Abstract
Background: Ebstein's anomaly is a rare congenital cardiac defect of the tricuspid valve (TV) leading to severe tricuspid insufficiency (Tl). Methods: In ten patients, 6 adults (39-53 years) and 4 children (5-10 years), operated between 1989 and 1995 echocardiography was performed pre and post repair and at follow-up. Patients were assessed in our Institution at two cut-off points, resulting in a mean first follow-up of 17 ±15 months and a mean second follow-up of 53 ±23 months. All patients had additional congenital cardiac defects (ASD,VSD). In all patients the TV was repaired by techniques described by Carpentier et al. with some modifications. The goal of this reparative attempt is to mobilize restricted leaflet tissue and aid coaptation through implantation of a ring. Results: Echocardiographically we were able to identify significant characteristics for the successful repair of Ebstein's anomaly. The severity of the disease is represented by the size and function of the right ventricle and the atrialized chamber, the most advanced cases exhibiting a dilated right ventricle with poor contractility. There was severe preoperative Tl ( mean grade 3.2 ± 0.3). Postoperatively Tl was significantly reduced to a mean grade of 2 ± 0.2. 60% of the patients demonstrated an improvement in the ratio of atrialized chamber to functional right ventricle. Right-ventricular function was improved, the mean score being 2.8 ± 0.1. At follow-up I and II right-ventricular function and tricuspid insufficiency was improved in most patients and all patients benefited in quality of life. Conclusions: These results suggest that surgical correction should not be delayed until severe right heart failure develops as, particularly in children, good results are achieved, improving the quality of life.
Key words
Ebstein's anomaly - Echocardiography - Tricuspid valve repair
