Valve replacement for Ebstein's anomaly of the tricuspid valve. Early and long-term results of eight cases.

The role of valve replacement in the treatment of Ebstein's anomaly of the tricuspid valve remains controversial. Since 1965, eight patients with Ebstein's anomaly ranging in age from seven to 22 years of age (mean 13 years) have undergone tricuspid valve replacement (TVR) in our institution. Seven were in functional class 3 or 4 (NYHA), while one was in class 2, but had an increase in cardiac size. All of these eight patients showed moderate to severe cardiomegaly (cardiothoracic ratio: 54-75 percent). The valve was placed on the tricuspid annulus in seven of the eight cases, the exception being one case in which it was placed above the coronary sinus. Seven artificial prostheses (two Starr-Edwards, one Harken, one Kay-Shilley and three Wada-Cutter) and one porcine valve (Hancock) were used. There was one operative death in an early case due to postoperative complete heart block. Follow-up (range three to 18 years) of the other seven survival patients showed three late deaths; the first a pulmonary embolus 2.6 years after TVR; the second with congestive heart failure two years following the operation; and the third with arrhythmias five years after TVR. The remaining four patients are now in functional class 1 (three cases) and class 2 (one case). Valve replacement for Ebstein's anomaly can produce good clinical improvement and provide excellent long-term results.