Treatment of Cardiomyopathy of Wild-Type or Hereditary Transthyretin-Mediated Amyloidosis

POLICY NUMBER

L.5.01.542

Amvuttra (vutrisiran)

Attruby (acoramidis)

Vyndaqel (tafamidis meglumine)

Vyndamax (tafamidis)

Please perform a formulary drug search on your patient’s member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific member’s benefit plan.

DESCRIPTION

Hereditary transthyretin-mediated amyloidosis (hATTR) is a rare, autosomal dominant, progressive, and fatal multi-system illness characterized by the extracellular deposition of misfolded transthyretin (TTR) protein. Though the rate at which hATTR amyloidosis progresses is unpredictable, these deposits cause significant neurological problems, functional limitations, and disability over time. These presentations include a predominantly neurologic phenotype, formerly known as familial amyloid polyneuropathy (FAP), and a predominantly cardiac phenotype, formerly known as familial cardiomyopathy, but the majority of cases express both neurologic and cardiac manifestations.

Vyndamax (tafamidis) and Vyndaqel (tafamidis meglumine), selective TTR stabilizers, are indicated for the treatment of the cardiomyopathy of wild type or hereditary transthyretin-mediated amyloidosis (ATTR-CM) in adults to reduce cardiovascular mortality and cardiovascular-related hospitalization.

Amvuttra (vutrisiran) is indicated for the treatment of ATTR-CM in adults to reduce cardiovascular mortality, cardiovascular hospitalizations and urgent heart failure visits and for the treatment of the polyneuropathy of hereditary transthyretin-mediated amyloidosis (hATTR-PN) in adults.

Attruby (acoramidis) is indicated for the treatment of ATTR-CM in adults to reduce cardiovascular death and cardiovascular-related hospitalization. 

Related medical policies - 

• Treatment of Polyneuropathy of Hereditary Transthyretin-Mediated Amyloidosis

POLICY

Prior authorization is required.

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements.

Initial Evaluation

Vyndaqel or Vyndamax may be considered medically necessary when ALL of the following are met:

1. Individual is ≥18 years of age;

2. Individual has a documented diagnosis of cardiomyopathy of wild type or hereditary transthyretin-mediated amyloidosis as confirmed by bone tracer cardiac scintigraphy or cardiac biopsy;

3. Medication is being prescribed by, or in consultation with, a cardiologist;

4. Individual is not taking and will not start another tafamidis agent while on therapy with the requested agent;

5. Individual does not have any FDA-labeled contraindication(s) to therapy with the requested medication; AND

6. The prescribed dosage is within the program quantity limits based on the individual’s age and FDA-approved labeled dosage.

Length of Approval: 12 months

Renewal Evaluation

Vyndaqel or Vyndamax may be approved for renewal when ALL of the following are met:

1. Individual has been previously approved for therapy with the requested agent through the BCBSMS PA process;

2. Individual has shown clinical benefit while using the requested agent;

3. Medication is being prescribed by, or in consultation with, a cardiologist;

4. Individual is not taking and will not start another tafamidis agent while on therapy with approved agent;

5. Individual does not have any FDA-labeled contraindication(s) to therapy with the requested medication; AND

6. The prescribed dosage is within the program quantity limits based on the individual’s age and FDA-approved labeled dosage.

Length of Approval: 12 months

Amvuttra and Attruby are considered not medically necessary as there are other formulary alternatives covered by the Plan for the treatment of ATTR-CM.

Services related to delivery and/or administration of a medication which have not been approved through the BCBSMS PA review process will be considered not medically necessary.

POLICY EXCEPTIONS

State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

B. appropriate with regard to standards of good medical practice; and

C. not solely for the convenience of the Member, his or her Provider; and

D. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

BCBSMS may request medical records for determination of medical necessity. When medical records are requested, letters of support and/or explanation are often useful, but are not sufficient documentation unless all specific information needed to make a medical necessity determination is included.

POLICY HISTORY

02/06/2020: New policy added.

07/13/2021: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee. Policy section updated to revise initial and renewal evaluation criteria. Length of approval for renewal is 12 months. Added statement that services related to delivery and/or administration of a medication which have not been approved through the BCBSMS PA review process will be considered not medically necessary. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity." Added information regarding BCBSMS request for medical records. Sources updated.

02/01/2023: Policy Exceptions updated to add the following: State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

06/03/2025: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy title updated to make the following correction: "meglumina" changed to "meglumine." Policy description updated. Policy statement revised to state that the use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Medically necessary and approval criteria updated to revise statement regarding dose requirements. Sources updated.

12/02/2025: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy title changed from "Vyndaqel (tafamidis meglumine) / Vyndamax (tafamidis)" to "Treatment of Cardiomyopathy of Wild-Type or Hereditary Transthyretin-Mediated Amyloidosis." Policy description updated to add indications for Amvuttra (vutrisiran) and Attruby (acoramidis). Added related medical policy. Added policy statement that Amvuttra and Attruby are considered not medically necessary as there are other formulary alternatives covered by the Plan for the treatment of ATTR-CM. Sources updated. Code Reference section updated to add HCPCS code J0225.

SOURCE(S)

1. Amvuttra prescribing information. Alnylam Pharmaceuticals, Inc. March 2025. Last accessed September 2025.

2. Attruby prescribing information. BridgeBio Pharma, Inc. August 2025. Last accessed September 2025.

3. Kittleson, M, Ruberg, F, Ambardekar, A. et al. 2023 ACC Expert Consensus Decision Pathway on Comprehensive Multidisciplinary Care for the Patient With Cardiac Amyloidosis: A Report of the American College of Cardiology Solution Set Oversight Committee. JACC. 2023 Mar, 81 (11) 1076–1126.https://doi.org/10.1016/j.jacc.2022.11.022

4. Vyndaqel and Vyndamax prescribing information. Pfizer Laboratories Div Pfizer Inc. January 2025. Last accessed September 2025.

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

Not Medically Necessary Codes

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