As shown in Figure 2, based on initial scoring, 6 genes (PKP2, DSP, DSC2, DSG2, JUP, and TMEM43) had strong evidence (12–18 points) and were judged to be definitive for ARVC causation as each had replication across ARVC cohorts. This evidence concerns the gene TMEM43 and Arrhythmogenic right ventricular dysplasia.