In patients with LV-dominant substrate, CS may be confused with left-dominant arrhythmogenic cardiomyopathy with a preponderance of desmoplakin (DSP) mutations, which can be found in around 50% of patients with ARVC, as well as acute and chronic myocarditis, and other familial dilated cardiomyopathies [21,22]. Here, DSP is linked to Cowden syndrome 1.