HMGCR and atherosclerosis: Regardless of the FH phenotype severity that generally varies between FH-causing mutations, high-intensity hydroxymethylglutaryl co-enzyme A (HMG CoA) reductase inhibitors (statins) therapy is highly recommended in asymptomatic atherosclerotic cardiovascular disease (ASCVD) if LDL-C > 4.9 mmol/L (190 mg/dL) [9] or whenever LDL-C levels remain above 1.4 mmol/L (>55 mg/dL) and less than 50% reduction from baseline are yet achieved in FH patients with ASCVD, who are considered at very high risk.