PKP2 and arrhythmogenic right ventricular cardiomyopathy: In contemporary cohorts, up to two-thirds of patients with definite ARVC per the 2010 TFC have a pathogenic or likely pathogenic (P/LP) variant in one of 5 genes encoding cardiac desmosome proteins (PKP2, DSP, DSG2, DSP, and JUP) (14, 29).