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

^a Department of Cardiothoracic Surgery, Thoraxcentre, Erasmus Medical Centre Rotterdam, Rotterdam, The Netherlands
^b Department of Cardiothoracic Surgery, University Hospital Maastricht, Maastricht, The Netherlands
^c Department of Cardiology, Thoraxcentre, Erasmus Medical Centre Rotterdam, Rotterdam, The Netherlands

Received 12 November 2007; received in revised form 27 February 2008; accepted 21 March 2008.

^_* Corresponding author. Address: Department of Cardiothoracic Surgery, University Hospital Maastricht, P. Debeyelaan 25, PO Box 5800, 6202AZ Maastricht, The Netherlands. Tel.: +31 433877070; fax: +31 433875070. (Email: meindert_palmen{at}hotmail.com).

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.

Key Words: Cardiac congenital • Cyanotic • Acyanotic • Tricuspid valve