Congenital Heart Disease Recognition Flashcards

A fetal echocardiogram reveals the liver on the left, stomach on the right, and apex of the heart pointing to the right. This is best described as:

Answer: Situs inversus totalis

Situs inversus totalis is a complete mirror-image arrangement of all thoracoabdominal organs including the heart apex pointing right.

Which echocardiographic feature is used to identify the morphologic right ventricle in complex congenital heart disease?

Answer: Moderator band, coarser trabeculations, and tricuspid valve attached lower on the septum

The morphologic right ventricle is identified by its coarse trabeculations, moderator band, and a tricuspid valve whose septal leaflet inserts more apically than the mitral valve.

In congenitally corrected transposition of the great arteries (ccTGA), the circulation is:

Answer: Physiologically corrected because both ventricles are discordant twice

In ccTGA, atrioventricular and ventriculoarterial discordance cancel each other out, resulting in a physiologically corrected but anatomically abnormal circulation.

Which fetal cardiac defect is most likely responsible for a markedly enlarged, globular heart occupying most of the thorax with severe tricuspid regurgitation?

Answer: Severe Ebstein anomaly

Severe Ebstein anomaly can cause massive cardiomegaly and severe tricuspid regurgitation due to the markedly displaced and dysplastic tricuspid valve.

On a fetal echocardiogram the pulmonary veins are seen draining into a confluence behind the left atrium which then drains via a vertical vein downward through the diaphragm. This is consistent with:

Answer: Infracardiac TAPVR

Infracardiac TAPVR is characterized by pulmonary veins draining to a confluence that connects via a descending vertical vein through the diaphragm to the portal or hepatic venous system.

The 'Y-sign' on the subcostal sweep in fetal echocardiography refers to:

Answer: The venous confluence of TAPVR joining a vertical vein, resembling a Y-shape

The Y-sign describes the echocardiographic appearance of pulmonary veins converging into a confluence that then drains via a vertical vein, forming a Y-shaped structure diagnostic of TAPVR.

A fetal echocardiogram demonstrates absence of the expected interventricular septal tissue at the crux of the heart, with a large defect straddling AV valves. The most likely diagnosis is:

Answer: Complete atrioventricular septal defect (AVSD)

Loss of the normal crux anatomy with a common AV junction and inlet VSD is the defining feature of complete AVSD.