Early and midterm results in anatomic repair of Ebstein anomaly

Objective

We report the results of surgical treatment of Ebstein anomaly during a 10-year period, especially with an anatomic repair technique that restores to near normal anatomic and physiologic function of the tricuspid valve and the right ventricle.

Methods

Between December 1997 and December 2006, 83 consecutive patients aged 9 months to 52 years underwent operation for Ebstein anomaly. Among them, 78 underwent the anatomic repair technique (male 39). Tricuspid incompetence was moderate in 22 patients and severe in 56 patients. The main surgical technique includes excision of the atrialized right ventricle, detachment and repair of the leaflet, transposition of the leaflet, and anulus plication of the tricuspid valve. In some patients in whom the septal leaflet was severely hypoplastic or absent, a new leaflet was created with autologous pericardium. Biventricular repair was not performed in 5 patients with very severely hypoplastic tricuspid valves and poor right ventricular function.

Results

All patients survived and recovered uneventfully. For the 78 patients who underwent anatomic repair, postoperative echocardiography showed that tricuspid incompetence disappeared in 65 patients and was mild in 13 patients. Right ventricular function was good. Patients have been doing well during a mean follow-up of 46 months (2 months to 9 years). The latest follow-up echocardiograms showed that tricuspid incompetence was eliminated in 55 patients and was mild in 17, moderate in 3, and severe in 3. The 5 patients with a total cavopulmonary connection procedure and one-and-one-half-ventricle correction procedure are also doing well.

Conclusions

Satisfactory early and midterm results can be achieved with the anatomic repair technique to correct Ebstein anomaly. Tricuspid valve replacement can be avoided in most patients.

CTSNet classification: 20, 21, 35