Successful Ablation of a Left-Sided Accessory Pathway in a Patient with Coronary Sinus Atresia and Arteriovenous Fistula: Clinical and Developmental Insights

BACKGROUND AND OBJECTIVES: While radiofrequency ablation catheter ablation of accessory pathways is generally safe and effective, anatomic variants can cause considerable challenges in effecting cure. Our objective was to use an unusual case where coronary sinus was absent and arterial venous fistula was present and a left-sided pathway required mapping and ablation to develop a framework to approach difficult cases. METHOD: A detailed literature search and review of contemporary cardiac embryology was undertaken to attempt and to explain a common developmental anomaly. Adjunctive approaches during the ablation procedure, including intracardiac ultrasound, were used to guide mapping and ablation despite the lack of coronary sinus access. RESULTS: The accessory pathway was successfully ablated using a transseptal approach and intracardiac ultrasound guided mapping of the mitral annulus. A potential common mechanism to explain the apparently disparate anatomic variants in this patient was formulated. CONCLUSIONS: Cardiac conduction development is complex and accessory pathway conduction may occur in the setting of arteriovenous anomalies thus providing insights as to the cause of WPW syndrome. Successful mapping and targeted ablation of left-sided pathways may be accomplished even when coronary sinus access is not possible.