To evaluate the early and long-term outcomes cardiac surgery of patients with Ebstein anomaly.

The clinic data of 237 patients with Ebstein anomaly received surgical procedures from March 2004 to December 2017 at Department of Cardiac Surgery, First Hospital of Tsinghua University was analyzed retrospectively. There were 105 male and 132 female patients with age of (19.4±16.7) years (ranging from 3 months to 64 years). The surgical procedures include anatomical repair in 188 patients, one and a half ventricle repair in 37 patients, tricuspid valve repair in 4 patients, tricuspid valve replacement in 10 patients, and Fontan procedure in 3 patients (total cavopulmonary connection in 2 patients; Glenn procedure in 1 patient).

The early mortality was 2.1% (n=5). One case of atrioventricular (0.4%) newly occurred. There were 228 patients available to follow-up. The range of follow-up duration was 3 to 168 months. Late survival was 99.1% (2 cases of late death) at 10 years. Three patients received reoperation (1.3%), including tricuspid valve repair of 1 patient and one and a half ventricle repair of 2 patients). Indication of tricuspid valve regurgitation improved from 3.6±0.3 to 1.5±0.4. Survival rate at 5 and 10 years was 98.6% and 98.2%, respectively.

The principle of the techniques is to reconstruct the tricuspid valve and right ventricle anatomically. For most cases, the anatomical repair was demonstrated with low mortality, less complications and excellent durability at long-term follow-up. If the tricuspid valve is severely hypoplastic, one and a half ventricle repair and valve replacement may be alternatie.

Ebstein anomaly; Cardiac surgical procedures; Treatment outcome