Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair

Objective We sought to review our institutional experience and midterm results with cone reconstruction in Ebstein anomaly with or without one and a half ventricle repair to evaluate these 2 important surgical methods for the treatment of Ebstein anomaly. Methods From January 1, 2004, to October 1, 2009, 30 consecutive patients with Ebstein anomaly underwent cone reconstruction. All patients underwent cone reconstruction, and 20 patients with severe Ebstein anomaly had a bidirectional cavopulmonary shunt constructed. The median age was 60 months (range, 2–192 months). Our criteria to define severe Ebstein anomaly include a severely enlarged right-sided chamber, significant abnormality of the septal leaflet of the tricuspid valve, and hemodynamic instability after cardiopulmonary bypass. Clinical or echocardiographic characteristics were studied both preoperatively and postoperatively. Results There was 1 (3.3%) hospital death. Before the operation, tricuspid incompetence was moderate in 8 and severe in 22 patients. Postoperative early echocardiographic analysis showed that tricuspid incompetence was mild in 26 patients, moderate in 3 patients, and severe in 1 patient. After a median follow-up time of 22 months, tricuspid incompetence of 20 patients with bidirectional cavopulmonary shunt was mild in 15 and moderate in 4. Tricuspid incompetence of 10 patients without a bidirectional cavopulmonary shunt from the latest echocardiogram (median follow-up time, 32 months) was mild in 4, moderate in 4, and severe in 2 patients. For patients whose postoperative tricuspid incompetence was beyond mild among the 10 patients without a bidirectional cavopulmonary shunt, preoperative echocardiographic analysis shows a severely dilated right ventricle. Conclusions Addition of a bidirectional cavopulmonary shunt to cone reconstruction of the tricuspid valve should be considered for young patients with severe Ebstein anomaly who are at high risk of right ventricular failure after the operation.