Ebstein's anomaly in adults: Doppler-echocardiographic evaluation

A systematic approach and segmental analysis are required for comprehensive assessment including both morphologic and functional abnormalities associa ted with Ebstein's anomaly. The essence of the disease is an apical displac ement of both the septal and the posterior tricuspid leaflets, exceeding 20 mm or 8 mm/m(2) in adults. As a consequence, the right heart consists of t hree components including the true right atrium, the functional right ventr icle (RV) and an intervening zone that is anatomically Ventricular but func tionally right atrial (atrialized RV). The thin wall of the atrialized RV m ay result in an aneurysm between the anatomic tricuspid annulus and the api cally displaced posterior leaflet. The annular attachment of the anterior l eaflet is normal, which may be dysplastic and adherent to the RV wall. Tric uspid regurgitation is usually moderate to severe. Size, shape and function of the functional RV must be described. The paradoxical motion of the inte l interventricular septum causes alterations in left Ventricular geometry a nd function. An interatrial communication is frequently present. Echocardio graphy is the method of choice to diagnose Ebstein's anomaly on its own or in association with other heart defects.