CurrentCignaPolicy N/A

Forzinity (elamipretide) for Barth syndrome — Coverage Criteria

This policy governs prior authorization and medical necessity criteria for coverage of Forzinity (elamipretide subcutaneous injection) for treatment of Barth syndrome under Cigna-administered health benefit plans; it affects prescribers and patients seeking coverage for this therapy.

Policy Summary

PayerCigna

PolicyForzinity (elamipretide) for Barth syndrome — Coverage Criteria

Policy CodePolicy N/A

Change TypeNew Policy

Effective DateN/A

Next Review DateDec 9, 2025

Key ActionObtain prior authorization with documentation of diagnostic confirmation and weight > 30 kg; approvals are for 1 year.

SourceLink

POLICY UPDATE CHANGES

New policy establishing coverage and prior authorization criteria for Forzinity (elamipretide) for Barth syndrome.

>30 kgminimum patient weight for eligibility

1 yrapproval duration for coverage

<250diagnosed Barth syndrome patients (approx.)

Week 168efficacy timepoint reported

Coverage and Medical Necessity Criteria

FDA-Approved Indication — Barth Syndrome

Forzinity is covered as medically necessary for Barth syndrome when the following conditions are met for the FDA‑approved indication. Approvals are for 1 year.

Main approval paths: Coverage is granted if ONE of the following (A or B) is met: A) Initial Therapy OR B) Currently Receiving Forzinity.

A) Initial Therapy: Approve if ALL of the following (i, ii, and iii) are met:

A.i: The patient weighs > 30 kg.> 30 kg

A.ii Diagnostic confirmation:

Policy Summary

PayerCigna

PolicyForzinity (elamipretide) for Barth syndrome — Coverage Criteria

Policy CodePolicy N/A

Change TypeNew Policy

Effective DateN/A

Next Review DateDec 9, 2025

Key ActionObtain prior authorization with documentation of diagnostic confirmation and weight > 30 kg; approvals are for 1 year.

SourceLink

On This Page

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Prior Authorization, Documentation, and Billing Guidance

Prior Authorization

Prior Authorization Required

Prior authorization is recommended for prescription benefit coverage of Forzinity. Approvals are provided for 1 year. Because of the specialized skills required for evaluation and monitoring, Forzinity must be prescribed by or in consultation with a physician who specializes in the condition being treated (examples: geneticist, cardiologist, metabolic specialist, hematologist, pediatrician, or a physician who specializes in mitochondrial disorders).

Prior authorization recommended — approvals valid for 1 year.
Prescriber requirement: must be prescribed by or in consultation with a specialist (geneticist, cardiologist, metabolic specialist, hematologist, pediatrician, or mitochondrial disorder specialist).
When in doubt, obtain specialist consultation documentation with the prior authorization request.

Note

Provider action: include specialty and consultation details with the prior authorization request. If the prescriber is not a listed specialist, document consultation with an appropriate specialist in the medical record and include that documentation with the request.

Document specialist consultation if the prescribing clinician is not one of the listed specialties.
Include dates and names of consulting specialists in the prior authorization submission.

Documentation Required

Documentation Required for Prior Authorization

Required documentation must be submitted with the prior authorization to support medical necessity and continuation requests.

Prescriber specialty and/or consultation note from a listed specialist.
Supporting clinical documentation establishing diagnosis (see diagnostic criteria below).
Documentation of diagnostic testing: MLCL/CL ratio result, urine organic acids with elevated 3-MGC/related metabolites, or molecular genetic test showing a hemizygous pathogenic TAZ variant.
For continuation requests: documentation patient has been on therapy ≥ 1 year and evidence of clinical response (stabilization or lack of decline from baseline in muscle strength, balance, 6-minute walk distance, or fatigue).
If patient has received < 1 year of therapy or is restarting, evaluate under Initial Therapy criteria and provide baseline measures.

Denial Risk

Coding and Claim Submission — Denial Risk

Claims will be denied if submitted without covered diagnosis or procedure codes that align with the policy. Use the most appropriate codes as of the date of submission and ensure the diagnosis supports the FDA-approved indication (Barth syndrome) or other covered use per the policy.

Denial risk if claim lacks covered diagnosis (Barth syndrome) or appropriate supporting documentation.
Ensure claims include corresponding documentation (diagnostic test results, genetic test, or specialist notes) to support the billed diagnosis.
Verify coding and modifier use prior to submission to prevent administrative denials.

Clinical Background

Barth syndrome is an ultra‑rare, X‑linked, infantile‑onset mitochondrial disorder caused by pathogenic variants in the TAZ gene that disrupt cardiolipin metabolism. Clinically it presents with cardiomyopathy, skeletal muscle weakness and hypotonia, growth delay, and neutropenia leading to recurrent infections, together with characteristic biochemical abnormalities such as increased monolysocardiolipin (MLCL) and an abnormal MLCL/cardiolipin (CL) ratio.

Forzinity (elamipretide) is a mitochondrial protective agent approved under accelerated approval to improve muscle strength in adult and pediatric patients with Barth syndrome who meet the labeled eligibility criteria (including a minimum weight threshold). Continued approval may depend on confirmatory clinical trials.

Key Definitions

inv-10: Definition of Barth syndrome

Inheritance and onsetX-linked, typically infantile-onset

Core clinical featuresCardiomyopathy, hypotonia (muscle weakness), growth delay, and neutropenia leading to increased infections

Biochemical abnormalitiesIncreased MLCL, decreased remodeled cardiolipin (CL), and abnormal MLCL/CL ratio; often increased 3-methylglutaconic and 3-methylglutaric acids on urine organic acids

Genetic causeMutations in the TAZ (tafazzin) gene

inv-11: Definition of Forzinity (elamipretide)

DrugForzinity (elamipretide subcutaneous injection)

Mechanism / classMitochondrial protective agent

FDA approval status and indicationApproved under accelerated approval to improve muscle strength in adult and pediatric patients with Barth syndrome

Weight eligibility thresholdPatient must weigh > 30 kg

OpenPayer

Forzinity (elamipretide) for Barth syndrome — Coverage Criteria

Coverage and Medical Necessity Criteria

Coding and Eligibility Thresholds

Prior Authorization, Documentation, and Billing Guidance

Clinical Background

Key Definitions

Policy Revision History