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

Bastiaenen, R; Deyell, MW; Krahn, AD (2017) Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management. CARDIOGENETICS, 7 (1). pp. 37-45. ISSN 2035-8253 https://doi.org/10.4081/cardiogenetics.2017.6995
SGUL Authors: Bastiaenen, Rachel Marie

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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.

Item Type: Article
Additional Information: This work is licensed under a Creative Commons Attribution NonCommercial 4.0 License (CC BY-NC 4.0). ©Copyright R. Bastiaenen et al., 2017 Licensee PAGEPress, Italy Cardiogenetics 2017; 7:6995 doi:10.4081/cardiogenetics.2017.6995
Keywords: Arrhythmogenic right ventricular cardiomyopathy, sudden cardiac death, ventricular arrhythmias
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Journal or Publication Title: CARDIOGENETICS
ISSN: 2035-8253
Dates:
DateEvent
22 December 2017Published
12 December 2017Accepted
Web of Science ID: WOS:000444918400002
URI: http://openaccess.sgul.ac.uk/id/eprint/110436
Publisher's version: https://doi.org/10.4081/cardiogenetics.2017.6995

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