[Ebstein's anomaly: valvular replacement in pediatric patients]

The surgical treatment of Ebstein's anomaly is still controversial. Therefore we have retrospectively studied the results of tricuspid valve replacement (TVR) performed for this anomaly at Sainte Justine Hospital. From October 1977 to December 1997, 9 patients with Ebstein's anomaly, aged from 31 to 248 months (mean 176 +/- 66), have undergone TVR. Eight children were in functional class III or IV (NYHA), while one was in class II. Seven patients underwent plication of the atrialized right ventricular segment. Eight bioprostheses (ranging in diameter from 31 to 35 mm) and one mechanical prosthesis (21 mm) were used. The valve was implanted on the tricuspid annulus in six cases. There was no operative death, nor postoperative complete heart block. Follow-up ranged from 11 to 264 months (mean 91 +/- 84). One late death occurred unrelated to surgery. The probability of 20 years survival is 88%. One patient required a second TVR 162 months after the first surgery because of bioprosthesis failure. Seven of the surviving patients are in functional class I, while one patient is in class II. This experience suggests that TVR with bioprosthesis is a good therapeutical option for children with Ebstein's anomaly since the operative risk is low, the functional status improved in all patients and the durability of bioprosthesis in tricuspid position has been good.