Results after surgical repair of Ebstein's anomaly.

BACKGROUND: Ebstein's anomaly of the tricuspid valve is a complex malformation. Various operations have been undertaken with varying results. Because valve replacement yielded poor results, surgical treatment has focused on valvuloplasties.

METHODS: Between April 1974 and February 1995, 60 patients with Ebstein's anomaly underwent surgical repair. Age ranged from 5 months to 54 years. In 56 patients (93.3%), tricuspid valvuloplasty was feasible, mainly by creating a monocusp valve with the single-stitch technique. The other 4 patients had valve replacement with a bioprosthesis. Six reoperations were necessary (10.0%): four valve replacements and two repeat valvuloplasties.

RESULTS: There were two hospital deaths (3.3%) and a late mortality rate of 10.0% (6 patients). Forty-nine (94.2%) of 52 survivors were followed for 5 months to 18.6 years (median follow-up, 5.0 years; mean follow-up, 6.9 years). The actuarial survival rate (Kaplan-Meier) was 96.5% +/- 2.4% at 1 year and 83.3% +/- 5.6% at 18 years. At follow-up evaluation, nearly all patients showed substantial improvement (93.9% were in functional class I or II) compared with their preoperative status. Doppler echocardiographic studies demonstrated good tricuspid valve function in most patients.

CONCLUSIONS: Valvuloplasty using the single-stitch technique is a rewarding operation. It yields good long-term results with substantial improvement in functional performance and clinical status.