ModifiedUnitedHealthcarePolicy 2026D0104J

Evkeeza (evinacumab-dgnb) for homozygous familial hypercholesterolemia (HoFH)

Defines UnitedHealthcare Medical Benefit Drug Policy coverage criteria for Evkeeza (evinacumab-dgnb) as adjunct therapy to lower LDL-C in patients with homozygous familial hypercholesterolemia (HoFH), including initial and continuation authorization requirements for commercial members.

Policy Summary

PayerUnitedHealthcare

PolicyEvkeeza (evinacumab-dgnb) for HoFH

Policy CodePolicy 2026D0104J

Change TypeCode removals and content updates

Effective DateApr 1, 2026

Next Review DateN/A

Key ActionObtain prior authorization with documentation of HoFH diagnosis and required prior therapies; initial and reauthorization periods are limited to no more than 12 months.

SourceLink

POLICY UPDATE CHANGES

Removed ICD-10 diagnosis codes E78.011, E78.019, and Z83.42.

Updated Clinical Evidence, FDA, and References sections to reflect the most current information.

Archived previous policy version 2025D0104I.

2026-04-01policy revision date

3ICD-10 codes removed

≥47%LDL-C reduction (ELIPSE)

15 mg/kg q4wEvkeeza dosing (trial)

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria for Evkeeza (evinacumab-dgnb)

Initial Therapy

Covered when ALL of the following are met for initial therapy:

Initial authorization: Diagnosis of HoFH by, or in consultation with, a lipid specialist (e.g., cardiologist, endocrinologist, lipid specialist/lipidologist); AND confirmation of HoFH by one of the following: (1) genetic confirmation of biallelic pathogenic/likely pathogenic variants on different chromosomes at LDLR, APOB, PCSK9, or LDLRAP1 (or ≥ 2 such variants at different loci); OR (2) both untreated LDL-C > 400 mg/dL and one of: xanthoma before 10 years of age OR evidence of familial hypercholesterolemia in at least one parent; AND one of the following: patient is < 10 years of age OR patient has failed to achieve LDL-C < 55 mg/dL despite required background therapy OR has documented intolerance/contraindication as specified (statin intolerance/contraindication, ezetimibe history or intolerance, PCSK9 therapy trial/nonresponse or intolerance, known two LDL-receptor negative alleles, prior lomitapide, or prior lipoprotein apheresis); AND patient will continue other traditional LDL-C lowering therapies (e.g., maximally tolerated statins, ezetimibe) while on Evkeeza; AND Evkeeza will not be used in combination with Juxtapid (lomitapide); AND Evkeeza dosing is in accordance with FDA-approved labeling; AND initial authorization will be for no more than 12 months.

Preserves Medicare-specific notes referenced in policy

Continuation Therapy

Covered when ALL of the following are met for continuation of therapy:

Continuation authorization: Documentation of a positive clinical response to Evkeeza therapy; AND Evkeeza will not be used in combination with Juxtapid (lomitapide); AND Evkeeza dosing is in accordance with FDA-approved labeling; AND reauthorization will be for no more than 12 months.

Positive clinical response is required for reauthorization as stated in policy.

Evkeeza (evinacumab-dgnb) must not be used in combination with Juxtapid (lomitapide). This prohibition applies to both initiation and continuation of therapy and is a required element for coverage consideration.

When determining coverage for Evkeeza, always consult the member-specific benefit plan document, as plan terms may differ from this standard policy. In the event of a conflict, the member-specific benefit plan document governs; verify any applicable federal or state mandates before applying this policy.

Listing a procedure or diagnosis code in this policy is for reference only and does not imply that the service is a covered or non-covered health service. Benefit coverage is determined by the member-specific benefit plan document and applicable laws, which may require or restrict coverage; inclusion of a code does not guarantee reimbursement or claim payment.

Provider Actions, Documentation, and Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required for Evkeeza (evinacumab-dgnb). Initial authorization will be limited to no more than 12 months; reauthorization (continuation) will also be limited to no more than 12 months.

Verify prior authorization requirements and coverage under the member-specific benefit plan before submitting (member plan governs if conflict).
UnitedHealthcare may use third-party utilization management tools (e.g., InterQual) and step therapy as applicable.

Denial Risk

Denial Triggers

Coverage may be denied if required documentation or specialist involvement is not provided, or if prior therapy or clinical criteria are not met.

Lack of documentation that diagnosis of HoFH was made by, or in consultation with, a lipid specialist (e.g., cardiologist, endocrinologist, lipid specialist/lipidologist).
Absence of genetic confirmation of biallelic pathogenic/likely pathogenic variants (or meeting the alternative clinical criteria).
Failure to document required prior therapies, intolerances, contraindications, or reasons for not using recommended agents (statin, ezetimibe, PCSK9 therapies, etc.).
Use in combination with Juxtapid (lomitapide) — not permitted.

