Morbus Ebstein: Wann ist die operation indiziert?

Ebstein's anomaly is a complex malformation of the tricuspid valve where the hinges of the septal and/or posterior leaflets are displaced downward into the right ventricle. The leaflets show variable deformations. In general, the anterior leaflet is enlarged. For those patients who have only mild symptoms, medical management is recommended, but operative treatment is indicated if progressive deterioration is evident. The timing of the surgical intervention is still a matter of controversy, especially since the results of surgical treatment were substantially improved by further development of repair techniques. Between 1974 and August, 1997, 69 patients with Ebstein's anomaly underwent surgical repair. In 65 patients (94,2%) tricuspid valvuloplasty was feasible, mainly by creating a monocusp valve with the 'single-stitch technique', developed in our clinic by F. Sebening. Four patients required primary valve replacement with a bioprosthesis. Ten reoperations (14.5%) were necessary: 6 repeat valvuloplasties, 4 valve replacements. There were 2 hospital deaths (2.9%), the late mortality was 8.7% (6 patients). Since 1992, our valvuloplasties have been evaluated by intra-operative transesophageal echocardiography (TEE). Follow-up was obtained in 58 patients (95%) over a period of 4 months to 21.0 years (median 5.3 years, mean 7.6 years). The actuarial survival rate (Kaplan-Meier) was 96.5% ± 2.4% at 1 year and 83.3% ± 5.6% at 21 years. Pre-operatively, the majority of patients were in New York Heart Association functional Class III. At follow-up evaluation, nearly all patients showed substantial improvement of their pre-operative status, 94.8% were in NYHA Class I or II. Doppler echocardiographic studies demonstrated good tricuspid valve function in most patients. The valvuloplasty developed in our hospital using a single-stitch technique is a rewarding operation, which yields good long-term results. An analysis of the postoperative deaths revealed that all patients but one suffered from endstage cardiac disease and had a cardio-thoracic ratio greater than 0.65. This supports the importance of surgical intervention in time. In our opinion, operation is even indicated for those patients in functional Class II who reveal clinical deterioration.