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). This evidence concerns the gene PLN and Arrhythmogenic right ventricular dysplasia.