This concept is now challenged, first and foremost through the recognition of LV-dominant ACM subforms involving DSP or non-desmosomal variants, as this article further explores, but also because studies report LV LGE in up to 43% and LV dysfunction in 16% of PKP2 variant carriers—the genotype most associated with “classical” ARVC—even without severe RV disease. This evidence concerns the gene DSP and arrhythmogenic right ventricular cardiomyopathy.