Acquired Pulmonary Vein Isolation in a Patient with Friedreich Ataxia

The electrophysiologic nature of atrial fibrillation (AF) and related atrial arrhythmias in Friedreich ataxia has not previously been characterized. In the presented case, dense atrial scar had progressed to the point of acquired pulmonary vein (PV) isolation before the delivery of a single radiofrequency lesion. AF was induced, and ultimately organized spontaneously into a microreentrant atrial tachycardia. Other atrial tachycardias were also identified near scar border zones; these potentially served as triggers for AF in this patient, independent of the PVs. This case emphasizes the need to address non-PV substrate in some patients undergoing catheter ablation of AF.