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Repatha (evolocumab) — Blue Cross Blue Shield of Oklahoma

heterozygous familial hypercholesterolemia (HeFH)

Preferred products

  • Repatha (evolocumab)

Initial criteria

  • ONE of the following:
  • A. Diagnosis of homozygous familial hypercholesterolemia (HoFH) AND ALL of the following:
  • 1. Diagnosis of HoFH confirmed by ONE of:
  • A. Genetic confirmation of bi-allelic pathogenic/likely pathogenic variants on different chromosomes at the LDLR, Apo-B, PCSK9, or LDLRAP1 genes, or ≥2 such variants at different loci OR
  • B. History of untreated LDL-C >400 mg/dL AND ONE of:
  • 1. Cutaneous or tendon xanthomas before 10 years of age OR
  • 2. Untreated elevated LDL-C levels consistent with heterozygous FH in both parents (or in digenic form, one parent may have normal LDL-C levels and the other may have LDL-C levels consistent with HoFH)
  • AND
  • 2. ONE of the following:
  • A. Tried a high-intensity statin (e.g., atorvastatin 40–80 mg, rosuvastatin 20–40 mg daily) for 8 weeks and had an inadequate response OR
  • B. Intolerance or hypersensitivity to ALL high-intensity statins OR
  • C. FDA labeled contraindication to ALL high-intensity statins
  • AND
  • 3. Will use other lipid-lowering therapy (e.g., statin, ezetimibe, lipoprotein apheresis, lomitapide, evinacumab)
  • OR
  • B. BOTH of the following:
  • 1. ONE of:
  • A. Diagnosis of heterozygous familial hypercholesterolemia (HeFH) AND ONE of:
  • 1. Genetic confirmation of one mutant allele at LDLR, Apo-B, PCSK9, or LDLRAP1 gene OR
  • 2. Pre-treatment LDL-C >190 mg/dL OR
  • 3. Clinical manifestations of HeFH (e.g., cutaneous xanthomas, tendon xanthomas, corneal arcus) OR
  • 4. 'Definite' or 'possible' FH as defined by Simon Broome criteria OR
  • 5. Dutch Lipid Clinic Network Criteria score >5 OR
  • 6. Treated LDL-C ≥100 mg/dL after statin treatment with or without ezetimibe
  • OR
  • B. Diagnosis of clinical atherosclerotic cardiovascular disease (ASCVD) AND ONE of:
  • 1. Acute coronary syndrome OR
  • 2. History of myocardial infarction OR
  • 3. Stable or unstable angina OR
  • 4. Coronary or other arterial revascularization OR
  • 5. Stroke OR
  • 6. Transient ischemic attack OR
  • 7. Peripheral arterial disease, including aortic aneurysm, presumed to be of atherosclerotic origin
  • OR
  • C. Diagnosis of primary hyperlipidemia AND ONE of:
  • 1. Coronary artery calcium or calcification (CAC) score ≥300 Agatston units OR
  • 2. Pre-treatment LDL-C ≥190 mg/dL
  • OR
  • D. 20–29% 10-year ASCVD risk AND LDL-C ≥130 mg/dL while on maximally tolerated statins OR
  • E. 30–39% 10-year ASCVD risk AND LDL-C ≥100 mg/dL while on maximally tolerated statin OR
  • F. ≥40% 10-year ASCVD risk AND LDL-C ≥70 mg/dL while on maximally tolerated statin
  • AND
  • 2. ONE of:
  • A. Adherent to high-intensity statin therapy (atorvastatin 40–80 mg or rosuvastatin 20–40 mg daily) for ≥8 consecutive weeks AND meets LDL-C thresholds per above OR equivalent statin intolerance/contraindication per plan definitions