Evinacumab-dgnb (Evkeeza) — CPB 0989 | AETNA Coverage Update

Aetna modified CPB 0989 for evinacumab-dgnb (Evkeeza), effective January 14, 2026. Here's what billing teams need to do.

Aetna, a CVS Health company, updated its coverage policy for evinacumab-dgnb (Evkeeza) under CPB 0989 in the CPB 0989 Aetna system, with the effective date of January 14, 2026. This drug is billed under HCPCS J1305 and administered via infusion codes CPT 96365–96368. The change tightens age-stratified therapy requirements and LDL-C thresholds that determine medical necessity — details your billing team needs before submitting claims.

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Quick-Reference Table

Field	Detail Copy
Payer	Aetna, a CVS Health company
Policy	Evinacumab-dgnb (Evkeeza) — CPB 0989
Policy Code	CPB 0989
Change Type	Modified
Effective Date	January 14, 2026
Impact Level	High
Specialties Affected	Cardiology, Lipid Disorders, Pediatric Endocrinology, Preventive Cardiology, Infusion Centers
Key Action	Confirm HoFH diagnosis documentation, LDL-C thresholds, and age-specific background therapy requirements before submitting precertification for J1305

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Aetna Evinacumab-dgnb Coverage Criteria and Medical Necessity Requirements 2026

The Aetna evinacumab-dgnb coverage policy covers Evkeeza for members aged one year and older diagnosed with homozygous familial hypercholesterolemia (HoFH). That's the narrow lane. Everything else is experimental.

To establish medical necessity, Aetna requires confirmed HoFH diagnosis through genetic or clinical criteria. Genetic confirmation means documented variants in two LDLR alleles, homozygous or compound heterozygous variants in APOB or PCSK9, or homozygosity/compound heterozygosity in LDLRAP1. These genetic findings link directly to CPT 81401, 81405, 81406, and 81407 — your molecular pathology codes for the relevant mutation analyses.

If genetic testing isn't available or was never done, Aetna accepts clinical criteria: untreated LDL-C above 400 mg/dL plus either cutaneous or tendinous xanthomas before age 10, or both parents with untreated LDL-C at or above 190 mg/dL. Confirm LDL-C results are documented via CPT 83721 (direct LDL measurement) or CPT 82465 (total cholesterol). Aetna will want those lab results in the prior authorization file.

LDL-C Thresholds for Initial Approval

Before initiating Evkeeza, the member's treated LDL-C must meet one of two thresholds:

#	Covered Indication Copy
1	Treated LDL-C ≥ 70 mg/dL — qualifies on its own
2	Treated LDL-C ≥ 55 mg/dL — qualifies only if the member also has a history of ASCVD or a major ASCVD risk factor (age 65+, familial hypercholesterolemia, diabetes, chronic kidney disease, or history of congestive heart failure)

This LDL-C threshold structure mirrors Aetna's PCSK9 inhibitor criteria. If your team already handles Repatha or Praluent prior authorizations, the documentation logic is familiar — but Evkeeza's HoFH-specific requirements add a genetic confirmation layer that PCSK9 inhibitor cases don't always need.

Age-Stratified Background Therapy Requirements

This is where the updated policy gets specific — and where prior authorization submissions commonly fail. Aetna now applies three distinct criteria tiers by age:

Age 10 and older: The member must be on stable treatment with at least three lipid-lowering therapies — such as statins, ezetimibe, and PCSK9-directed therapy — all at maximally tolerated doses. They must also continue that combination therapy alongside Evkeeza.

Age 7 to under 10: Aetna requires either stable treatment with at least one lipid-lowering therapy (e.g., a statin or LDL apheresis) at maximally tolerated dose, or documented intolerance or contraindication to other lipid-lowering therapies.

Age 1 to under 7: No background therapy requirement. Age alone satisfies this criterion.

Document the current therapy regimen explicitly in the prior auth submission. "Maximally tolerated dose" needs to be defined in the chart, not assumed. If a statin was discontinued for intolerance, document that too — Aetna will ask.

Prior Authorization and Site of Care

Prior authorization is required for all Aetna participating providers and members in applicable plan designs. Call (866) 752-7021 or fax (888) 267-3277. Use the Specialty Pharmacy Precertification SMN forms from Aetna's health professional portal.

Aetna's Site of Care Utilization Management Policy also applies. Evkeeza is an IV infusion — billed under CPT 96365 for the initial hour, CPT 96366 for each additional hour, CPT 96367 for additional drug sequential infusion, and CPT 96368 for concurrent infusion. Where that infusion happens affects reimbursement and may require separate site-of-care justification. Check Aetna's infusion site-of-care policy before routing patients to a higher-cost setting.

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Aetna Evkeeza Exclusions and Non-Covered Indications

Aetna states plainly: all indications other than HoFH are experimental, investigational, or unproven. There is no off-label coverage pathway here.

If a claim comes through with ICD-10 E78.1 (pure hyperglyceridemia), E78.3 (hyperchylomicronemia), or E88.1 (lipodystrophy), expect a claim denial. Those codes appear in the policy's code set, but they are not covered indications for Evkeeza. The only covered ICD-10 diagnosis code is E78.010 — homozygous familial hypercholesterolemia.

Billing J1305 against anything other than E78.010 is a fast path to a denial and a potential compliance issue. Audit your charge capture now.

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Coverage Indications at a Glance

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Aetna Evinacumab-dgnb Billing Guidelines and Action Items 2026

These steps apply now. The effective date is January 14, 2026 — this policy is already active.

Copy Action Items

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CPT, HCPCS, and ICD-10 Codes for Evinacumab-dgnb Under CPB 0989

HCPCS Codes

Code	Type	Description	Status Copy
J1305	HCPCS	Injection, evinacumab-dgnb, 5 mg	Covered when selection criteria are met
G9664	HCPCS	Patients who are currently statin therapy users or received an order (prescription) for statin therapy	Related code — not a primary billing code for Evkeeza

CPT Codes

Key ICD-10-CM Diagnosis Codes

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