The principal causes of FH are mutations in LDLR, followed by APOB and PCSK9. Work by our group and others suggest that approximately 1 in 115 individuals carry a pathogenic mutation in LDLR. Using sequencing, we evaluated four Mendelian LDL-C genes—LDLR, ABCG5, APOB, and PCSK—for association with LDL and early-onset CHD risk in the population. Here, PCSK9 is linked to familial hyperaldosteronism.