Ebstein's anomaly--experiences with surgical treatment in adulthood

INTRODUCTION: Long time results with operative treatment of Ebstein anomaly were examined. PATIENTS AND METHODS: From January 1985 to March 2001 16 patients with Ebstein anomaly were operated on. Ages ranged from 16 to 49 years at the time of operation. In 7 cases tricuspid valve repair was possible, and in 9 cases prosthetic valve was inserted. In all but one biological prosthesis has been used. In 15 cases atrial septal defect occurred as a concomitant anomaly, which was closed by direct suture in 9 cases and with patch (2 Dacron, 4 pericardium) in 6 cases. RESULTS: There was no early death (30 days postoperatively). 1 patient following tricuspid repair was reoperated on at the 9th postoperative day because of significant tricuspid insufficiency. Tricuspid valve replacement was performed with a biological prostheses. There were 3 late deaths: 2 patients (12.5%) in the first postoperative year (1 cardiac cause, 1 unknown), 1 patient died 6 years postoperatively following reoperation. There were 3 more patients requiring reoperation (total reoperation rate 28.6%) one of them a few days after the primary operation and two others 9 and 11 years following the first operation. 13 patients were recalled to control investigations. The authors could not contact 2 patients, 1 patient living abroad could not appear at our clinic. 10 patients have been investigated 6 months to 16 years after the operation. There were 9 patients in New York Heart Association class I or II. 2 patients had their own repaired valve; both had tricuspid insufficiency grade III. Both were completely active. 8 patients had previously tricuspid valve replacement and good valve function, but six of them have not been working any more. There were 5 female patients under 35 at the time of operation and 2 of them had successful pregnancies. CONCLUSIONS: Patients with Ebstein anomaly in NYHA stage III-IV. can be successfully treated surgically.