Current knowledge regarding clinical phenotypes and outcomes of desmoplakin cardiomyopathies is largely based on published reports of small populations [5], including (1) large cohorts of individuals with ARVC [11–13,15,17,18,24], (2) other cardio-myopathy cohorts with fewer than 10 probands [25–28], and (3) single family/single DSP variant cohorts [29,30]. The gene discussed is DSP; the disease is myopathy.