CurrentBlue Cross Blue Shield - North CarolinaPolicy N/A

Evinacumab-dgnb (Evkeeza) IV infusion — Coverage Criteria for Homozygous Familial Hypercholesterolemia

Know when Blue Cross Blue Shield - North Carolina releases new policies or updates existing guidance.

Defines medical necessity, site-of-care, authorization, and dosing/units for evinacumab-dgnb (Evkeeza) IV infusion for patients aged ≥1 year with homozygous familial hypercholesterolemia (HoFH); applies to Blue Cross NC membership and providers requesting coverage.

Policy Summary

PayerBlue Cross Blue Shield - North Carolina

PolicyEvinacumab-dgnb (Evkeeza) IV infusion — Coverage Criteria for HoFH

Policy CodePolicy N/A

Change TypeOperational revisionspediatric expansion

Effective DateAug 1, 202613d

Next Review DateJan 1, 2027

Key ActionObtain prior authorization and document genetic confirmation or specified LDL-C history plus trials of required LDL‑C lowering therapies per age-specific criteria.

SourceLink

POLICY UPDATE CHANGES

Reformatted initial diagnostic confirmation criteria and removed required submission of baseline LDL-C within 60 days; adjusted age-specific statin trial requirements and refined statin intolerance definitions.

Added requirement that patients ≥10 years must be adherent to ezetimibe in addition to statin therapy for initial approval.

Expanded indication to pediatric patients 1 year of age and older (previously older ages).

≥1 yrMinimum patient age eligible

Related Billing Codes

HCPCS-J1305-EVINACUMAB-INJECTION-5MG

HCPCS J1305: Evinacumab-dgnb Injection, 5 mg

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Coverage Criteria for Evinacumab-dgnb (Evkeeza)

Initial Therapy Criteria

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

Age: Patient is 1 year of age or olderage >= 1 year

Diagnosis of homozygous familial hypercholesterolemia (HoFH)

Diagnostic confirmation: Diagnosis confirmed by ONE of: (a) genetic confirmation of two mutant alleles in LDLR, APOB, PCSK9, or LDLRAP1; OR (b) history of untreated LDL-C >400 mg/dL with clinical manifestations of HoFH before age 10 OR untreated elevated LDL-C consistent with heterozygous FH in both parents (or digenic form)

Medical record documentation required

Age-specific prior therapy requirements: ONE of the following must be met by age group: (a) patient <7 years of age (may bypass some prior trial requirements); OR (b) patient 7–9 years: currently treated with and adherent to the highest age-appropriate or maximally tolerated statin for ≥8 continuous weeks OR statin intolerant per policy definition; OR (c) patient ≥10 years: currently treated with and adherent to high-intensity or maximally tolerated statin, ezetimibe, and a PCSK9 inhibitor for ≥8 continuous weeks unless intolerant/contraindicated or genetically null homozygous

See policy for detailed exceptions and definitions (statin/ezetimibe/PCSK9 intolerance, genetic null homozygous)

LDL-C despite therapy: Patient's LDL-C remains ≥100 mg/dL (or ≥55 mg/dL with clinical ASCVD or major ASCVD risk factors) despite treatment with other LDL-C lowering therapiesLDL-C >=100 mg/dL; or >=55 mg/dL with ASCVD

Medical record documentation required

Concomitant therapies: Requested agent will be used in conjunction with other LDL-C lowering therapies (e.g., maximally tolerated statin therapy, ezetimibe, PCSK9 antibodies, lomitapide, lipoprotein apheresis)

Contraindications: Patient does not have any FDA labeled contraindications to evinacumab-dgnb

Prescriber specialty: Prescriber is a specialist in the area of the patient's diagnosis (cardiology, endocrinology, geneticist, and/or lipid specialist) or has consulted a specialist

Quantity limits: Requested quantity does not exceed the maximum units allowed for duration of approval (initial 1,800 units)Initial 1,800 units

Site of Care: If administered inpatient or in an outpatient hospital setting, Site of Care criteria apply; inpatient admission must not be solely for infusion

