Long-term follow-up after repair of Ebstein's anomaly

OBJECTIVE: We studied the long-term results of vertical plication repair of Ebstein's anomaly according to Carpentier.

METHODS: Between 1988 and 2007, 28 patients (mean age 28.8+/-15.7 years, range 4-58 years) underwent vertical plication repair of Ebstein's anomaly. At operation the anomaly was classified according to Carpentier. In three patients (11%) a cavopulmonary shunt was added at the repair on the indication of impaired right ventricular function.

RESULTS: There was no operative mortality. Early mortality was 3.6% (one patient). Actuarial survival and actuarial freedom from reoperation at 19 years were 96% (95% CI; 96-97%) and 72% (95% CI; 53-92%), respectively. Six patients required reoperation, with a successful re-repair in three patients. Mean duration of follow-up was 10.7+/-6.5 years. One year postoperatively, tricuspid incompetence had decreased significantly (p<0.001), as had New York Heart Association (NYHA) functional class (p<0.001). In addition, exercise tolerance had increased (70+/-19% to 92+/-9% of predicted values, p<0.05). Both tricuspid function and NYHA functional class remained essentially unchanged at the end of follow-up, indicating durable haemodynamic and functional results.

CONCLUSION: This study demonstrates favourable long-term results following vertical plication repair of Ebstein's anomaly with low mortality, acceptable morbidity and good haemodynamic and functional results.