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.