Repair of Ebstein anomaly: early and mid-term results.

Background: Ebstein anomaly is the downward displacement and adherence of dysplastic septal and posterior tricuspid leaflets into the right ventricle, thereby dividing the ventricle into a so-called atrialized chamber and a functionally reduced right ventricle. We evaluated the early and mid-term results of primary repair of Ebstein anomaly in adult patients.

Methods: Eight consecutive patients undergoing repair of Ebstein anomaly with Danielson technique at Imam Khomeini Medical Center, Tehran, Iran from January 1997 through July 2004 were evaluated. Functional and echocardiographic parameters were studied both preoperatively and postoperatively, as well as demographic status and adverse events.

Results: Hospital mortality occurred in one patient because of right ventricular failure. The average follow-up period was 5.3 +/- 3.4 years (median: 3.8 years). The actuarial survival rate was 85.7 +/- 4.8% at 7 years. During the follow-up, six patients were in New York Heart Association functional class I, and one patient was in class II. None of the patients required reoperation related to their Ebstein repair. One patient demonstrated atrioventricular dissociation perioperatively; however, only one patient required permanent pacemaker insertion later. One patient had minimal (1+) regurgitation, with the jet localized along the anterior part of the ventricular septum. Two patients had residual tricuspid valve insufficiency (2+) on echocardiography.

Conclusion: Ebstein repair has a good functional and hemodynamic outcome in adult patients.