Valve-preserving treatment of Ebstein's anomaly: Perioperative and follow-up results

Background: Ebstein's anomaly is a rare congenital cardiac defect of the tr icuspid valve (TV) leading to severe tricuspid insufficiency (TI). Methods: In ten patients, 6 adults (39-53 years) and 4 children (5-10 years), opera ted between 1989 and 1995 echocardiography was performed pre and post repai r and at follow-up. Patients were assessed in our institution at two cut-of f points, resulting in a mean first follow-up of 17 +/- 15 months and a mea n second follow-up of 53 +/- 23 months. All patients had additional congeni tal cardiac defects (ASD,VSD). In all patients the TV was repaired by techn iques described by Carpentier et a[. with some modifications. The goal of t his reparative attempt is to mobilize restricted leaflet tissue and aid coa ptation through implantation of a ring. Results: Echocardiographically we w ere able to identify significant characteristics for the successful repair of Ebstein's anomaly. The severity of the disease is represented by the siz e 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 TI ( mean grade 3.2 +/- 0.3). Postoperative ly TI was significantly reduced to a mean grade of 2 +/- 0.2. 60% of the pa tients demonstrated an improvement in the ratio of atrialized chamber to fu nctional 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 surgi cal correction should not be delayed until severe right heart failure devel ops as, particularly in children, good results are achieved, improving the quality of life.