Pt-2 exhibited progression of LV dysfunction (LVDd/Ds: 64/51 mm, EF: 43%, FS: 19%) at 52 years of age compared with the previously reported natural history, suggesting that interventricular plate dysfunction combined with the DSG2 p.Arg119Ter variant might contribute to the pathogenesis of DCM. The gene discussed is DSG2; the disease is familial dilated cardiomyopathy.