CurrentCignaPolicy IP0084

Metabolic Disorders - Dojolvi

Cigna coverage policy for Dojolvi (triheptanoin oral liquid) specifying prior authorization requirements, documentation, clinical criteria for FDA‑approved indication (long-chain fatty acid oxidation disorders), duration of approval, and exclusions for non‑FDA uses.

Policy Summary

PayerCigna

PolicyMetabolic Disorders - Dojolvi

Policy CodePolicy IP0084

Change TypeRevised (annual revisions)

Effective Date

Next Review Date

Key ActionSubmit prior authorization with documentation of molecular confirmation (at least two of: disease-specific acylcarnitine elevations, enzyme activity assay below reference range, and/or genetic testing showing a pathogenic variant); prescription must be by or in consultation with a metabolic disease specialist.

SourceLink

POLICY UPDATE CHANGES

Policy title updated from 'Triheptanoin' to 'Metabolic Disorders - Dojolvi' (Annual Revision 11/01/2024 and 10/01/2025 entries).

1FDA‑approved indication covered

1Approval duration (years)

2Diagnostic criteria required (of 3)

Coverage Summary

Coverage stance: covered_with_criteria. Subject: Dojolvi (triheptanoin) for long-chain fatty acid oxidation disorders. Status: CURRENT. Scope: Cigna prior authorization policy for Dojolvi specifying clinical criteria, required documentation, approval duration, and exclusions for non‑FDA uses. Approvals are provided for 1 year. Diagnostic confirmation requires at least 2 of the following: disease‑specific acylcarnitine elevations on newborn blood spot or plasma, enzyme activity assay below the laboratory reference range, or genetic testing demonstrating a pathogenic variant.

Provider Actions & Operational Notes

Prior Authorization

Prior authorization required

Prior authorization is required for Dojolvi. Approvals are provided for 1 year. The medication must be prescribed by or in consultation with a metabolic disease specialist or a physician who specializes in management of long‑chain fatty acid oxidation disorders.

Documentation Required

Documentation of diagnostic evidence

Documentation supporting molecular confirmation is required. Provide at least two of the following diagnostic elements: disease‑specific elevations of acylcarnitines (newborn blood spot or plasma), an enzyme activity assay in cultured fibroblasts or lymphocytes below the reporting laboratory's normal reference range, and/or genetic testing demonstrating a pathogenic variant in a gene associated with long‑chain fatty acid oxidation disorders. Documentation may include chart notes, laboratory tests, and claims records.

Disease‑specific elevations of acylcarnitines (newborn blood spot or plasma)
Enzyme activity assay in cultured fibroblasts or lymphocytes below the laboratory reference range (examples: CPT‑2, VLCAD, LCHAD, TFP)
Genetic testing demonstrating a pathogenic variant in an associated gene (examples: CPT2, ACADVL, HADHA, HADHB)

Documentation Required

Concomitant MCT products prohibited

Patients must discontinue any other medium‑chain triglyceride (MCT) products prior to the first dose of Dojolvi; concomitant use of other MCT products with Dojolvi is not allowed.

Billing Rule

Sample product exclusion

Receipt of a sample product does not satisfy coverage criteria and cannot be used to justify coverage.

Denial Risk

Use for non‑FDA indications

Dojolvi is considered not medically necessary for any use other than long‑chain fatty acid oxidation disorders; claims for other indications will be denied.

Background

Background: Dojolvi (triheptanoin) is indicated as a source of calories and fatty acids for molecularly confirmed long‑chain fatty acid oxidation disorders (LC‑FAODs). LC‑FAODs are autosomal recessive metabolic disorders in which the body cannot properly oxidize long‑chain fatty acids; commonly affected enzymes include CPT‑2, VLCAD, LCHAD, and TFP. Onset ranges from neonatal period to adulthood and clinical presentations are heterogeneous. Diagnosis may begin with newborn screening acylcarnitine measurements in dried blood spots and, when abnormal or symptomatic, further evaluation with plasma acylcarnitine testing, enzyme activity assays (in cultured fibroblasts or lymphocytes), and/or genetic testing to identify causative variants. Documentation required: documentation may include chart notes, laboratory tests, claims records, and/or other information to support the diagnostic criteria.

Revision History

2024-11-01annual_revision

Policy title updated from 'Triheptanoin' to 'Metabolic Disorders - Dojolvi'. For genetic testing diagnosis, language rephrased from 'mutation' to 'variant'. Added 'according to the prescriber' wording in criteria regarding inadequate efficacy or intolerance to over-the-counter medium-chain triglyceride products and history of severe/recurrent LC-FAOD manifestations.

2025-10-01annual_revisionLatest

Annual revision added a policy statement, added documentation requirements, and updated the conditions not covered statement; continued use of updated title 'Metabolic Disorders - Dojolvi' and maintained revised genetic testing and prescriber language.

Policy Summary

PayerCigna

PolicyMetabolic Disorders - Dojolvi

Policy CodePolicy IP0084

Change TypeRevised (annual revisions)

Effective Date

Next Review Date

SourceLink

On This Page

OpenPayer

Metabolic Disorders - Dojolvi

Coverage Summary

Provider Actions & Operational Notes

Conditions Not Covered

Applicable Codes & Policy Identifier

Background

Clinical Evidence

Definitions

Revision History