Evinacumab-dgnb (Evkeeza^TM) is an angiopoietin-like protein 3 (ANGPTL3) inhibitor. ANGPTL3 is expressed primarily in the liver and plays a role in the regulation of lipid metabolism by inhibiting lipoprotein lipase and endothelial lipase. Inhibition of ANGPTL3 leads to a reduction in LDL-C, HDL-C and triglycerides. 

Preferred Products

Proprotein convertase substilisin kexin 9 (PCSK9) inhibitors are the preferred products required for members initiating new therapy for homozygous familial hypercholesterolemia. The non-preferred product [evinacumab-dgnb (Evkeeza)] will be considered when the member has a documented therapy failure after an adequate therapeutic trial of a preferred product, the preferred product has not been tolerated or is contraindicated or the individual does not meet minimum age requirements based on FDA approval for the preferred products.

Adequate therapeutic trial is defined as three (3) months following the injection series at Food and Drug Administration (FDA) or compendia based therapeutic doses of preferred product.

New therapy is defined as no previous utilization within the last 365 calendar days.

Evinacumab-dgnb (Evkeeza) may be considered medically necessary when the following criteria are met:

• Individual is five (5) years of age or older; and
• Evinacumab-dgnb (Evkeeza) will be prescribed by or in consultation with a cardiologist, endocrinologist, or lipid specialist; and 
• The individual will continue on current lipid lowering treatment regimen in combination with evinacumab-dgnb (Evkeeza) based on age-appropriate FDA approved treatments; and
• There is genetic confirmation or clinical documentation of homozygous familial hypercholesterolemia (see table 1 below); and
• Individual has failure of or intolerance to statin therapy (see table 2 below); and
  • Note: statin therapy is not FDA approved in individuals younger then seven (7) years old
• Individual has failure of proprotein convertase substilisin kexin 9 (PCSK9) inhibitor (e.g., alirocumab or evolocumab based upon FDA approval for age) for at least three (3) months (see table 3 below); and
• Initial authorization will be for 12 months.

Reauthorization Criteria

• Documentation of LDL-C reduction from baseline; and
• Reauthorization will be for a period of 12 months. 

Table 1

Table 2

Table 3

Evinacumab-dgnb (Evkeeza) for any other indication is considered not medically necessary.

Evinacumab-dgnb (Evkeeza) may be considered medically necessary for individuals 18 years of age and older when applicable clinical criteria for individual medication policies are met and when administered in a physician’s office not affiliated with a hospital, specialized infusion centers not affiliated with a hospital or in the home.

Outpatient facility (Outpatient Hospital IV Infusion Department or Hospital-based Outpatient Clinical Level of Care) administration may be considered medically necessary if ANY of the following criteria are present to indicate the member is medically unstable for infusions in other than an outpatient facility setting:

• Member’s home is considered unsuitable for care by the home infusion provider; or
• Individual’s medical status requires enhanced monitoring beyond that which would routinely be needed for infusion therapy; or
• Previous severe adverse reaction (including but not limited to anaphylaxis, seizure, thromboembolism, myocardial infarction, renal failure) during or following administration of prescribed medication despite standard pre-medication; or
• Individual is receiving other medications that require close monitoring with a higher level of care (e.g., cytotoxic chemotherapy or blood products); or
• Individual is at high risk for complications due to medication administration (e.g., at risk for post-transplant complications, increased risk of infusion reactions due to presence of circulating antibodies, unstable vascular access, cardiopulmonary condition at risk for severe adverse reactions, unstable renal function with inability to safely tolerate IV volume loads, etc.); or 
• Individual is initiating therapy or re-initiating therapy after a period of at least 6 months with no therapy; or
• Physically and/or cognitively impaired AND a home caregiver is not available to comply with the required treatment regimen and schedule.

Home health services may be considered medically necessary when utilized for the administration of home infusion therapy and when provided by licensed eligible provider. Each case will be addressed on an individual basis.

The medications identified in this policy will be considered not medically necessary if administered in an unapproved hospital outpatient setting when an approved site of care is a viable option for treatment.