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Introduction <p>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.</p> Methods and results <p>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.</p> Conclusion <p>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.</p> Graphical Abstract <p></p>

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

  • 期刊论文

  • 发表日期：

    2025年1月16日

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  • Laura Valverde Soria,
  • Pablo J. Sanchez-Millan,
  • José Antonio Fernandez-Sanchez,
  • Rosa Macías-Ruiz,
  • Juan Jimenez-Jaimez,
  • Luis Tercedor

摘要

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.

Graphical Abstract