The aim of this study was to compare aortic valve calcium score (AVCS) between patients with severe hypercholesterolemia and DNA-based diagnosis of familial hypercholesterolemia with confirmed LDLR mutation (LDLR-M group) versus patients with non-familial severe hypercholesterolemia with no LDLR alteration (LDLR-WT group). Here, LDLR is linked to familial hypercholesterolemia.