Evkeeza (evinacumab-dgnb intravenous infusion) — Cigna

Homozygous Familial Hypercholesterolemia (HoFH)

Initial criteria

Diagnosis of homozygous familial hypercholesterolemia (HoFH) has been confirmed by genetic testing (e.g., two mutant alleles at the LDLR, APOB, PCSK9, or LDLRAP1 gene locus)
Patient has an untreated LDL-C level > 400 mg/dL OR a treated LDL-C level ≥ 300 mg/dL
At least one parent of the patient had untreated LDL-C or total cholesterol levels consistent with familial hypercholesterolemia (e.g., untreated LDL-C level ≥ 190 mg/dL and/or untreated total cholesterol level > 250 mg/dL)
Patient has tried one high-intensity statin (e.g., atorvastatin, rosuvastatin) unless contraindicated or not tolerated
Patient has tried ezetimibe unless contraindicated or not tolerated
Patient meets ONE of the following: (a) has tried one PCSK9 inhibitor for ≥ 8 continuous weeks AND LDL-C level after this PCSK9 inhibitor therapy remains ≥ 70 mg/dL; OR (b) patient is known to have two LDL-receptor negative alleles; OR (c) patient is age 5 to 9 years

According to the prescriber, the patient has experienced a response to therapy (e.g., decreasing LDL-C, total cholesterol, non-high-density lipoprotein [non-HDL-C], or apolipoprotein B levels)

12 months