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

Background. - 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. Objectives. - We report our initial experience with the cone procedure versus medical follow-up. Methods. - Patients aged >= 15 years 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. Results. - The cone procedure was performed in 20 patients (mean age 34.3 +/- 14.4 years; 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. Conclusion 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. (C) 2017 Elsevier Masson SAS. All rights reserved.