Pulmonary Vein–Triggered Atrial Fibrillation in Unrepaired Double-Outlet Right Ventricle With Pulmonary Atresia

BACKGROUND: Direct electrophysiological evidence that the pulmonary vein (PV) triggers atrial fibrillation (AF) in patients with complex adult congenital heart disease (ACHD) is lacking; nevertheless, PV isolation (PVI) has been empirically applied. CASE SUMMARY: A 49-year-old man with an unrepaired double-outlet right ventricle with pulmonary atresia presented with AF and worsening heart failure. Radiofrequency catheter ablation was performed. Spontaneous ectopy originating from the left superior PV reproducibly triggered AF. Ipsilateral PVI achieved clinical success without complications, although right PV could not be isolated because of anatomical difficulties. The patient has remained free of arrhythmias or cardiovascular events for 4.5 years. DISCUSSION: Electrophysiological confirmation of AF triggers in a patient with complex ACHD is challenging because of limited procedural tolerance, making this single case a valuable piece of evidence. TAKE-HOME MESSAGE: PV-triggered AF was demonstrated in a patient with complex ACHD, which provides rare electrophysiological evidence supporting the empirical strategy of PVI.