Ebstein-anomalie - Langzeitergebnisse nach trikuspidalklappenrekonstruktion ohne ventrikelplikatur

Ebstein's anomaly is a rare congenital heart defect in which the hinges of the septal and/or posterior leaflets are displaced downward to the right ventricle. The anterior leaflet is usually not displaced but is enlarged and sail-like and valve closure is likewise displaced downwards. Since 1988 we have operated on 37 patients with Ebstein's anomaly using a modified repair technique of the tricuspid valve. This technique restructures the valve mechanism at the level of the true tricuspid annulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. We evaluated our long-term results with regard to functional capacity (New York Heart Association functional class), tricuspid valve function, rhythm disturbances and re-operation rate. We quantified the right ventricular function by measuring flow velocity integral of the pulmonary artery (VTI_PA). All patients survived the operation. There were three hospital deaths (8%) and the late mortality was 2,7%. The mean follow-up period was 59 months (range, 6 months to 15 years). So far no re-operation has been necessary. Preoperatively, the majority of all patients were in NYHA classes III and IV (89%). After the first postoperative follow-up examination (2.9 months), 72% were in NYHA class II. Long-term follow-up examinations showed an additional improvement of 43% to NYHA class I. Echocardiographic studies demonstrated a significant improvement of tricuspid valve function. No tricuspid valve stenosis was observed. Significant improvement of VTI_PA was observed. Analysis of the postoperative deaths demonstrated that all patients were cyanotic, older than 50 years and were classified as NYHA class III or IV with a severe form of Ebstein's anomaly. We conclude that reconstruction of the tricuspid valve without ventricle plication not only achieves good functional results immediately after the operation but that follow-up examinations demonstrate stable or improved functional capacity in the long term.