Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease of myocardial tissue whose hallmarks include fatty and fibrofatty replacement of normal myocardial tissue. Clinically, ARVC is typified by ventricular arrhythmias, progressive systolic dysfunction, syncope, congestive heart failure, and sudden death. At an ultrastructural level, ARVC appears to be a disease of the myocyte intercalated disc, which is the junction between adjacent myocardial cells. The intercalated disc contains mechanical cell-to-cell attachments (i.e., the adherens junction and desmosome) as well as ion pores (i.e., gap junctions), which facilitate propagation of the action potential.
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