Catheter ablation of multiple, surgically created, atrioventricular connections following Fontan-Björk procedure

An increased incidence of Wolff-Parkinson-White (WPW) syndrome with tricuspid atresia has been reported. Although atrioventricular accessory pathways may develop across suture lines after the Fontan-Björk procedure, the presence of multiple acquired accessory pathways has only been described rarely. We report on a case of a female with tricuspid atresia who underwent the Fontan operation at 5 years of age. One year later, she developed a WPW pattern. Narrow complex tachycardias started at the age of 18. An electrophysiological study revealed the presence of three accessory pathway connections at the surgical anastomosis level. All of them were successfully ablated and there were no recurrences.