Radiofrequency Ablation of Post-Incisional Atrial Flutter and High-Output Heart Failure in a Patient with Interrupted Inferior Vena Cava and Hereditary Hemorrhagic Telangiectasia

A 61-year-old female with a history of secundum atrial septal defect repair and hereditary hemorrhagic telangiectasia presented with epistaxis. She was found to have atypical atrial flutter with 2:1 atrioventricular conduction. Radiofrequency ablation was planned, but inferior vena cava interruption precluded right atrial (RA) access. The RA was then accessed through both subclavian veins, and activation mapping revealed a dense atriotomy scar in the posterolateral inferior RA. Wavefront propagation proceeded caudally through an area of slow conduction confined by the atriotomy scar. Atypical atrial flutter terminated during a second radio-frequency application to an isthmus confined by 2 regions of dense scar. The arrhythmia did not recur, although the patient later experienced typical atrial flutter and atrial fibrillation. High-output heart failure due to systemic arteriovenous shunt was confirmed by cardiac catheterization and improved markedly with bevacizumab therapy.