Evkeeza (evinacumab-dgnb) — Coverage Criteria for Homozygous Familial Hypercholesterolemia (HoFH)
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This policy governs prior-authorization medical benefit coverage of Evkeeza (evinacumab-dgnb) for members with homozygous familial hypercholesterolemia (HoFH) in Arkansas PASSE. It describes initial and reauthorization clinical criteria, required documentation, dosing, and coverage stance.
Changed LDL threshold for diagnostic consideration to >400 mg/dL and specified at least 15% LDL reduction for reauthorization.
Updated age limit and J-code information in prior revisions.
Coverage Criteria for Evkeeza (evinacumab-dgnb)
Initial Therapy
Covered when ALL of the following are met:
If all requirements met, approve for 6 months.
Continuation Therapy / Reauthorization
Covered for continuation when ALL of the following are met:
If met, approve for an additional 12 months.
CareSource considers Evkeeza (evinacumab-dgnb) not medically necessary for the treatment of conditions that are not listed in this document. Off‑label uses of Evkeeza should be evaluated against the plan’s Off‑Label policy and require documentation supporting the medical necessity for that specific indication.
Specifically, use of Evkeeza for indications other than homozygous familial hypercholesterolemia (HoFH) as defined in this policy is not medically necessary. The policy’s reauthorization and initial‑coverage criteria apply only to HoFH; requests for other diagnoses will be denied unless the Off‑Label policy justification is provided and approved.
Coding, Thresholds, and Response Metrics
| J-code | Updated J code referenced in policy history (specific J-code not listed in document) |
Provider Requirements and Documentation
Prior Authorization Required
Evkeeza (evinacumab-dgnb) is covered under the medical benefit and requires prior authorization.
- Benefit type: Medical
- Prior authorization required
- Prescriber specialty: Must be prescribed by or in consultation with a lipid specialist or cardiologist
Required Prior Lipid‑Lowering Therapy
Before initiating Evkeeza, members must have documented trials of intensive lipid-lowering therapy unless an exception applies. Trials must be for at least 8 weeks.
- High-intensity or max-tolerated statin in combination with ezetimibe (unless documented contraindication to all statins or intolerance to at least 2 different statins, including low/moderate intensity or alternate dosing) — 8-week trial required
- PCSK9 inhibitor (e.g., evolocumab or alirocumab) — 8-week trial required unless there is evidence of no LDL receptor function (receptor‑negative HoFH) or the member does not meet the labeled age for PCSK9 inhibitors
- Evkeeza is used as an adjunct to other lipid-lowering treatments (statin, ezetimibe, PCSK9 inhibitor, LDL apheresis) unless contraindicated or intolerant
Required Documentation
Document the clinical information that supports the diagnosis and prior therapy requirements. Chart notes must include baseline laboratory and diagnostic evidence and attestations.
- Baseline LDL‑C documented within the past 90 days and above goal (e.g., adults <70 mg/dL or <55 mg/dL with additional ASCVD risk; children <115 mg/dL considered goal thresholds)
- Genetic testing confirming two mutant alleles in LDLR, APOB, PCSK9, or LDLRAP1 OR diagnostic evidence of HoFH (LDL‑C >400 mg/dL pre‑treatment plus clinical findings such as cutaneous/tendon xanthoma before age 10 or evidence of heterozygous FH in both parents)
- Documentation of 8‑week trials of required lipid‑lowering therapies or documented contraindications/intolerance as noted
- Prescriber specialty or documented consultation with a lipid specialist or cardiologist
- Prescriber attestation that the member will adhere to a low‑fat diet and exercise regimen
- Dosage/quantity: 15 mg/kg IV infusion once monthly
Triggers for Denial
Requests that do not meet the policy requirements are at risk for denial.
- Member does not meet HoFH criteria (no genetic confirmation or insufficient diagnostic evidence)
- Age <1 year
- Prescriber is not a lipid specialist or cardiologist and no documented consultation exists
- Missing baseline LDL‑C within the past 90 days or baseline LDL‑C not above goal
- Missing documentation of 8‑week trials of statin+ezetimibe and PCSK9 inhibitor (or appropriate documented exceptions)
- Concomitant initiation with Juxtapid at the same time may be a basis for clarification — policy specifies Evkeeza is not being concomitantly initiated with Juxtapid
Initial Approval Criteria
Initial therapy
Covered when ALL of the following are met:
If all requirements met, approve for 6 months (initial).
Reauthorization / Continuation Requirements
Reauthorization requirement
Reauthorization requires demonstrated LDL‑C reduction.
If met, approve for an additional 12 months.
Step Therapy / Prior Treatment Requirements
| Step | Required trial | Duration | Exceptions |
|---|---|---|---|
| 1 | High-intensity or max-tolerated statin in combination with ezetimibe | 8 weeks | Documented statin intolerance or contraindication |
| 2 | PCSK9 inhibitor (e.g., Repatha or Praluent) | 8 weeks | Receptor‑negative HoFH or member below labeled age for PCSK9 inhibitor |
Dosing and Quantity Limits
Site of Care and Administration
Infusion center (medical benefit) — site‑of‑care note
Evkeeza is administered as an IV infusion under the medical benefit and should be delivered in an appropriate infusion setting; site‑of‑care and billing should reflect an IV infusion (15 mg/kg once monthly).
- Dose/quantity limit: 15 mg/kg IV once monthly.
- Document infusion center or other medical infusion site on the claim/authorization.
Definitions and Diagnostic Criteria
Background
Evkeeza (evinacumab‑dgnb) is an angiopoietin‑like 3 (ANGPTL3) inhibitor approved as an adjunct to diet, exercise, and other LDL‑C–lowering therapies to reduce LDL‑C in patients with homozygous familial hypercholesterolemia (HoFH). The agent lowers LDL, HDL, and triglycerides and is the first therapy in its class; within this policy Evkeeza is covered only as adjunctive therapy for HoFH when the documented clinical criteria are met.
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