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

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.

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.

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.

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.