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Review
. 2022 Apr;8(4):533-553.
doi: 10.1016/j.jacep.2021.12.002.

Arrhythmogenic Right Ventricular Cardiomyopathy

Andrew D Krahn  1 , Arthur A M Wilde  2 , Hugh Calkins  3 , Andre La Gerche  4 , Julia Cadrin-Tourigny  5 , Jason D Roberts  6 , Hui-Chen Han  7
Affiliations
Free article
Review

Arrhythmogenic Right Ventricular Cardiomyopathy

Andrew D Krahn et al. JACC Clin Electrophysiol. 2022 Apr.
Free article

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) encompasses a group of conditions characterized by right ventricular fibrofatty infiltration, with a predominant arrhythmic presentation. First described in the late 1970s and early 1980s, it is now frequently recognized to have biventricular involvement. The prevalence is ∼1:2,000 to 1:5,000, depending on geographic location, and it has a slight male predominance. The diagnosis of ARVC is determined on the basis of fulfillment of task force criteria incorporating electrophysiological parameters, cardiac imaging findings, genetic factors, and histopathologic features. Risk stratification of patients with ARVC aims to identify those who are at increased risk of sudden cardiac death or sustained ventricular tachycardia. Factors including age, sex, electrophysiological features, and cardiac imaging investigations all contribute to risk stratification. The current management of ARVC includes exercise restriction, β-blocker therapy, consideration for implantable cardioverter-defibrillator insertion, and catheter ablation. This review summarizes our current understanding of ARVC and provides clinicians with a practical approach to diagnosis and management.

Keywords: cardiomyopathy; inherited; sudden death; ventricular arrhythmia.

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Conflict of interest statement

Funding Support and Author Disclosures The study was supported by the Heart in Rhythm Organization (Dr Krahn, principal investigator), which receives support from the Canadian Institute of Health Research (RN380020-406814). Dr Krahn has received support from the Sauder Family and Heart and Stroke Foundation Chair in Cardiology (Vancouver, British Columbia, Canada), the Paul Brunes Chair in Heart Rhythm Disorders (Vancouver, British Columbia, Canada), and the Paul Albrechtson Foundation (Winnipeg, Manitoba, Canada). Dr Wilde has received support from the Netherlands CardioVascular Research Initiative, the Dutch Heart Foundation, the Dutch Federation of University Medical Centres, the Netherlands Organisation for Health Research and Development, and the Royal Netherlands Academy of Sciences (PREDICT2). Dr Calkins has received grant support from the Fondation Leducq; and has received research support from Boston Scientific. Dr Han is supported by a Robert and Elizabeth Albert Travel Grant from the RACP Foundation, Australia. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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