Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a cardiac disease characterized by fibrofatty replacement of the cardiac myocytes. Patients with ARVD/C frequently present with ventricular tachycardia and many are thought to have sudden cardiac death as the initial manifestation of the disease. Over the past decade, our understanding of the disease has increased dramatically along with delineation of the genetic basis of ARVD/C and characteristic features on diagnostic imaging. The management of patients with ARVD/C remains a challenge, especially in the light of incomplete genotype-phenotype characterization, and poor predictors of sudden cardiac death. In this article, we review the pathologic and genetic basis of ARVD/C, focusing on the diagnostic features and therapeutic challenges emerging with our enhanced knowledge of this rare disease.
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