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.