Last Review Date: 04/04/2024

Date of Origin: 03/01/2021

Dates Reviewed: 03/2021, 07/2021, 10/2021, 04/2022, 04/2023, 04/2024

1. Length of Authorization

Coverage is provided for 3 months for initial approval and may be renewed every 12 months.

2. Dosing Limits

A. Quantity Limit (max daily dose) [NDC Unit]:

• Evkeeza 345 mg/2.3 mL single-dose vial: 2 vials per 28 days
• Evkeeza 1200 mg/8 mL single-dose vial: 1 vial per 28 days

B. Max Units (per dose and over time) [HCPCS Unit]:

• 345 billable units (1725 mg) every 28 days

3. Initial Approval Criteria ¹

Coverage is provided in the following conditions:

• Patient is at least 5 years of age; AND
• Baseline low-density lipoprotein cholesterol (LDL-C) labs have been obtained prior to initiating treatment (required for renewal); AND
• Patient does not have a diagnosis of heterozygous familial hypercholesterolemia (HeFH); AND

Universal Criteria

• Must be prescribed by, or in consultation with, a specialist in cardiology, lipidology, or endocrinology; AND
• Will not be used in combination with lomitapide; AND

Homozygous Familial Hypercholesterolemia (HoFH) † Ф ^{1,3,5,6,11,12}

• Patient has a confirmed diagnosis of Homozygous Familial Hypercholesterolemia (HoFH) by any of the following:
  • Confirmed DNA test for functional mutation(s) in LDL receptor alleles or alleles known to affect LDL receptor functionality (i.e. LDLR, PCSK9, or apoB gene(s)); OR
  • Untreated LDL-C > 500 mg/dL or treated LDL-C ≥ 300 mg/dL; AND
    • Cutaneous or tendon xanthoma before age 10 years; OR
    • Untreated LDL-C levels in both parents consistent with HeFH; AND

• Must be used as an adjunct to a low-fat or heart-healthy diet; AND

• Patient has been receiving stable background lipid lowering therapy for at least 4 weeks; AND
• Therapy will be used in conjunction with other LDL-lowering therapies (e.g., statins, ezetimibe, PCSK9 inhibitors, LDL apheresis); AND
• Patient has tried and failed at least a 3 month trial of adherent therapy with: ezetimibe used in combination with the highest available (or maximally tolerated*) dose of atorvastatin OR rosuvastatin, unless contraindicated; AND
• Patient has tried and failed at least a 3 month trial of adherent therapy with: combination therapy consisting of the highest available (or maximally tolerated*) dose of atorvastatin OR rosuvastatin, ezetimibe, AND a PSCK9 inhibitor indicated for HoFH (e.g., evolocumab), unless contraindicated; AND
• Despite pharmacological treatment, unless contraindicated, with a PCSK9 inhibitor, statin, and ezetimibe, the patient’s LDL cholesterol ≥ 100 mg/dL (or ≥ 70 mg/dL for patients with clinical atherosclerotic cardiovascular disease [ASCVD])

† FDA Approved Indication(s); ‡ Compendia Recommended Indication(s); Ф Orphan Drug

4. Renewal Criteria ^1,8

Coverage can be renewed based upon the following criteria:

• Patient continues to meet the universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
• Absence of unacceptable toxicity from therapy. Examples of unacceptable toxicity include: severe hypersensitivity reactions, etc.; AND
• Patient has had a reduction in LDL-C, when compared to the baseline labs (prior to initiating evinacumab); AND
• Patient continues to adhere to diet and background lipid lowering therapy (e.g., statin, ezetimibe, PCSK9-I, LDL apheresis)

5. Dosage/Administration ¹

6. Billing Code/Availability Information

HCPCS code:

• J1305 – Injection, evinacumab-dgnb, 5 mg; 1 billable unit = 5 mg

NDC:

• Evkeeza 345 mg/2.3 mL (150 mg/mL) single-dose vial:  61755-0013-xx
• Evkeeza 1,200 mg/8 mL (150 mg/mL) single-dose vial:  61755-0010-xx

