Single-stage, three-fold repair for Ebstein's anomaly

Objectives: Favourable outcomes in the repair of Ebstein's anomaly are predicated on tricuspid valve competence, right ventricular function and presence of arrhythmia. We report our experience with a single-stage, three-fold repair of Ebstein's anomaly, namely, cone reconstruction of the tricuspid valve supplemented by bidirectional cavopulmonary anastomosis and right atrial electrocautery maze. Methods: From 2010 to 2014, 37 consecutive patients with Ebstein's anomaly, median age 17.3 (9.1-56.2) years, underwent this single-stage, three-fold surgical procedure. The principal elements of the procedure include (i) cone reconstruction of the tricuspid valve, limited plication at the level of the displaced valve, insertion of a homemade annuloplasty ring, defect repair and reduction atrioplasty supplemented by (ii) right atrial electrocautery maze and (iii) bidirectional cavopulmonary anastomosis. Postoperatively, all patients were followed up regularly for a mean period of 2.3 (1-4) years by clinical, electrocardiographic and echocardiographic examinations. Results: The in-hospital mortality rate was 2.7% (1 patient) with no late deaths. Mean cardiopulmonary bypass time was 110 ± 18.3 min and aortic cross-clamp time was 48.5 ± 7.4 min. Echocardiographic examination showed significant improvement of valve regurgitation (P < 0.0001). NYHA functional class was I in 77.8% of the survivors and II in 22.2%. The cardiothoracic ratio decreased significantly (P < 0.05). No deleterious effects of the Glenn shunts have been reported. Sinus rhythm has remained stable in 31 patients (86.1%) during the follow-up period. Conclusions: Single-stage, three-fold repair for the management of Ebstein's anomaly offers good outcome in terms of low mortality and morbidity rates. It can achieve a durable valve-sparing repair, good functional mid-term outcomes and good quality of life among survivors.