PKP2 and Arrhythmogenic right ventricular dysplasia: Recently, a panel of international experts conducted a rigorous reappraisal of 26 ARVC genes reported in the literature using the semi-quantitative ClinGen framework and found only eight genes had definitive (PKP2, DSP, DSG2, DSC2, JUP, TMEM43) or moderate (PLN, DES) evidence for causing ARVC (Figure 4) (35).