This evidence-based reevaluation of published ARVC genes by experts in the field shows that only a small number of genes (PKP2, DSP, DSG2, DSC2, JUP, TMEM43, PLN, and DES) are definitively or moderately associated with ARVC and these genes account for the overwhelming majority of P/LP variants in patients with ARVC. Here, TMEM43 is linked to Arrhythmogenic right ventricular dysplasia.