CurrentEmblemHealthPolicy MG.MM.PH.343

Nexviazyme (avalglucosidase alfa-ngpt) intravenous infusion

Clinical coverage and authorization criteria for Nexviazyme (avalglucosidase alfa-ngpt) intravenous infusion for treatment of late‑onset Pompe disease for EmblemHealth members and contracted plans.

Policy Summary

PayerEmblemHealth

PolicyNexviazyme (avalglucosidase alfa-ngpt) intravenous infusion

Policy CodePolicy MG.MM.PH.343

Change TypeMaterial revision — diagnostic and renewal criteria updated

Effective Date

Next Review DateMar 17, 2025

Key ActionTreating physician must submit clinical evidence including laboratory or molecular genetic testing and baseline percent predicted FVC and/or 6MWT for prior authorization.

SourceLink

POLICY UPDATE CHANGES

Initial criteria revised to require demonstration of biallelic pathogenic or likely pathogenic GAA variants (molecular genetic test) as diagnostic option and added requirement for documented baseline FVC and/or 6MWT.

Renewal criteria added requiring continued benefit (stabilization or improvement in FVC and/or 6MWT), monitoring for antibody formation, and absence of unacceptable toxicity.

Billing/coding updated to specify HCPCS J0219 and NDC 58468-0426-01 and removed prior codes C9085 and J3590.

12 monthsLength of authorization

Up to 40 mg/kgDose limit every 2 weeks

575

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Coverage and Medical Necessity Criteria

Initial Therapy

Covered when ALL of the following are met

Initial Approval Criteria: Patient is > 1 year of age; AND patient has late-onset acid alpha-glucosidase deficiency (late-onset Pompe disease); AND patient has laboratory evidence of deficient acid alpha-glucosidase activity in fibroblasts or muscle tissue OR the diagnosis is established by molecular genetic testing demonstrating biallelic pathogenic or likely pathogenic GAA variants; AND patient has documented baseline percent predicted forced vital capacity (FVC) and/or 6-minute walk test (6MWT); AND medication is prescribed by or in consultation with a geneticist, neurologist, metabolic disorder subspecialist, or a physician who specializes in lysosomal storage disorders; AND will not be used in combination with other enzyme replacement therapies.

Age, diagnostic evidence, baseline functional assessment, specialist involvement, and no concurrent ERT are required.

Renewal Therapy

Renewal/Continuation Therapy — Coverage can be renewed when ALL of the following are met

Renewal Criteria: Patient continues to meet identified Initial criteria; AND absence of unacceptable toxicity from the drug (examples include anaphylaxis, severe hypersensitivity reactions, severe infusion-associated reactions, acute cardiorespiratory failure); AND patient has demonstrated beneficial response to therapy compared to pretreatment baseline in one or more of: stabilization or improvement in FVC and/or 6-MWT; AND patient is monitored for antibody formation including neutralizing antibodies.

Renewal requires documented clinical benefit and safety/antibody monitoring.

Coverage is limited to the circumstances and clinical criteria explicitly listed in this policy. Coverage is not recommended for circumstances not listed in the guideline; the criteria will be updated as new published data become available. Requests for use outside these criteria should include supporting clinical evidence and may not be approved.

Nexviazyme (avalglucosidase alfa‑ngpt) is not to be used in combination with other enzyme replacement therapies. Examples of prohibited concomitant therapies include alglucosidase alfa and cipaglucosidase alfa. Requests for concurrent administration with another ERT will be considered not medically necessary.

Applicable Procedure Codes, NDCs, Diagnoses, and Dosing Limits

Applicable Procedure CodesHCPCSCovered

J0219

Injection, avalglucosidase alfa-ngpt, 4 mg

Applicable NDCsNDCCovered

58468-0426-01

Solution reconstituted 100 mg per single dose vial

ICD-10 DiagnosesICD-10

E74.02

Pompe disease

Dosing limits

Dose limitUp to 40 mg/kg of actual body weight every 2 weeks

Max billable units per 14 days575 billable units (equivalent to 2300 mg) every 14 days

Authorization length related noteAuthorization decisions reference dosing every 2 weeks and the 14‑day HCPCS unit maximum

Prior Authorization and Documentation Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for coverage of this therapy. The treating physician or primary care provider must submit clinical evidence demonstrating that the member meets the policy criteria before services are provided. Without required documentation EmblemHealth cannot properly review the request for preauthorization or post-payment review.

Submit detailed clinical documentation including diagnosis, relevant laboratory and genetic test results, baseline functional measures (percent predicted FVC and/or 6-minute walk test), treating or consulting specialist information, and proposed drug regimen.
Requests must indicate prescriber specialty (geneticist, neurologist, metabolic disorders subspecialist, or physician experienced in lysosomal storage disorders).

Denial Risk

Concomitant Enzyme Replacement Therapy Prohibited

Cipaglucosidase alfa (or other listed enzyme replacement therapies) must not be used concurrently with any other enzyme replacement therapy. Requests that indicate concomitant use of multiple ERTs will be denied.

Background and Rationale

Nexviazyme (avalglucosidase alfa‑ngpt) is a recombinant human acid alpha‑glucosidase enzyme replacement therapy indicated for patients with late‑onset Pompe disease (acid alpha‑glucosidase deficiency). The policy requires baseline functional assessment with percent predicted forced vital capacity (FVC) and/or a 6‑minute walk test (6MWT) to document pretreatment status and assess response. Molecular genetic testing demonstrating biallelic pathogenic or likely pathogenic GAA variants or laboratory demonstration of deficient acid alpha‑glucosidase activity can be used to establish the diagnosis.

Drug Definition

Nexviazyme (avalglucosidase alfa-ngpt)

Generic name / trade nameavalglucosidase alfa‑ngpt (Nexviazyme)

Mechanism / classRecombinant human acid alpha‑glucosidase enzyme (hydrolytic lysosomal glycogen‑specific)

IndicationIndicated for patients > 1 year of age with late‑onset Pompe disease (lysosomal acid alpha‑glucosidase deficiency)