Abstract

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.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 pre-existing level and does not attempt to reposition the hingepoints at the anatomic annulus. The median age at surgery was 17 years (range 2 to 53 years). Pre-operatively, the median degree of tricuspid regurgitation was graded as moderate-to-severe and the median right ventricular function was slightly below normal.Forty-seven patients underwent surgical repair without any mortality. Forty-three of the 47 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 of the 47 (29%) underwent a concomitant bi-directional Glenn procedure. There were 8 patients (17%) who have required late re-operation. Seven of these 8 patients initially had undergone a successful repair but returned with recurrent tricuspid regurgitation. Six of the 8 late re-operations entailed a re-repair, while two (4%) required ; tricuspid valve replacement.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.

View details for DOI 10.1016/j.athoracsur.2019.06.026

View details for PubMedID 31394091