Evinacumab-dgnb (Evkeeza™)

Covered: Evkeeza is authorized only as adjunctive treatment for FDA‑approved homozygous familial hypercholesterolemia (HoFH) in patients ≥12 years, dosed 15 mg/kg IV every 4 weeks with 12‑month approvals and no coverage for off‑label use. Key requirements: documented age and specialist prescribing/consultation, genetic confirmation of two mutant alleles OR LDL criteria (untreated LDL‑C >500 mg/dL or treated LDL‑C ≥300 mg/dL) with early HoFH manifestations or parental HeFH, completion of high‑intensity statin and high‑intensity statin+ezetimibe ≥8 weeks (or meeting strict statin‑intolerance definitions), trial of a PCSK9 inhibitor ≥8 weeks unless two LDL‑receptor negative alleles, and submission of all relevant LDL‑C labs and medication trial documentation.

"Evkeeza is indicated as an adjunct to other low-density lipoprotein cholesterol (LDL-C) lowering therapies for the treatment of homozygous familial hypercholesterolemia (HoFH) in patients aged 12 y..."