PKP2 mutations associate with most cases of gene-positive familial arrhythmogenic right ventricular cardiomyopathy (ARVC) (Basso et al., 2009; Groeneweg et al., 2015; Philips and Cheng, 2016), a disease characterized by fibrofatty infiltration of right ventricular (RV) predominance, ventricular arrhythmias, and high propensity for sudden death in the young. The gene discussed is PKP2; the disease is arrhythmogenic right ventricular cardiomyopathy.