Given the novelty of the TMEM43 mutation in the context of previously reported mutations that were primarily in genes encoding desmosomal proteins, we first sought to determine the prevalence of mutations in TMEM43 relative to five desmosomal genes, namely, DSC2, DSG2, DSP, JUP, PKP2, in 11 ARVC probands. Here, DSC2 is linked to arrhythmogenic right ventricular cardiomyopathy.