Surgical Treatment of Atrioventricular Atresia Combined With Wolff-Parkinson-White Syndrome

Abstract

Tachyarrhythmia has been thought to be an absolute contraindication for the Fontan operation. Three patients, 22, 9, and 11 years of age, diagnosed as having atrioventricular atresia combined with the Wolff-Parkinson-White syndrome underwent surgical treatment. Each had drug-resistant atrioventricular tachycardia that required direct cardioversion. Two patients with tricuspid atresia had an intermittent right-sided accessory pathway (ACP), and one with mitral atresia had a concealed left-sided ACP. The ACP was divided using an epicardial approach in two patients and an endocardial approach in one. Simultaneously, the Fontan operation was performed with atrioventricular connection (modified Fontan operation) in one patient, and a total cavopulmonary connection performed in another patient. In the remaining patient, ACP division was performed 3 years after the Fontan operation. There was no early death or other fatal complication, and the hemodynamic results were excellent. During the mean follow-up period of 68 months (range, 5 to 127 months), there has been no late death or recurrence of tachyarrhythmia. Tachyarrhythmias caused by ACPs are not contraindications for the Fontan operation. Concomitant surgery is advocated, as excellent short- and long-term results may be expected in these patients.