Cardiac arrhythmia in patients undergoing surgical repair of Ebstein's anomaly

BACKGROUND: Arrhythmias remain an unsolved problem in Ebstein's anomaly. The aim of this study was to investigate the evolution of arrhythmias after surgical repair. METHODS: Forty-five patients with Ebstein's anomaly and arrhythmias were studied. Mean age was 33 +/- 15 years. Twenty-four patients (53%) had paroxysmal supraventricular tachycardia, 12 (27%) had atrial fibrillation or flutter, 8 (18%) had ventricular preexcitation (Wolff-Parkinson-White syndrome), and 1 (2%) had a nonsustained ventricular tachycardia. Surgical technique included detachment of the tricuspid anterior leaflet and suture on the atrioventricular annulus associated with right ventricular longitudinal plication. RESULTS: There were four hospital deaths (9%). A pacemaker was implanted early after operation in 5 patients (11%). During a mean follow-up of 57 +/- 50 months (range, 4 to 226 months), there were six additional deaths, three of which were sudden. Two patients were lost to follow-up. Of the 33 surviving patients, 8 (24%) continued to have symptomatic arrhythmias, and 15 (45%) were in permanent sinus rhythm. Of the 24 patients with preoperative paroxysmal supraventricular tachycardia and the 12 with atrial fibrillation or flutter preoperatively, 9 and 2 of the survivors, respectively, have had no further episodes of arrhythmia. The incidence of arrhythmia with or without symptoms was reduced to 39% (13/33) of the surviving patients. CONCLUSIONS: Arrhythmia is not totally abolished after operation. However, patients with Ebstein's anomaly and arrhythmia show substantial improvement after conservative surgical intervention.