NBE Pediatric Echocardiography 5

Question 1

A child with Williams syndrome is referred for echocardiography. Which cardiovascular lesion is most commonly found in this condition?

Accepted Answer

Supravalvar aortic stenosis

Answer

Williams syndrome (elastin gene deletion) is most commonly associated with supravalvar aortic stenosis, characterized by narrowing of the ascending aorta just above the sinuses of Valsalva.

Question 2

During a neonatal echocardiogram, you identify that the pulmonary veins drain into the left atrium but the left atrium connects to the right ventricle via the mitral valve. What is the most likely diagnosis?

Accepted Answer

Corrected transposition of the great arteries (L-TGA)

Answer

In L-TGA (congenitally corrected transposition), there is both atrioventricular discordance (left atrium connects to right ventricle via mitral valve) and ventriculoarterial discordance.

Question 3

Which Doppler measurement is the gold standard for assessing diastolic dysfunction in children with cardiomyopathy?

Accepted Answer

Tissue Doppler imaging of the mitral annulus (e' velocity) combined with E/e' ratio

Answer

Tissue Doppler-derived mitral annular e' velocity and the E/e' ratio provide load-independent assessment of diastolic function and are the preferred parameters in pediatric cardiomyopathy.

Question 4

In a neonate with suspected total anomalous pulmonary venous return (TAPVR), which finding on echocardiography indicates obstructed infracardiac TAPVR?

Accepted Answer

High-velocity turbulent flow in the descending vertical vein below the diaphragm with severe pulmonary hypertension

Answer

Obstructed infracardiac TAPVR is characterized by high-velocity turbulent flow in the vertical vein as it traverses the diaphragm (portal system obstruction), resulting in severe pulmonary venous hypertension.

Question 5

A 12-year-old athlete has an echocardiogram showing concentric left ventricular hypertrophy with a non-dilated LV cavity. Which condition must be distinguished from athlete's heart?

Accepted Answer

Hypertrophic cardiomyopathy (HCM)

Answer

Hypertrophic cardiomyopathy presents with asymmetric or concentric LVH and non-dilated LV, and must be distinguished from athlete's heart because HCM carries risk of sudden cardiac death with exercise.

(NBE) National Board of Echocardiography Practice Test

(NBE) National Board of Echocardiography Practice Test

NBE Pediatric Echocardiography 5