See Site of Care Medical Necessity Criteria

Continuation Therapy Criteria

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

Prior approval: Patient was approved through Blue Cross NC initial criteria OR would have met initial criteria at therapy start

Clinical benefit: Patient has demonstrated clinical benefit with the agent, shown by a reduction in LDL-C from baseline prior to starting therapydocumented LDL-C reduction from baseline

Medical record documentation required

Concomitant therapies: Requested agent will continue to be used in conjunction with other LDL-C lowering therapies

Contraindications: Patient does not have any FDA labeled contraindications to the requested agent

Inpatient admissions that are scheduled solely for the purpose of administering an injection or infusion of evinacumab-dgnb (Evkeeza) are excluded from coverage unless the admission meets other inpatient medical necessity criteria. Per the Site of Care Medical Necessity Criteria, an inpatient administration is allowable only when the standard medical necessity criteria for the medication are met AND the inpatient admission is not for the sole purpose of infusion. If the inpatient Site of Care criteria are not satisfied, administration should occur in a home-based infusion setting or physician office.

Use of evinacumab-dgnb (Evkeeza) is limited to patients with a diagnosis of homozygous familial hypercholesterolemia (HoFH) who meet the specific medical necessity criteria. Requests for diagnoses other than HoFH, or requests that do not meet the required criteria (including age ≥1 year, required diagnostic confirmation, age-specific prior therapy trials or documented statin intolerance, required concomitant LDL-C lowering therapies, prescriber specialty, and quantity limits), are considered not medically necessary and will not be covered.

Coding and LDL-C Thresholds

Covered HCPCS Codes / Dosing referenceHCPCSCovered

J1305

Evinacumab-dgnb, 1 mg (per HCPCS in policy dosing reference)

LDL-C thresholds for continued consideration (continuation criteria)

Continuation LDL-C thresholdLDL-C remains ≥100 mg/dL despite treatment with other LDL‑C lowering therapies

Lower threshold with ASCVD or major risk factorsLDL-C remains ≥55 mg/dL if the patient has clinical ASCVD or major ASCVD risk factors despite other LDL‑C lowering therapies

Therapies considered in threshold assessmentIncludes maximally tolerated statin therapy, ezetimibe, PCSK9 inhibitor antibodies, lipoprotein apheresis, bempedoic acid, etc.

Documentation requiredMedical record documentation demonstrating LDL‑C level meeting the threshold despite other LDL‑C lowering therapies is required for continuation

Prior Authorization, Documentation, and Provider Requirements

Prior Authorization

Prior Authorization and Duration

Prior authorization required. Prior authorization is required for initiation and continuation of therapy. Initial approvals are for 180 days (6 months); continuation approvals are for 365 days (1 year). The requested quantity must not exceed the maximum units allowed for the duration of approval.

Initial duration: 180 days (6 months).
Continuation duration: 365 days (1 year).
Maximum units: Initial: 1,800; Continuation: 3,600 (see dosing table).

Step Therapy

Required Prior LDL-C Lowering Therapies

Required prior LDL-C lowering therapies. Medical record documentation must show trials or intolerance to required LDL-C lowering therapies prior to approval. For patients ≥1 year with HoFH, documentation of concurrent use of other LDL-C lowering therapies is required and LDL-C thresholds must be met despite those therapies.

Patients <7 years: no specific statin requirement listed beyond age criteria.

Key Definitions

Genetic confirmation of HoFH

DefinitionGenetic confirmation = presence of two mutant alleles at the LDLR, Apo‑B, PCSK9, or LDLRAP1 genes documented in the medical record

Alternative clinical confirmationHistory of untreated LDL‑C >400 mg/dL with clinical HoFH manifestations before age 10, or untreated elevated LDL‑C consistent with heterozygous FH in both parents (or digenic form)

DocumentationMedical record documentation of genetic test results or the specified LDL‑C/clinical history is required

