Background: The optimal management of atrial tachyarrhythmia (AT) late after Fontan operation has not yet been established.
Methods and results: Of 199 patients who were followed for more than 10 years after Fontan operation, 60 patients in whom late postoperative arrhythmias were observed were the subjects of this study. These arrhythmias were managed with anti-arrhythmic drugs. Twenty-one of 60 patients (35%) did not respond to the drugs and they needed further interventions. Fourteen catheter ablation procedures were performed in nine patients (atrial fibrillation in one patient, AT in eight patients) and the success rate was 44%. Conversion to total cavopulmonary connection (TCPC) was performed in 14 patients and Maze operation was performed at the time of Fontan conversion in 6 patients. Sinus rhythm or pacemaker rhythm was maintained in 7 of 14 (50%) patients postoperatively.
Conclusions: Although arrhythmogenic substrates accumulate and tachyarrhythmia becomes frequent over the long-term following Fontan operation, most patients with AT can be managed medically. The success rate of catheter ablation and Maze operation is low but those interventions may be indicated in patients with intractable arrhythmias.