LDLR and coronary artery disorder: Although cardiovascular risk in patients with FH is mainly driven by the degree of elevation of LDL-C level, the risk of CHD in FH is not solely due to elevated LDL-C level and its severity and clinical expression is even variable within a family, where all relatives carry the same LDLR gene defect.15 A family history of an early coronary event in first-degree or second-degree relatives generally puts the patient at higher risk.14 Low HDL-C level and high total:HDL-C ratio are strongly associated with a risk of CHD in FH.16