7. References

1. Evkeeza [package insert]. Tarrytown, NY; Regeneron, Inc.; March 2023. Accessed February 2024.
2. Mozaffarian D, et al. Heart disease and stroke statistics--2015 update: a report from the American Heart Association. Circulation. 2015 Jan 27;131(4):e29-322. doi: 10.1161/CIR.0000000000000152. Epub 2014 Dec 17.
3. Rosenson RS, Durrington P. (2023) Familial hypercholesterolemia in adults: Overview. In: Freeman MW, Parikh N (Eds). UpToDate. Last updated: Dec 07, 2023. Accessed February 27, 2024. Available from: https://www.uptodate.com/contents/familial-hypercholesterolemia-in-adults-overview.
4. Stone NJ, et al. 2013 ACC/AHA guideline on the treatment of blood cholesterol to reduce atherosclerotic cardiovascular risk in adults: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation, 2014; 129(25 Suppl 2): S1–45.
5. Jacobson et al. National Lipid Association recommendations for patient-centered management of dyslipidemia: Part 1 – executive summary. Journal of Clinical Lipidology. 2014. Available at: http://www.sciencedirect.com/science/article/pii/S1933287414002748. Accessed February 27, 2024.
6. Gidding SS, Champagne MA, de Ferranti SD, et al. The Agenda for Familial Hypercholesterolemia: A Scientific Statement From the American Heart Association. Circulation. 2015 Dec 1;132(22):2167-92. doi: 10.1161/CIR.0000000000000297.
7. Lloyd-Jones DM, Morris PB, Ballantyne CM, et al. 2017 Focused Update of the 2016 ACC Expert Consensus Decision Pathway on the Role of Non-Statin Therapies for LDL-Cholesterol Lowering in the Management of Atherosclerotic Cardiovascular Disease Risk: A Report of the American College of Cardiology Task Force on Expert Consensus Decision Pathways. J Am Coll Cardiol. 2017 Oct 3;70(14):1785-1822. 
8. Grundy SM, Stone NJ, Bailey AL,  et al. AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/APhA/ASPC/NLA/PCNA Guideline on the Management of Blood Cholesterol: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2018;000:e1–e120. DOI: 10.1161/CIR.0000000000000625.
9. Jacobson TA, Ito MK, Maki KC, et al. National Lipid Association recommendations for patient-centered management of dyslipidemia: Part 1 – executive summary. Journal of Clinical Lipidology. 2014:8(5):473–488. DOI: 10.1016/j.jacl.2014.07.007.
10. Jacobson TA, Maki KC, Orringer C, et al. National Lipid Association Recommendations for Patient-Centered Management of Dyslipidemia: Part 2. J Clin Lipidol. 2015 Nov-Dec;9(6 Suppl):S1-122.e1. doi: 10.1016/j.jacl.2015.09.002.
11. Cuchel M, Bruckert E, Ginsberg HN, et al. Homozygous familial hypercholesterolaemia: new insights and guidance for clinicians to improve detection and clinical management. A position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society. Eur Heart J. 2014 Aug 21;35(32):2146-57. doi: 10.1093/eurheartj/ehu274. Epub 2014 Jul 22
12. Raal FJ, Rosenson RS, Reeskamp et al; ELIPSE HoFH Investigators. Evinacumab for Homozygous Familial Hypercholesterolemia. N Engl J Med. 2020 Aug 20;383(8):711-720. doi: 10.1056/NEJMoa2004215.
13. Lloyd-Jones DM, Morris PB, Ballantyne CM, et al. 2022 ACC Expert Consensus Decision Pathway on the Role of Nonstatin Therapies for LDL-Cholesterol Lowering in the Management of Atherosclerotic Cardiovascular Disease Risk: A Report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2022 Oct 4;80(14):1366-1418. doi: 10.1016/j.jacc.2022.07.006.
14. Wiegman A, Greber-Platzer S, Ali S, et al. Evinacumab for Pediatric Patients With Homozygous Familial Hypercholesterolemia. Circulation. 2024 Jan 30;149(5):343-353. doi: 10.1161/CIRCULATIONAHA.123.065529. Epub 2023 Oct 20. PMID: 37860863; PMCID: PMC10814999.

Appendix 1 – Covered Diagnosis Codes

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

The preceding information is intended for non-Medicare coverage determinations. Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determinations (NCDs) and/or Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. Local Coverage Articles (LCAs) may also exist for claims payment purposes or to clarify benefit eligibility under Part B for drugs which may be self-administered. The following link may be used to search for NCD, LCD, or LCA documents: https://www.cms.gov/medicare-coverage-database/search.aspx. Additional indications, including any preceding information, may be applied at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA): N/A