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

Arrhythmias remain an unsolved problem in Ebstein's anomaly. The aim of this study was to investigate the evolution of arrhythmias after surgical repair.

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.

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.

Arrhythmia is not totally abolished after operation. However, patients with Ebstein's anomaly and arrhythmia show substantial improvement after conservative surgical intervention.