Ebstein’s Anomaly

Abstract

Ebstein’s anomaly of the tricuspid valve causes apical displacement of the effective orifice of the tricuspid valve resulting in large right atrium and smaller right ventricle. Ebstein’s anomaly is associated with bypass tracts between the right atrium and ventricle which may precipitate supraventricular tachycardia. Hemodynamic effects of the abnormal tricuspid valve vary from mild to severe. In mild cases there are limited or no symptoms and signs. In severe cases the tricuspid valve is severely regurgitant and the right ventricular outflow tract is obstructed. These changes will lead to high right atrial pressure and right to left shunting at the foramen ovale leading to cyanosis. Surgical repair of tricuspid valve is reserved to severe cases. Patients with small right ventricle may eventually require Fontan procedure.