Statin intolerance

DefinitionStatin intolerance = one of: statin‑related rhabdomyolysis; OR statin‑related skeletal muscle symptoms (myopathy or myalgia) with trials of at least two statin therapies that resolved upon discontinuation; OR elevations in hepatic transaminases due to statin therapy; OR FDA‑labeled contraindication/hypersensitivity to all statins

Required trial documentation

Background

Evinacumab-dgnb (Evkeeza) is an intravenous monoclonal antibody directed against angiopoietin-like 3 (ANGPTL3) used as an adjunct to diet and other LDL‑C lowering therapies to reduce LDL‑C in patients with homozygous familial hypercholesterolemia (HoFH). Dosing per the label is 15 mg/kg every 4 weeks. Use is intended in coordination with other lipid-lowering strategies and specialist oversight.

Policy Summary

PayerBlue Cross Blue Shield - North Carolina

PolicyEvinacumab-dgnb (Evkeeza) IV infusion — Coverage Criteria for HoFH

Policy CodePolicy N/A

Change TypeOperational revisionspediatric expansion

Effective DateAug 1, 202613d

Next Review DateJan 1, 2027

Key ActionObtain prior authorization and document genetic confirmation or specified LDL-C history plus trials of required LDL‑C lowering therapies per age-specific criteria.

SourceLink

On This Page

Documentation Required

Required Medical Record Documentation

Medical record documentation required. Maintain documentation of diagnostic confirmation, therapy history, adherence, and LDL-C response before and after initiation of therapy. The prescriber should be a specialist or have consulted a specialist.

Diagnosis confirmation: genetic confirmation of two mutant alleles in LDLR, Apo-B, PCSK9, or LDLRAP1 OR history of untreated LDL-C >400 mg/dL with clinical criteria consistent with HoFH.
Therapy history: records of statin trials (including at least two different statins if muscle symptoms), ezetimibe, PCSK9 inhibitors, apheresis, bempedoic acid, lomitapide where applicable, and reasons for intolerance or contraindication.
Adherence: documentation of ≥8 continuous weeks of adherence to required therapies when applicable.
LDL-C response: baseline LDL-C prior to the requested agent and on-therapy LDL-C demonstrating clinical benefit for continuation requests.
Specialist involvement: prescriber is a cardiologist, endocrinologist, geneticist, lipid specialist, or has consulted one.

Denial Risk

Failure to Meet Initial Medical Necessity Criteria

Denial risk if initial medical necessity criteria not met. Requests that do not meet age, diagnosis confirmation, required prior therapy trials/intolerance documentation, LDL-C thresholds, specialist involvement, or exceed maximum units/duration are subject to denial.

Common denial reasons: missing genetic confirmation or clinical documentation for HoFH, insufficient trial of or documentation of intolerance to required LDL-C lowering therapies, lack of LDL-C lab values showing thresholds, no evidence of specialist involvement, or request exceeds allowed units/duration.

Denial Risk

Site-of-Care Inpatient-Only Admissions

Site-of-care: inpatient-only admissions and outpatient hospital infusion criteria. Injection or infusion administration in an inpatient or outpatient hospital setting must meet Site of Care Medical Necessity Criteria. Inpatient admission solely for administration may lead to denial.

Inpatient: Allowed only if medical necessity criteria are met AND the inpatient admission is NOT for the sole purpose of administering the injection/infusion.
Outpatient hospital setting: Allowed only if medical necessity criteria are met AND ONE of the following applies: history of severe adverse event to the drug, conditions increasing risk for severe adverse events, history of mild adverse events not managed with pre-medication, inability to adhere to treatment schedule, new to therapy (<3 months) or re-initiation as defined, or requirement to change product formulation.
If Site of Care criteria are not met, administration should occur in a home-based infusion or physician office setting (with or without supervision by certified healthcare professional).

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Evinacumab-dgnb (Evkeeza) IV infusion — Coverage Criteria for Homozygous Familial Hypercholesterolemia

Related Billing Codes

Coverage Criteria for Evinacumab-dgnb (Evkeeza)

Coding and LDL-C Thresholds

Prior Authorization, Documentation, and Provider Requirements

Key Definitions

Background