Arrhythmogenic Cardiomyopathy: Evolving Diagnostic Criteria and Insight from Cardiac Magnetic Resonance Imaging

Abstract

Arrhythmogenic cardiomyopathy (ACM) is an umbrella term encompassing a wide variety of overlapping hereditary and nonhereditary disorders that can result in malignant ventricular arrhythmias and sudden cardiac death. Cardiac MRI plays a critical role in accurate diagnosis of various ACM entities and is increasingly showing promise in risk stratification that can further guide management particularly in decisions regarding use of implantable cardioverter defibrillator. Genotyping plays an important role in cascade testing but challenges remain due to incomplete penetrance and wide phenotypic variability of ACM as well as the presence of gene-elusive cases.

Keywords: Arrhythmogenic cardiomyopathy (ACM); Arrhythmogenic right ventricular cardiomyopathy (ARVC); Cardiac magnetic resonance imaging (CMR, MRI); Cardiac sarcoidosis; Dilated cardiomyopathy; Genotyping; Hereditary cardiomyopathy; Sudden cardiac death (SCD).