CurrentCignaPolicy N/A

Barth Syndrome - Forzinity

Defines Cigna's coverage rules and prior authorization requirements for Forzinity (elamipretide) when used to treat Barth syndrome for members of Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyBarth Syndrome - Forzinity

Policy CodePolicy N/A

Change TypeNew policytitle revision

Effective DateApr 1, 2026

Next Review DateFeb 5, 2026

Key ActionObtain prior authorization and document diagnostic confirmation (MLCL/CL ratio, urine organic acids, or pathogenic TAZ variant) with prescribing by or in consultation with a relevant specialist.

SourceLink

POLICY UPDATE CHANGES

New policy established describing coverage and criteria for Forzinity for Barth syndrome.

Policy title updated to 'Barth Syndrome - Forzinity'.

1 yearapproval duration

>=30 kgminimum weight

<250documented patients

168 wksefficacy follow-up

Coverage Criteria for Forzinity (elamipretide)

Policy Summary

PayerCigna

PolicyBarth Syndrome - Forzinity

Policy CodePolicy N/A

Change TypeNew policytitle revision

Effective DateApr 1, 2026

Next Review DateFeb 5, 2026

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.

FDA-Approved Indication

Approve for 1 year if the patient meets ONE of the following (A or B):

A or B: A) Initial Therapy OR B) Currently Receiving Forzinity

A) Initial Therapy: Approve if ALL of the following are met

A.i-iii: i. Patient weighs > 30 kg; AND ii. Diagnosis established by ONE of (a, b, or c); AND iii. Medication prescribed by or in consultation with a listed specialist

A.ii (diagnostic confirmation): a) Patient has a laboratory test demonstrating an increased ratio of monolysocardiolipin (MLCL)/cardiolipin (CL) on erythrocytes, tissue, fibroblasts, or stored neonatal bloodspots [documentation required]; OR b) Patient has a laboratory test demonstrating elevated 3-methylglutaric acid, 3-methylglutaconic acid (3-MGC), and 2-ethylhydracrylic acid on urine organic acids analysis [documentation required]; OR c) Patient has a molecular genetic test demonstrating a hemizygous pathogenic variant in the tafazzin (TAZ) gene [documentation required]

B) Currently Receiving Forzinity: Approve if BOTH are met: i. Patient has been established on therapy for at least 1 year; AND ii. According to the prescriber, the patient has demonstrated a clinical response defined as stabilization or lack of decline from baseline prior to initiating Forzinity

Examples of clinical response include stabilization or lack of decline in muscle strength, balance, six-minute walking distance, or fatigue.

Forzinity (elamipretide) for any indication other than the FDA-approved indication for Barth syndrome is considered not medically necessary. Criteria will be updated as new published data become available.

Use of Forzinity outside the FDA-approved indication for Barth syndrome (as specified in this policy) is considered not medically necessary.

Prescriber & Prior Authorization Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Forzinity. All approvals are provided for the duration noted (1 year). Because of the specialized skills required for evaluation, diagnosis, and monitoring of patients treated with Forzinity, the medication must be prescribed by or in consultation with a physician who specializes in the condition being treated (e.g., geneticist, cardiologist, metabolic specialist, hematologist, pediatrician, or physician specializing in mitochondrial disorders).

Approval duration: 1 year
Prescriber requirement: specialist or in consultation with a specialist

Denial Risk

Claims and Billing Note

Claims submitted without a covered diagnosis or appropriate procedure codes will be denied as not covered. Coverage is governed by the terms of the member’s applicable benefit plan document; where conflicts exist, the plan document controls.

Claims lacking covered diagnosis/procedure codes → deny as not covered
Use most appropriate codes as of the service submission date
Benefits determined by applicable benefit plan document

Documentation Required

Documentation Required for Diagnostic Confirmation and Review

Documentation is required for diagnostic confirmation and for review of continued therapy. Acceptable documentation may include chart notes, laboratory tests, genetic test results, claims records, and other relevant clinical information. All documentation must include patient-specific identifying information.

Required diagnostic documentation examples: MLCL/CL ratio on erythrocytes/tissue/fibroblasts/neonatal bloodspots; urine organic acids showing elevated 3-MGC and related metabolites; molecular genetic test showing hemizygous pathogenic TAZ variant
Documentation may include chart notes, laboratory tests, claims records, and other clinical information
All documentation must include patient identifiers

Billing Rule

PROVIDER ACTIONS

Providers must ensure that prior authorization requests include the required documentation and that claims are submitted with covered diagnosis and procedure codes consistent with the coverage criteria. Failure to include appropriate supporting records or covered codes may result in denial.

Include documentation supporting diagnostic criteria and clinical response for continuation requests
Ensure claims include covered diagnosis/procedure codes that match the approved indication and documentation

Background on Barth Syndrome and Forzinity

Barth syndrome is an ultra-rare, X-linked mitochondrial disorder caused by pathogenic variants in the TAZ gene that disrupt cardiolipin remodeling and lead to abnormal cardiolipin composition. Clinical features commonly include cardiomyopathy, skeletal myopathy with reduced muscle strength, neutropenia, growth delay, and recurrent infections; globally there are fewer than 250 documented diagnosed patients. Forzinity (elamipretide) received accelerated approval to improve muscle strength in pediatric and adult patients with Barth syndrome who weigh at least 30 kg, based on demonstrated improvement in knee extensor strength in clinical study follow-up (open-label data up to 168 weeks in available reports). Prior authorization is required and approvals are provided for 1 year when the coverage criteria are met.

Key Definitions

inv-11: MLCL/CL ratio

DefinitionThe MLCL/CL ratio is the biochemical measure of monolysocardiolipin (MLCL) relative to cardiolipin (CL) in bloodspots, cells, or tissues used as a diagnostic marker for Barth syndrome

Diagnostic roleAn increased MLCL/CL ratio on erythrocytes, tissue, fibroblasts, or stored neonatal bloodspots can establish the diagnosis (documentation required)

Biologic basisTAZ gene mutations cause accumulation of MLCL and deficiency of remodeled CL, producing an abnormal MLCL/CL ratio

inv-12: TAZ gene

Gene identityTAZ encodes tafazzin, the enzyme required for cardiolipin remodeling

Pathogenic effectPathogenic hemizygous variants in TAZ disrupt production of mature cardiolipin, leading to MLCL accumulation and mitochondrial dysfunction

Clinical relevanceTAZ pathogenic variants can be used to confirm Barth syndrome by molecular genetic testing (documentation required)

OpenPayer

Barth Syndrome - Forzinity

Coverage Criteria for Forzinity (elamipretide)

Coding and Eligibility Metrics

Prescriber & Prior Authorization Requirements

Background on Barth Syndrome and Forzinity

Key Definitions