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. The gene discussed is PKP2; the disease is Arrhythmogenic right ventricular dysplasia.