In a large-scale sequencing study by Khera et al., only approximately 2% of individuals with severe hypercholesterolemia from the general population, defined as untreated LDL-C ≥ 190 mg/dL, showed a pathogenic variant in an autosomal dominant FH gene.7 Conversely, in another study using next-generation sequencing on referred patients with severe hypercholesterolemia, Wang et al. found a monogenic FH-causing variant in 47.3%; this percentage increased to 53.7% when analysis of copy number variations were included.20 Here, FH is linked to familial hyperaldosteronism.