Cholesterol‐lowering treatments are indicated for patients with FH regardless of genetic test results. Early initiation of intensive LDL‐C‐lowering treatment is critical to reduce ASCVD morbidity and mortality. FH can usually be sufficiently treated with lipid‐lowering therapies, including statins, ezetimibe, bile acid sequestrants, niacin, and PCSK9 inhibitors. However, in patients 30-75 years of age with an LDL‐C level ≥ 100 mg/dL (≥ 2.6 mmol/L) on maximally tolerated statin and ezetimibe therapy, the addition of a PCSK9 inhibitor may be considered. An LDL‐C threshold of 70 mg/dL (1.8 mmol/L) is recommended before the addition of nonstatins to statin therapy. Niacin may also be beneficial for additional LDL‐C lowering in some patients with FH who are unable to achieve LDL‐C goals despite multidrug treatment regimens.
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