Ebstein's anomaly: repair based on functional analysis

'Classical' repair of Ebstein's anomaly is usually performed with transverse plication of the atrialized chamber. However, the anterior leaflet has restricted motion which is an important factor of the tricuspid valve insufficiency. We studied the long term results of mobilization of the anterior leaflet associated with longitudinal plication of the right ventricule.

From 1980 to July 2002, 191 patients (mean age 24.4+/-15 years (1-65)) were operated on. Anterior leaflet function was assessed on pre-op echocardiography and on surgical examination. Conservative surgery was possible in 187 patients (98%) and included mobilization of the anterior leaflet, longitudinal plication of the right ventricle and prosthetic annuloplasty in adults. Bidirectional cavo-pulmonary shunt was associated in 60 patients. Four patients had valve replacement.

Hospital mortality occurred in 18 patients: 9% (95%CL: 6-15%) due to right ventricle (RV) failure in nine patients. Mean follow-up was 6.4 years (0.07-22). Actuarial survival was 82% at 20 years. Tricuspid valve insufficiency was 1 or 2+ in 80% of the cases. Reoperation occurred in 8% (16 patients). A successful second repair was obtained in ten patients. Electron beam computerized tomography (20 patients) demonstrated improved left ventricle ejection fraction 56-66% (P<0.05). Supraventricular tachycardia and pre-excitation syndromes were reduced from 23 to 5%.

Conservative surgery is indicated for all symptomatic patients. The incidence of valve repair is high when leaflet mobilization is performed. Valve replacement can be avoided in most cases. Functional and hemodynamic results are excellent.