Modified repair in patients with Ebstein's anomaly

Background and aim of the study: Since 1988, a modified repair technique has been used at the authors' institution to treat patients with Ebstein's anomaly. This technique restructures the valve mechanism at the level of the true tricuspid annulus by using the most mobile leaflet for valve closure, without plication of the atrialized chamber. A total of 19 patients had additional attachment of the anterior right ventricular wall to the interventricular septum (Sebening's stitch) and reconstruction of the tricuspid valve as a double-orifice valve. The long-term results of the study are presented. Methods: Between 1988 and 2008, tricuspid valve repair was performed in 50 patients with Ebstein's anomaly (33 females, 17 males; median age 22 years; range: 0.6 to 60 years), at the authors' institution. The median follow up was 68 months (range: 5 to 238) months. Details of the survival rate, reoperations, NYHA class, maximal VO₂, right ventricular function (velocity-time integral pulmonary artery (VTI-PA)), and tricuspid valve insufficiency were documented. Results: No patient deaths occurred during surgery; the early mortality was 7.1%, and late mortality 2.4%. Those patients who died were all aged >50 years, and in NYHA class III or IV. No additional patient deaths have occurred since 2004. Four reoperations were necessary. Both, the NYHA class and tricuspid valve insufficiency were improved significantly (from 3.1 to 1.8; p <0.001 and from 3.2 to 1.9; p <0.001, respectively). The VTI-PA was increased significantly, with a stable heart rate (p = 0.01). No aneurysm of the right ventricle was observed. Conclusion: The long-term follow up demonstrated good clinical results in tricuspid repair, without plication of the right ventricle, even in cases where tricuspid valve replacement was discussed. Modifications seemed to support these results. Surgery in older patients with a progressive NYHA class seemed to carry a higher operative mortality.