Evinacumab-dgnb (Evkeeza™)

Evkeeza (evinacumab‑dgnb) is covered as an adjunct to other LDL‑C–lowering therapies for FDA‑approved treatment of homozygous familial hypercholesterolemia (HoFH) in patients ≥12 years who have either genetic confirmation (two mutant alleles in LDLR, APOB, PCSK9, or LDLRAP1) or meet specified untreated/treated LDL‑C thresholds with qualifying clinical manifestations or parental hypercholesterolemia, and is excluded for patients <12 years or those who do not meet these entry criteria. Coverage requires documentation of age/diagnosis/genetic or LDL‑C and parental/clinical evidence, prior therapy trials (high‑intensity statin and statin+ezetimibe ≥8 weeks with LDL‑C ≥70 mg/dL unless documented statin intolerance or known two LDL‑receptor negative alleles; statin‑intolerant patients generally must trial a PCSK9 inhibitor ≥8 weeks unless two LDL‑receptor negative alleles), prescribing/consultation by a cardiology/endocrine/lipid specialist, dosing of 15 mg/kg IV no more often than every 4 weeks, and is authorized for 12 months (not covered without required specialist documentation).