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Arrhythmic outcome of arrhythmogenic right ventricular cardiomyopathy patients without implantable defibrillators

Weijia Wang1, Julia Cadrin-Tourigny1, Aditya Bhonsale1

  • 1Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, MD, USA.

Journal of Cardiovascular Electrophysiology
|June 13, 2018
PubMed

Insights

Arrhythmogenic right ventricular cardiomyopathy (ARVC) patients without implantable cardioverter-defibrillators (ICDs) face a significant risk of malignant arrhythmias and sudden cardiac death. Early identification of risk factors is crucial for timely intervention in these high-risk individuals.

Area of Science:

  • Cardiology
  • Electrophysiology
  • Genetics

Background:

  • Implantable cardioverter-defibrillators (ICDs) are a key therapy for arrhythmogenic right ventricular cardiomyopathy (ARVC) patients at high risk of sudden cardiac death.
  • Concerns exist that ICD implantation may inflate the observed rate of malignant arrhythmias in ARVC.

Purpose of the Study:

  • To evaluate the arrhythmic course of ARVC patients who did not receive ICDs after meeting diagnostic criteria.
  • To identify predictors of malignant ventricular arrhythmias in ARVC patients.

Main Methods:

  • A cohort of 131 definite ARVC patients was analyzed.
  • Patients were either without ICDs or received an ICD >6 months after fulfilling diagnostic criteria.
  • The primary outcome was a composite of cardiac arrest and sustained ventricular tachyarrhythmias.

Main Results:

  • Over a median of 8 years, 29% of patients without ICDs experienced primary outcomes, including cardiac arrests and sustained ventricular arrhythmias.
  • One-year and 5-year event-free survival rates were 92% and 72%, respectively.
  • Spontaneous sustained ventricular arrhythmias, syncope, male sex, proband status, and electrophysiology study inducibility predicted adverse outcomes.

Conclusions:

  • A significant risk of malignant arrhythmias persists in ARVC patients even without ICD implantation.
  • These findings underscore the importance of risk stratification and timely intervention in ARVC management.
Abstract

Keywords:
arrhythmogenic right ventricular cardiomyopathyimplantable defibrillatorsudden deathventricular tachycardia

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