• N Nagdyman, P Ewert, B Stiller, E Riesenkampff, T Fleck, P E Lange, and R Hetzer.
• Abt. für Angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353 Berlin, Germany. nagdyman@dhzb.de
• Z Kardiol. 2003 Sep 1; 92 (9): 730-4.

AbstractEbstein'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 22 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 (VTIPA). All patients survived the operation. There were two hospital deaths (9%) and the late mortality was 4.5%. The mean followup period was 9 years (range, 1.5 to 13 years) for 19 patients. So far no re-operation has been necessary. Preoperatively, the majority of all patients were in NYHA classes III and IV (79%). After the first postoperative follow-up examination (2.9 months), 17 patients were in NYHA class II. Long-term follow-up examinations showed an additional improvement of 11 patients 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 in NYHA class III or IV and had a cardiothoracic ratio of 0.65 or more. A severe reduction in functional capacity seems to be an additional risk factor for mortality beside a cardiothoracic ratio greater than 0.65. 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. We postulate that incorporation of the atrialized chamber into the right ventricle may contribute to right ventricular contraction and thereby account for the improved functional capacity of the patients.

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