Ebstein Anomaly in the Adult Patient

Ebstein anomaly is a congenital malformation involving primarily the tricuspid valve, with failure of delamination from the underlying myocardium and right ventricular myopathy. Echocardiography is diagnostic in most patients and demonstrates apical displacement of the septal leaflet and variable tethering of leaflet tissue to the right ventricular myocardium. Operative intervention is considered for exertional symptoms, progressive right ventricular enlargement, or right ventricular dysfunction. Tricuspid valve cone repair is the preferred surgical approach. Tricuspid valve replacement and bidirectional cavopulmonary shunt also are considered in patients with advanced disease. Pregnancy generally is well tolerated. Patients with Ebstein anomaly require lifelong follow-up.