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Invited Commentary
March 2017

Quantifying Disease Progression in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Key to Advancing Therapy

Author Affiliations
  • 1Heart Research Follow-up Program, Cardiology Division, University of Rochester Medical Center, Rochester, New York
JAMA Cardiol. 2017;2(3):303-304. doi:10.1001/jamacardio.2016.5035

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a disorder characterized by progressive degeneration of cardiac myocytes with replacement by fat and fibrous tissue with inflammation.1 The subepicardium of the right ventricular wall is most commonly affected, although the left ventricle can be involved with myocardial degeneration and fibrosis localized in subepicardium and midmyocardium.1 There is a wide spectrum of presentations at the time of ARVD/C diagnosis: asymptomatic genetic carriers of the disease (usually family members of previously identified probands), patients with ventricular arrhythmias but without significant structural disease, and patients with structural disease affecting the first right ventricle and next the left ventricle as well as ventricular tachyarrhythmias and ultimately heart failure. The progressive nature of the disease is well recognized by pathologists and by clinicians caring for patients with ARVD/C; however, there are limited attempts to quantify this progression.

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