Anatomic twist to a straightforward ablation

Atrioventricular (AV) junction ablation for treatment of refractory atrial fibrillation is a well defined, standardized procedure and the simplest of commonly performed radiofrequency ablations in the field of cardiac electrophysiology. We report successful AV junction ablation using an inferior approach in a case of inferior vena cava interruption. Inability during the procedure to initially pass the ablation catheter into the right ventricle, combined with low amplitude electrograms, led to suspicion of an anatomic abnormality. This was determined to be a heterotaxy syndrome with inferior vena cava interruption and azygos continuation, draining in turn into the superior vena cava. Advancing Schwartz right 0 (SRO) sheath through the venous abnormality into the right atrium allowed adequate catheter stability to successfully induce complete AV block with radiofrequency energy.