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Arrhythmogenic right ventricular cardiomyopathy

Sex and Cardiac Electrophysiology, Page: 375-388
2020
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Book Chapter Description

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary heart disease characterized by fibrofatty replacement of the ventricular myocardium, frequent ventricular arrhythmias, a substantial risk of sudden cardiac death, and progressive ventricular dysfunction. ARVC is associated with pathogenic variants (e.g., mutations) primarily in genes encoding the cardiac desmosome. Pathogenic variants can be identified in up to two-thirds of patients with ARVC. ARVC is characterized by incomplete penetrance and has a highly variable clinical course among patients once they are diagnosed. Identifying demographic, clinical, and environmental factors associated with patient outcomes has been an area of focused research over the past decade. Despite being a genetic disease, ARVC is more prevalent among males in most cohorts. Females have lower penetrance, later ages of onset, and, in some cohorts, lower risk of ventricular arrhythmias but no reduced risk of heart failure. This chapter describes insights gained from both natural history studies and experimental research to discuss potential explanations for these associations of sex with patient outcomes. We also discuss the literature on pregnancy in ARVC.

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