Ebstein's anomaly in adults: Modified cone reconstruction of the tricuspid valve is associated with promising outcomes

Abstract

Ebstein's anomaly is a complex malformation. Justification of a procedure in mildly symptomatic adults is debatable: repair techniques are demanding and valve replacement is associated with poorer outcome. We report our initial experience with the cone procedure versus medical follow-up. Patients aged≥15years with Ebstein's anomaly were enrolled during 2007-2014. The cone procedure was performed in consecutive patients with severe tricuspid regurgitation (TR); those with less severe disease did not undergo surgery, although some underwent percutaneous catheter atrial septal defect closure. The cone procedure was performed in 20 patients (mean age 34.3±14.4years; TR grade 3.3±0.7) because of impaired functional capacity: six New York Heart Association (NYHA) class II, 14 class III. No surgical patient died during a mean (range) follow-up of 2.8 (0.5-5.0) years. One patient required subsequent repair for suture dehiscence 6 months postoperatively. All patients presented with mild or less TR at last echocardiographic follow-up. NYHA functional class was significantly improved at follow-up (P<0.0001): 16 NYHA class I and four class II. Of 24 non-surgical patients (mean age 37.3±16.9 years; TR grade 2.2±0.8), seven underwent percutaneous ASD closure. During a mean (range) follow-up of 4.8 (3.6-5.0) years, two patients died: one sudden death and one stroke. The cone repair of the tricuspid valve in adults with Ebstein's anomaly provided excellent mid-term results and significantly improved functional status. This procedure might be considered even in mildly symptomatic patients in the presence of severe valve regurgitation.