A new procedure for Ebstein's anomaly.

BACKGROUND: A new procedure for correction of Ebstein's anomaly that restores to near normal the anatomic and physiologic function of the tricuspid valve and the right ventricle is reported.

METHODS: Between December 1997 and September 2002, 34 consecutive patients with Ebstein's anomaly underwent this new procedure. There were 13 male and 21 female patients aged 9 months to 48 years (mean, 17 years). Tricuspid incompetence was moderate in 12 patients and severe in 22. Our repair technique is as follows: the displaced posterior leaflet with some chordae tendineae and corresponding papillary muscle are detached from the annulus and ventricular wall, respectively. The leaflet is then reattached to the native posterior annulus with reimplantation of the papillary muscle. The displaced septal leaflet is treated in the same manner. Most of the atrialized portion of the ventricular wall is excised; the tricuspid annulus is plicated. In 8 of the patients the septal leaflet was severely hypoplastic and necessitated creation of a new leaflet using autologous pericardium.

RESULTS: All patients survived and recovered uneventfully. Postoperative echocardiography showed that tricuspid incompetence disappeared in 29 patients and was mild in 5. Right ventricular size decreased significantly with complete disappearance of the atrialized segment. Follow-up of patients ranged from 1 to 55 months (mean, 25 months), with 9 patients having more than 3 years of follow-up. They are doing well and their exercise tolerance improved to normal.

CONCLUSIONS: This new procedure anatomically corrects Ebstein's anomaly with the satisfactory early and midterm results.