Some of these relate to well-characterized recessive conditions, for example, the cardiocutaneous Naxos and Carvajal syndromes associated with biallelic variants in desmosome genes, and skeletal myopathy diseases associated with biallelic variants in DCM genes like TTN and LMNA. In this study, we focused our analysis on sarcomeric CM genes to explore the spectrum of dominance and recessiveness in genes of predominantly dominant inheritance. This evidence concerns the gene LMNA and arrhythmogenic cardiomyopathy with wooly hair and keratoderma.