PKP2 and arrhythmogenic right ventricular cardiomyopathy: 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.