In all of them, variable signs of hypertrophic cardiomyopathy were found.<h4>Conclusions</h4>Our findings report the presence of a homozygous variant in a sarcomeric gene (MYH7) which gave rise to early HCM, whereas the variant in a heterozygous state was associated to much milder cardiac phenotypes in the affected relatives. The gene discussed is MYH7; the disease is hypertrophic cardiomyopathy.