Evkeeza (evinacumab-dgnb intravenous infusion) — Cigna

Homozygous Familial Hypercholesterolemia

Initial criteria

Patient age ≥ 5 years; AND
Diagnosis confirmed by ONE of the following (a, b, or c):
a) Genetic testing confirming pathogenic variant in LDLR, APOB, PCSK9, or LDLRAP1 gene; OR
b) Untreated LDL-C level > 400 mg/dL AND ONE of the following: (1) clinical manifestations of homozygous familial hypercholesterolemia before age 10 years; OR (2) at least one parent with untreated LDL-C ≥ 190 mg/dL or total cholesterol > 250 mg/dL; OR
c) Treated LDL-C level ≥ 300 mg/dL AND ONE of the following: (1) clinical manifestations of homozygous familial hypercholesterolemia before age 10 years; OR (2) at least one parent with untreated LDL-C ≥ 190 mg/dL or total cholesterol > 250 mg/dL; AND
Patient meets ONE of the following (a or b):
a) Has tried one high-intensity statin (atorvastatin ≥ 40 mg daily; rosuvastatin ≥ 20 mg daily) AND has tried one high-intensity statin plus ezetimibe for ≥ 8 continuous weeks AND LDL-C after these treatments remains ≥ 70 mg/dL; OR
b) Determined to be statin intolerant by meeting ONE of the following: (1) experienced statin-related rhabdomyolysis; OR (2) meets ALL of the following: (a) experienced skeletal-related muscle symptoms (myopathy, myalgia); AND (b) symptoms occurred during separate trials of both atorvastatin and rosuvastatin; AND (c) symptoms resolved upon discontinuation of each statin)

1 year