Abstract

The prognosis for surgical repair of Ebstein's anomaly depends on the tricuspid valve repair and on the right ventricular function. In order to decrease the preload of the compromised right ventricle, a bi-directional cavopulmonary shunt was added to the intracardiac repair. Among 113 patients operated on for Ebstein' s anomaly at our institution from 1980-1997, a cohort of 60 patients was selected for high risk for postoperative complications. Patients presented with one or more several criteria: massive tricuspid valve dysfunction, extended atrialized right ventricle, poor right ventricular contractility, or long standing atrial fibrillation. After prior informed consent, this cohort was divided into two groups. Both groups had similar preoperative clinical patterns: Group I (45 patients), surgical technique included longitudinal right ventricular plication and tricuspid valve valvuloplasty; Group II (15 patients), where the surgical technique was similar to Group I except a bi-directional cavopulmonary shunt was added at the end of the procedure. Operative mortality was 24% (11/45) in Group I and 0% (0/15) in Group II (P < 0.05). The survival at 5 years was 66.1 +/- 14% in Group I and 80 +/- 16% in Group II (not significant). Reoperation rate was 11% (5/45) in Group I and 0% (0/15) in Group II. No deleterious effects of the bi-directional cavopulmonary shunts were observed clinically. Residual tricuspid valve insufficiency rate was 26% in both groups. However, patients with the bi-directional cavopulmonary shunt had a better tolerance and have not needed reoperations to date. In high risk patients with Ebstein's anomaly, an associated bi-directional cavopulmonary shunt seems to offer several distinct advantages including decreased operative mortality and better tolerance of the residual tricuspid valve dysfunction.

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