What is Ebstein anomaly and what is the key echocardiographic finding?

Prepare for the Fetal Echocardiography Test. Utilize flashcards and multiple-choice questions that come with hints and explanations. Ace your exam preparation!

Ebstein anomaly presents as downward (apical) displacement of the tricuspid valve leaflets, especially the septal and posterior leaflets. Because the leaflets are displaced toward the apex, a portion of the right ventricle near the apex is effectively incorporated into the right atrium—this is the atrialization of part of the RV. The functional right ventricle becomes small, while the right atrium enlarges due to chronic severe tricuspid regurgitation and volume overload. On echocardiography, the hallmark finding is the apical displacement of the tricuspid valve with an atrialized portion of the RV and a relatively small functional RV, often accompanied by marked RA enlargement and severe TR on Doppler. This constellation distinguishes Ebstein anomaly from other defects such as left ventricular hypoplasia, a large VSD without outflow obstruction, or a persistent ductus arteriosus.