SURGICAL-TREATMENT OF ATRIOVENTRICULAR ATRESIA COMBINED WITH WOLFF-PARKINSON-WHITE SYNDROME

Background: Tachyarrhythmia has been thought to be an absolute contrai ndication for the Fontan operation. Patients and methods: Three patien ts, 22, 9, and 11 years of age, diagnosed as having atrioventricular a tresia combined with the Wolff-Parkinson-White syndrome underwent surg ical treatment. Each had drug-resistant atrioventricular tachycardia t hat 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 us ing an epicardial approach in two patients and an endocardial approach in one. Simultaneously, the Fontan operation was performed with atrio ventricular 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 Fonta n operation. Results: There was no early death or other fatal complica tion, and the hemodynamic results were excellent. During the mean foll ow-up period of 68 months (range, 5 to 127 months), there has been no late death or recurrence of tachyarrhythmia. Conclusion: Tachyarrhythm ias caused by ACPs are not contraindications for the Fontan operation. Concomitant surgery is advocated, as excellent short- and longterm re sults may be expected in these patients.