Documentation Required

Required Documentation

Submit complete medical records to support the diagnosis, prior therapy history, and clinical response for continuation. Documentation must demonstrate the genetic or clinical criteria for HoFH and required concomitant therapies.

Medical records (chart notes, laboratory values) confirming genetic testing showing biallelic pathogenic/likely pathogenic variants in LDLR, APOB, PCSK9, or LDLRAP1 genes, or ≥2 such variants at different loci.
If genetic confirmation not available, documentation of untreated LDL-C > 400 mg/dL and either xanthoma before age 10 or evidence of familial hypercholesterolemia in at least one parent.
Document current lipid specialist involvement (consultation or management notes).
Medication history showing maximally tolerated statin therapy and use of ezetimibe as an adjunct, or documentation of intolerance/contraindication to statins and/or ezetimibe as specified.
Documentation of PCSK9 therapy use and response, or physician attestation of LDL-receptor negative alleles, or documentation of intolerance/contraindication to PCSK9 therapy, if applicable.
For continuation requests, documentation of positive clinical response to Evkeeza (e.g., LDL-C reductions, clinical notes).
Treatment plan showing Evkeeza dosing per FDA labeling and confirmation Evkeeza will not be used with Juxtapid (lomitapide).

Step Therapy

Prior Therapy Requirements

Prior therapy requirements must be documented prior to approval unless patient is < 10 years of age or has documented contraindication/intolerance or other specified exceptions.

Evidence of maximally tolerated statin therapy OR documentation of statin intolerance (persistent symptoms >2 weeks, myalgia without CK elevation, myositis with CK <10x ULN, rhabdomyolysis or CK >10x ULN, or contraindication to all statins).
Evidence of adjunctive ezetimibe therapy with maximally tolerated statin OR documentation of intolerance/contraindication to ezetimibe.
Evidence of treatment with PCSK9 therapy (e.g., alirocumab, evolocumab) and lack of response, OR physician attestation of two LDL-receptor negative alleles, OR documentation of intolerance/contraindication to PCSK9 therapy.
Documentation of prior treatment with lomitapide (Juxtapid) or lipoprotein apheresis, if applicable.

Definitions and Key Terms

HoFH diagnostic criteria — genetic confirmation and clinical criteria

Genetic confirmationBiallelic pathogenic/likely pathogenic variants on different chromosomes at LDLR, APOB, PCSK9, or LDLRAP1 (or ≥2 such variants at different loci)

Clinical criteria — LDL-CUntreated LDL-C > 400 mg/dL plus one of: xanthoma before age 10 OR evidence of familial hypercholesterolemia in at least one parent

Specialist involvementDiagnosis of HoFH must be made by, or in consultation with, a lipid specialist (e.g., cardiologist, endocrinologist, lipidologist)

Evkeeza (evinacumab-dgnb) — description, mechanism, administration, HCPCS

Drug descriptionEvinacumab-dgnb (Evkeeza) is a recombinant human monoclonal antibody that binds and inhibits ANGPTL3, promoting VLDL processing and lowering LDL-C as adjunct therapy

Route and administrationAdministered intravenously (IV) per FDA labeling; dosing evaluated in trials was 15 mg/kg every 4 weeks

HCPCS codingJ1305 — Injection, evinacumab-dgnb, 5 mg

References — cited clinical guidance and evidence

Key clinical guidanceCuchel M, Raal FJ, Hegele RA, et al. 2023 Update on European Atherosclerosis Society Consensus Statement on HoFH. Eur Heart J. 2023;44(25):2277-2291. doi:10.1093/eurheartj/ehad197.

Systematic reviewGu J, Gupta RN, Cheng HK, Xu Y, Raal FJ. Current treatments for management of HoFH: systematic review and commentary. Eur J Prev Cardiol. 2024;31(15):1833-1849. doi:10.1093/eurjpc/zwae144.

Professional society criteriaEuropean Atherosclerosis Society 2023 consensus statement updating clinical diagnostic criteria for HoFH (including LDL-C >10 mmol/L / >400 mg/dL as suggestive)

OpenPayer

Evkeeza (evinacumab-dgnb) for homozygous familial hypercholesterolemia (HoFH)

Coverage Criteria for Evkeeza (evinacumab-dgnb)

Coding — Relevant Procedure and Diagnosis Codes

Provider Actions, Documentation, and Authorization

Background on Condition and Therapy

Definitions and Key Terms

E78.011	ICD-10 diagnosis code removed
E78.019	ICD-10 diagnosis code removed
Z83.42	ICD-10 diagnosis code removed