Successful ablation of Purkinje-related ventricular ectopy leading to ventricular fibrillation in Emery-Dreifuss dilated cardiomyopathy

Introduction: Mutations in EMD are related to an increased risk of ventricular arrhythmias and sudden cardiac death. There is a lack of data concerning ventricular arrhythmia ablation in Emery-Dreifuss patients.

Methods and results: We present a case of successful ablation of a short-coupled ventricular ectopy (VE) triggering recurrent ventricular fibrillation (VF) episodes in a EMD patient with an intraseptal substrate. Our approach combined substrate ablation with ICD-guided pacemapping.

Conclusion: VF ablation of Purkinje triggers may be an alternative treatment for patients with dilated cardiomyopathy and recurrent ICD shocks due to VF induced by monomorphic VE.