Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle

ObjectiveRepair of Ebstein anomaly and impaired right ventricular function pose challenges for the cardiac surgeon. The bidirectional cavopulmonary shunt may improve early outcomes. We reviewed our experience with the 1.5-ventricle repair in this patient population.MethodsBetween July 1999 and March 2006, 169 patients underwent operations to repair Ebstein anomaly. Fourteen patients had a bidirectional cavopulmonary shunt constructed. The median age at operation was 6 years (17 months-57.8 years). All of the patients had severe Ebstein anomaly with dilated right-sided chambers and/or right ventricular dysfunction. The mean left ventricular ejection fraction was 54.5% (range 35%–72%). Three patients were initially referred for heart transplantation, and the bidirectional cavopulmonary shunt allowed a conventional repair.ResultsProcedures included bidirectional cavopulmonary shunting (14), tricuspid valve replacement (11), tricuspid valve repair (2), and right ventricular resection (3). Shunting was planned preoperatively in 9 patients; the indication in 5 other patients was hemodynamic instability after separation from cardiopulmonary bypass. One patient died of multiple organ failure. Median follow-up in 10 patients was 18 months (3 months-6.5 years). The preoperative left ventricular ejection fraction of less than 50% improved in 3 patients to greater than 50% postoperatively.ConclusionsThe 1.5-ventricle repair can be utilized in patients with severe Ebstein anomaly and impaired right ventricular function who are at high risk for surgical treatment. We believe the bidirectional cavopulmonary shunt may be considered as a planned procedure, as an intraoperative salvage maneuver, or as an alternative to cardiac transplantation in selected patients.