Anatomic repair of Ebstein's anomaly with isolated anterior leaflet downward displacement

Objective Ebstein's anomaly with isolated anterior leaflet downward displacement is rare and has not been reported in the literature to our knowledge. In this article, our experience of the surgical treatment in 6 cases with this anomaly is reported. Methods From November 2005 to November 2013, 6 patients (3 male, 3 female, aged 2-39 years) with Ebstein's anomaly and isolated anterior leaflet downward displacement received anatomic repair at the First Hospital of Tsinghua University. The diagnosis was made by echocardiography and confirmed at operation. Surgery was performed under hypothermic cardiopulmonary bypass. Surgical technique included excision of a huge atrialized portion of the right ventricle located in the anterior wall of the heart; reconstruction of the right ventricle by repairing the "V"-shaped defect left by the excision procedure; detachment, repair, and reimplantation of the anterior leaflet; and reconstitution of the right atrioventricular connection. Intraoperative transesophageal echocardiography was used to evaluate the position, morphology, structure, and function of the tricuspid valve, as well as right ventricular function. Results Five patients were discharged uneventfully, and 1 patient died of postoperative pneumonia. At follow-up from 2 months to 7 years, no notable tricuspid valve regurgitation or stenosis was found and all patients were maintaining a normal lifestyle. The 27-year-old female patient gave birth to a normal infant uneventfully 3 years after surgery. Conclusions Ebstein's anomaly with isolated anterior leaflet downward displacement is a complex and severe abnormality, and has several unique anatomic and clinical features and specific surgical requirements. Preoperative diagnosis can be made by clinical investigation and echocardiography. Excellent results can be achieved by anatomic correction.