Ebstein anomaly (EA) is a congenital defect of the tricuspid valve (TV) and the 
right ventricle (RV) in which the attachments of the septal and posterior valve 
leaflets are apically displaced. The latter creates 3 morphologic components 
inside the right heart, namely the right atrium proper, the atrialized RV, and 
the functional RV. This rare anomaly accounts for <1.5% of all congenital heart 
diseases. The current opinion among authors is that it is a genetically 
heterogeneous condition caused by failure of delamination of the TV leaflets 
from the underlying myocardium and the interventricular septum. Its 
characteristic electrocardiographic findings include tall, broad, right atrial P 
waves, prolonged PR intervals, and deep Q waves in the right precordial leads. 
Echocardiography is currently the best technique for diagnosing this anomaly, 
although cardiac magnetic resonance imaging is also gaining traction as an 
alternative modality. The management strategies for EA correlate with the age of 
the patient, severity of the heart disease, and/or associated cardiac 
abnormalities. TV repair, rather than valve replacement, is preferred because of 
its favorable long-term prognosis. Nevertheless, a large, randomized study is 
still needed to compare the different valve repair techniques used in patients 
with EA.