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

Long time results with operative treatment of Ebstein anomaly were examined.

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.

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.

Patients with Ebstein anomaly in NYHA stage III-IV. can be successfully treated surgically.