Surgical treatment in Ebstein's malformation.

Between January, 1967, and July, 1981, 24 patients with Ebstein's malformation underwent surgical repair. Sixteen had tricuspid valve replacement; 8 did not. All interatrial communications were closed. Two patients had plication of atrialized ventricle. Twenty had dysrhythmias; these were surgically treated in 4. Four patients (17%), each with valve replacement, died in the hospital (70% confidence limits, 9-28%); 3 of these were among the 6 patients who were preoperatively in New York Heart Association Functional Class IV. There were 3 late deaths, 1 from noncardiac causes and 2 from persistent tricuspid regurgitation in patients without valve replacement. No late deaths or valve-related complications occurred in the valve replacement group. Dysrhythmias remain a problem, although most patients are symptomatically improved after operation. This experience suggests that good early and late results are obtained with replacement of incompetent tricuspid valves if this is done before advanced symptomatic deterioration, that plication rarely is necessary, and that rhythm disturbances should be recognized and appropriately managed.