Circulation Cardiovascular Case Series

A 21-year-old man with a history of Ebstein anomaly and atrial fibrillation who had previously undergone tricuspid valve repair, pulmonary venous isolation, and the Cox maze III procedure was referred from an outside institution for the evaluation of an acute onset of left-sided upperand lower-extremity weakness. The patient had initially presented 3 years earlier with abdominal pain and symptomatic palpitations. Physical examination at that time revealed a jugular venous pressure of 12 cm without a prominent V wave. Cardiac palpation noted a sternal heave. On auscultation, a grade II holosystolic murmur was appreciated at the left sternal border that increased with inspiration. Abdominal inspection noted an enlarged liver that was not pulsatile. A standard 12-lead ECG demonstrated sinus rhythm with first-degree atrioventricular block (Figure 1), and a chest x-ray noted enlargement of the right atrium (Figure 2). A 24-hour Holter monitor noted that the predominant rhythm was atrial fibrillation interrupted by brief periods of sinus rhythm. Transthoracic echocardiography demonstrated severe right atrial enlargement, dilatation of the tricuspid annulus, apical displacement of the tricuspid valve (displacement index, 10.2 mm/m2), large anterior tricuspid valve leaflet, and severe tricuspid regurgitation consistent with Ebstein anomaly.