Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management

Main Article Content

Rachel Bastiaenen
Marc W. Deyell
Andrew D. Krahn *
(*) Corresponding Author:
Andrew D. Krahn | akrahn@mail.ubc.ca

Abstract

Our understanding of arrhythmogenic right ventricular cardiomyopathy (ARVC) has advanced considerably over the past 30- 40 years. This is an inherited cardiomyopathy with complicated genetic inheritance and variable penetrance. Desmosomal dysfunction underlies most cases, and appreciating this pathophysiology has contributed to patient management, particularly with respect to exercise restriction to reduce disease progression. The diagnosis is made according to a series of Task Force Criteria, and subsequent management is guided by expert consensus in the absence of comparative data. ARVC is associated with sudden cardiac death (SCD), particularly in young athletic individuals who unknowingly harbour the condition. Risk stratification is important to guide implantable cardioverter- defibrillator use and reduce SCD. Residual gaps in our understanding, particularly surrounding incomplete penetrance, the underlying pathophysiology and risk stratification, are being targeted by collaborative efforts, large registries, prospective studies and translational research.

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Author Biographies

Rachel Bastiaenen, Heart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, BC

Consultant Cardiologist

Marc W. Deyell, Heart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, BC

Consultant Cardiologist

Andrew D. Krahn, Heart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, BC

Professor of Cardiology and Consultant Cardiologist