Evinacumab-dgnb (Evkeeza) IV infusion — Coverage Criteria for Homozygous Familial Hypercholesterolemia
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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.
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).
Coverage Criteria for Evinacumab-dgnb (Evkeeza)
Initial Therapy Criteria
Covered when ALL of the following are met for initial approval:
Medical record documentation required
See policy for detailed exceptions and definitions (statin/ezetimibe/PCSK9 intolerance, genetic null homozygous)
Medical record documentation required
See Site of Care Medical Necessity Criteria
Continuation Therapy Criteria
Covered when ALL of the following are met for continuation approval:
Medical record documentation required
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
| J1305 | Evinacumab-dgnb, 1 mg (per HCPCS in policy dosing reference) |
Prior Authorization, Documentation, and Provider Requirements
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).
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.
- Patients 7–9 years: documentation of ≥8 weeks adherence to highest age-appropriate or maximally tolerated statin therapy OR documented statin intolerance (see definitions) is required.
- Patients ≥10 years: must be currently treated with and adherent to ALL of the following for ≥8 continuous weeks unless intolerant or contraindicated: high-intensity or maximally tolerated statin therapy (or documented statin intolerance/contraindication), ezetimibe (unless intolerant/contraindicated), AND a PCSK9 inhibitor unless intolerant/contraindicated or genetic confirmation of two LDLR null alleles is present.
- LDL-C thresholds for approval: 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 therapies.
- Required documentation: medical records confirming therapy trials, intolerances/contraindications, adherence for ≥8 weeks, and LDL-C lab values.
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.
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.
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).
Key Definitions
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.
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