Surgical Repair of Ebstein's Anomaly Using a Bicuspidization Approach.

BACKGROUND

Ebstein's anomaly of the tricuspid valve is a rare and heterogeneous form of congenital heart defect. The purpose of this study was to review the surgical experience with Ebstein's anomaly at a single institution.

METHODS

This was a retrospective review of 47 patients with Ebstein's anomaly who were previously unrepaired and underwent surgical repair of the tricuspid valve. Our surgical approach repairs the leaflets at the preexisting level and does not attempt to reposition the hinge points at the anatomic annulus. The median age at surgery was 17 years (range, 2-53). Preoperatively the median degree of tricuspid regurgitation was graded as moderate-to-severe, and the median right ventricular function was slightly below normal.

RESULTS

Forty-seven patients underwent surgical repair without any mortality. Forty-three patients (91%) had a successful repair as evidenced by a decrease in the amount of tricuspid regurgitation to trace/mild. Four patients (9%) had an unsuccessful initial repair. Thirteen patients (29%) underwent a concomitant bidirectional Glenn procedure. Eight patients (17%) required late reoperation. Seven of these 8 patients initially had undergone a successful repair but returned with recurrent tricuspid regurgitation. Six of the 8 late reoperations entailed a re-repair, whereas 2 (4%) required tricuspid valve replacement.

CONCLUSIONS

The bicuspidization surgical technique resulted in 91% percent of patients undergoing a successful initial repair. These results suggest this technique may be a viable alternative to the "cone" procedure.