Revised 2010 Task Force Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy: Arrhythmogenic Right Ventricular Cardiomyopathy

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 has shown that electrical abnormalities precede detectable structural changes in ARVC. Cardiovascular magnetic resonance (CMR) imaging is an ideal technique in ARVC workup because 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 chapter provides a review on diagnosis, current state-of-the-art CMR acquisition/analysis, genetics, and management of patients with ARVC.