Surgical treatment of Ebstein's anomaly. Early and late results in seven consecutive cases

The surgical treatment of Ebstein's anomaly is discussed on the basis of our experience with 7 patients who underwent operation at this clinic during the last ten years. The age of the patients (2 males, 5 females) ranged from 17 to 47 years (mean 27 years) at the time of operation. Two patients were in functional class II and five in classes III or IV (N.Y.H.A.). The Wolff-Parkinson-White syndrome was concomitant in 2/7 cases and patent foramen ovale or atrial septal defect (ASD) with right-to-left shunt was present in 6/7 cases. The valvular anomaly was typical in 5 patients and stenosis of displaced tricuspid leaflets into right ventricular outflow region was detected in two patients. Tricuspid valve replacement (TVR) with disc valve prosthesis was performed in 5 patients (Björk-Shiley model in 4 cases, Kay-Shiley model in one) and tricuspid valve commissurotomy in two patients. All six patients with interatrial communication underwent closure of this communication simultaneously. One patient had concomitant epicardial mapping and attempted division of the anomalous conduction pathways was unsuccessful. This patient died on the 14th postoperative day from ventricular fibrillation. No other early or late mortality was recorded. Life-long anticoagulation therapy was introduced in all 5 patients with TVR. Thrombotic malfunction of the prosthetic valves necessitated re-operation and new prosthetic valve replacement in two patients 15 and 16 months after the first TVR. One of these patients had recurrent prosthetic thrombosis twice which was successfully treated with streptokinase. The other patient's prosthesis is functioning well 18 months after re-operation. All 6 long-term survivors were improved and remained in good condition, in functional class I, 1, 3, 3, 8, 9, and 10 years, respectively, after operation.