Arrhythmogenic right ventricular cardiomyopathy (ARVC): cardiovascular magnetic resonance update

Abstract

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is one of the most arrhythmogenic forms of inherited cardiomyopathy and a frequent cause of sudden death in the young. Affected individuals typically present between the second and fourth decade of life with arrhythmias coming from the right ventricle. Pathogenic mutations in genes encoding the cardiac desmosome can be found in approximately 60% of index patients, leading to our current perception of ARVC as a desmosomal disease. Although ARVC is known to preferentially affect the right ventricle, early and/or predominant left ventricular involvement is increasingly recognized. Diagnosis is made by combining multiple sources of diagnostic information as prescribed by the “Task Force” criteria. Recent research suggests that electrical abnormalities precede structural changes in ARVC. Cardiovascular Magnetic Resonance (CMR) is an ideal technique in ARVC workup, as it provides comprehensive information on cardiac morphology, function, and tissue characterization in a single investigation. Prevention of sudden cardiac death using implantable cardioverter-defibrillators is the most important management consideration. This purpose of this paper is to provide an updated review of our understanding of the genetics, diagnosis, current state-of-the-art CMR acquisition and analysis, and management of patients with ARVC.

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“…Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare heart muscle disease, characterized by a progressive myocardial dystrophy with fibro-fatty replacement (18). It is a genetically determined cardiomyopathy caused by heterozygous, or compound heterozygous mutations in genes, mostly encoding proteins of the desmosome complex (about 50 % of probands).…”