ABSTRACT
Ebstein’s anomaly is a rare congenital heart anomaly of the tricuspid valve and right ventricle. It is characterized by abnormal adherence of the tricuspid valve leaflets to the underlying myocardium, and displacement of the functional tricuspid valve orifice more apically and anteriorly into the right ventricle and towards the right ventricular outflow trace. In adults with Ebstein’s anomaly, echocardiography is difficult to perform because of the heart dislocation and dilatation. Adequate depth of the examination has to be selected. The critical feature for establishing a diagnosis of Ebstein’s anomaly is the apical shift of the tricuspid leaflet coaptation site, which is ideally verified using color Doppler mapping in the presence of tricuspid regurgitation. One-and-half ventricular repair is usually performed in high-risk patients with a severely reduced size of the right ventricle, or a severely dysfunctional and enlarged right ventricle, extended atrialized right ventricle and long-standing atrial fibrillation.
