Arrhythmogenic Right Ventricular Cardiomyopathy

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 in...

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Published in:	JACC. Clinical electrophysiology Vol. 8; no. 4; p. 533
Main Authors:	Krahn, Andrew D, Wilde, Arthur A M, Calkins, Hugh, La Gerche, Andre, Cadrin-Tourigny, Julia, Roberts, Jason D, Han, Hui-Chen
Format:	Journal Article
Language:	English
Published:	United States 01.04.2022
Subjects:	Arrhythmogenic Right Ventricular Dysplasia - diagnosis Arrhythmogenic Right Ventricular Dysplasia - genetics Arrhythmogenic Right Ventricular Dysplasia - therapy Catheter Ablation - adverse effects Death, Sudden, Cardiac - etiology Death, Sudden, Cardiac - prevention & control Defibrillators, Implantable - adverse effects Female Humans Male Tachycardia, Ventricular - surgery ventricular arrhythmia sudden death cardiomyopathy inherited
ISSN:	2405-5018, 2405-5018
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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.
AbstractList	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.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. 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.
Author	Wilde, Arthur A M Calkins, Hugh La Gerche, Andre Krahn, Andrew D Cadrin-Tourigny, Julia Han, Hui-Chen Roberts, Jason D
Author_xml	– sequence: 1 givenname: Andrew D surname: Krahn fullname: Krahn, Andrew D email: akrahn@mail.ubc.ca organization: Center for Cardiovascular Innovation, Heart Rhythm Services, Division of Cardiology, University of British Columbia, Vancouver, British Columbia, Canada. Electronic address: akrahn@mail.ubc.ca – sequence: 2 givenname: Arthur A M surname: Wilde fullname: Wilde, Arthur A M organization: Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam University Medical Centers, Amsterdam, the Netherlands; European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARD-Heart), Academic University Medical Center, Amsterdam, the Netherlands – sequence: 3 givenname: Hugh surname: Calkins fullname: Calkins, Hugh organization: Department of Medicine, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA – sequence: 4 givenname: Andre surname: La Gerche fullname: La Gerche, Andre organization: Clinical Research Domain, Baker Heart and Diabetes Institute, Melbourne, Victoria, Australia – sequence: 5 givenname: Julia surname: Cadrin-Tourigny fullname: Cadrin-Tourigny, Julia organization: Cardiovascular Genetics Center, Montréal Heart Institute, Faculty of Medicine, University of Montréal, Montréal, Québec, Canada – sequence: 6 givenname: Jason D surname: Roberts fullname: Roberts, Jason D organization: Population Health Research Institute, McMaster University, and Hamilton Health Sciences, Hamilton, Ontario, Canada – sequence: 7 givenname: Hui-Chen surname: Han fullname: Han, Hui-Chen organization: Center for Cardiovascular Innovation, Heart Rhythm Services, Division of Cardiology, University of British Columbia, Vancouver, British Columbia, Canada; Victorian Heart Institute, Monash University, Clayton, Victoria, Australia
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SubjectTerms	Arrhythmogenic Right Ventricular Dysplasia - diagnosis Arrhythmogenic Right Ventricular Dysplasia - genetics Arrhythmogenic Right Ventricular Dysplasia - therapy Catheter Ablation - adverse effects Death, Sudden, Cardiac - etiology Death, Sudden, Cardiac - prevention & control Defibrillators, Implantable - adverse effects Female Humans Male Tachycardia, Ventricular - surgery
Title	Arrhythmogenic Right Ventricular Cardiomyopathy
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