superior mediastinum,
enlargement of the cardiac
silhouette with abnormal
convexity of the right atrial
border, and increased vascular
flow—typical features of
transposition of the
great arteries.

5. 2-SNOW MAN
TAPVR; 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.
In type I ... (55% of cases), the anomalous
pulmonary veins terminate at the supracardiac
level. On chest radiographs, this cardiovascular
anomaly resembles a snowman.

6. SNOW MAN

7. TAPVR 11&111
Type II TAPVR (30% of cases) involves a pulmonary venous
connection at the cardiac level. The pulmonary veins join
either the coronary sinus or the right atrium.
Type III TAPVR (13% of cases) involves a connection at the
infracardiac or infradiaphragmatic level. The pulmonary
veins join behind the left atrium to form a common vertical
descending vein, which courses anterior to the esophagus
and passes through the diaphragm at the esophageal
hiatus. This vertical vein usually joins the portal venous
system but occasionally connects directly to the ductus
venosus, the hepatic veins, or the inferior vena cava. Type
III TAPVR is virtually always accompanied by some degree
of obstructed venous return.

8. 3-Scimitar sign
The scimitar sign is produced by an anomalous
pulmonary vein that drains any or all of the lobes
of the right lung. The so-called scimitar 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.
Although the diameter of the scimitar vein
depends on whether it drains the entire right
lung or only a portion of it, the diameter
generally increases as the vein descends

9. 3-Scimitar sign

10. 4-Gooseneck deformity
• The gooseneck-shaped deformity in endocardial
cushion defect is caused by a deficiency of both
the conus and sinus portions of the
Interventricular septum, with narrowing of the
left ventricular outflow tract. The concavity of the
interventricular septum below the mitral valve,
along with the elongation and narrowing of the
left ventricular outflow tract, produces a
characteristic shape that has been compared to a
sitting goose with an elongated neck on the
anteroposterior projection in left ventricular
angiography.

11. 4-Gooseneck deformity
Endocardial cushion defects,
which account for 4% of all cases
of congenital heart disease,

12. 5-Boot shape
Tetralogy of Fallot accounts for 10%–11% of cases of
congenital heart disease. On chest radiographs in
those affected by this syndrome, the heart has the shape
of a wooden shoe or boot (in French, coeur en
sabot). This deformity is due to uplifting of the cardiac
apex because of right ventricular hypertrophy and
concavity of the main pulmonary artery. The shadow of
the pulmonary arterial trunk is almost invariably
absent, and blood flow to the lungs is usually reduced.

13. 5-BOOT SHAPE
Ventricular septal defect (1), infundibular pulmonary stenosis
(2), overriding aorta (3), and right ventricular hypertrophy (4).
The oxygen-rich blood in the left side of the heart (5) mixes
with oxygen-poor blood in the right side of the heart (6) before
it proceeds to the aorta (7).

14. 6-Ebestine anomaly
Ebstein anomaly is characterized by the downward displacement of
the septal leaflets and posterior leaflets of the tricuspid valve into
the inflow portion of the right ventricle. This displacement results
in the formation of a common right ventriculoatrial chamber and
causes tricuspid regurgitation. The most consistent
imaging feature is right atrial enlargement; the right atrium may be
huge and fill the entire right hemithorax. The left atrium is normal
in size, but the left cardiac contour has a shelved appearance
because of the dilated right ventricular outflow tract. The aorta is
small, and the pulmonary trunk, which normally appears as a
discrete convex bulge, is absent. This combination of features
produces a cardiac silhouette that has been described as box
shaped

15. 6- Box shape
Ebstein anomaly, first
described by German
physician Wilhelm Ebstein
in 1866, accounts for 0.5%–
0.7% of cases of congenital
heart disease

16. 7- coarctation of the aorta
Two classic radiologic signs associated with aortic
coarctation are the figure-of-three sign and the
reverse figure-of-three sign. The aortic segment
affected by coarctation has a shape that
resembles the number 3 on frontal chest
radiographs. The reverse figure-of-three sign, a
mirror image of the number 3, is observed on the
left anterior oblique view during barium
esophagography. This sign is seen in 50%–66% of
adults with aortic coarctation

17. 7-figure 3 sign

18. Coarctation
Coarctation of the aorta accounts for 5%–10%
of congenital cardiac lesions and is usually sporadic.
However, it occurs with increased frequency
among patients with Turner syndrome,
20%–36% of whom are affected. Clinical
manifestations vary from congestive heart failure
in infancy to hypertension with differential
pressures between the upper and lower
extremities in adulthood.

19. I AA
According to the Celoria-Patton classification of
IAA , type A is defined as interruption distal to
the left subclavian artery (42% of cases); in
type B, the interruption is between the left
common carotid artery and subclavian artery
(53%), finally, in type C, the interrupted
segment is between the innominate artery
and left common carotid artery (4%).

20. Interupted Coarctation of the aorta
30 y ,F ,HYPERTENSIVE