CurrentUnitedHealthcarePolicy 1 = 2025 P 2221-6

Dojolvi (triheptanoin) prior authorization / medical necessity

Defines UnitedHealthcare prior authorization and medical necessity criteria for Dojolvi (triheptanoin) for treatment of long-chain fatty acid oxidation disorders (LC-FAOD) for pediatric and adult members.

Policy Summary

PayerUnitedHealthcare

PolicyDojolvi (triheptanoin) prior authorization / medical necessity

Policy CodePolicy 1 = 2025 P 2221-6

Change TypeNo material changes

Effective Date8/1/2025

Next Review Date

Key ActionSubmit prior authorization with medical records confirming LC-FAOD diagnosis (at least two diagnostic criteria) and dosing at or below 35% DCI.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsauthorization duration

35%max dosage % DCI

10/2020–5/2025P&T history

pediatric & adultindicated populations

Coverage and Medical Necessity Criteria

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Initial Authorization - Diagnostic confirmation: Submission of medical records confirming the diagnosis of long-chain fatty acid oxidation disorders (LC-FAOD) with at least two of the following diagnostic criteria: (1) disease-specific elevation of acylcarnitines on a newborn blood spot or in plasma; (2) low enzyme activity in cultured fibroblasts; (3) genetic testing demonstrating one or more pathogenic mutations in a gene associated with LC-FAOD (e.g., CPT2, ACADVL, HADHA, or HADHB).

Initial Authorization - Concomitant therapy: Patient is not receiving Dojolvi in combination with any other medium-chain triglyceride (MCT) products.

Initial Authorization - Prescriber: Prescribed by a board-certified medical geneticist experienced in the treatment of long-chain fatty acid oxidation disorders (LC-FAOD).

Initial Authorization - Dosing: Target recommended daily dosage does not exceed 35% of the patient's total prescribed daily caloric intake (DCI).<=35% DCI

Initial Authorization - Dietary management: Patient is receiving disease-related dietary management.

Initial Authorization - Clinical presentation: If not diagnosed by newborn screening, patient has a history of clinical manifestations of LC-FAOD (e.g., rhabdomyolysis).

Reauthorization

Covered when ALL of the following are met for reauthorization:

Reauthorization - Clinical response: Documentation of positive clinical response to Dojolvi therapy (e.g., increased cardiac efficiency, decreased left ventricular wall mass, decreased incidence of rhabdomyolysis, etc.).

Reauthorization - Concomitant therapy: Patient is not receiving Dojolvi in combination with any other medium-chain triglyceride (MCT) product.

Reauthorization - Prescriber: Prescribed by a board-certified medical geneticist experienced in the treatment of long-chain fatty acid oxidation disorders (LC-FAOD).

Reauthorization - Dosing: Target recommended daily dosage does not exceed 35% of the patient's total prescribed daily caloric intake (DCI).<=35% DCI

Reauthorization - Dietary management: Patient is receiving disease-related dietary management.

Initial Therapy Requirements

Initial Therapy

Initial therapy coverage requires diagnostic confirmation, appropriate prescriber, dosing limit, dietary management, and avoidance of concomitant MCT products.

Initial therapy criteria (drug-specific): Submission of medical records confirming LC-FAOD with at least two diagnostic criteria; not receiving Dojolvi with any other MCT product; prescribed by a board-certified medical geneticist experienced in LC-FAOD; target recommended daily dosage does not exceed 35% of total prescribed daily caloric intake (DCI); patient is receiving disease-related dietary management. If not diagnosed by newborn screening, patient has history of clinical manifestations of LC-FAOD (e.g., rhabdomyolysis).

Reauthorization / Continuation Therapy

Reauthorization requires demonstration of continued clinical benefit and adherence to prescriber, dosing, and dietary criteria.

Continuation criteria: Documentation of positive clinical response to Dojolvi; patient is not receiving Dojolvi in combination with any other MCT product; prescribed by a board-certified medical geneticist experienced in LC-FAOD; target recommended daily dosage does not exceed 35% of total prescribed daily caloric intake (DCI); patient is receiving disease-related dietary management.

Dosing and Coding Guidance

inv-04: Target recommended daily dosage as percent of total prescribed daily caloric intake (DCI) — key/value

Target DCI percentageRecommended daily dosage does not exceed 35% of the patient’s total prescribed daily caloric intake (DCI).

ContextThis dosing threshold applies for both initial authorization and reauthorization criteria for Dojolvi (triheptanoin).

Authorization impactExceeding 35% of total prescribed DCI is a trigger for potential denial of coverage.

Provider Requirements and Operational Notes

Prior Authorization

Prior Authorization Required

Prior authorization is required for Dojolvi. Approvals are contingent on the diagnostic, prescriber, dosing, and dietary criteria listed in the clinical criteria (Initial Authorization and Reauthorization). Prior authorization must be obtained before initiating therapy.

Prior authorization required: approvals contingent on diagnostic confirmation of LC-FAOD, prescriber requirements, dosing limits (<= 35% of DCI), and documentation of disease-related dietary management.
Authorization duration: approvals issued for up to 12 months (subject to state mandates and member benefit plan).

Note

Automated Approval and Supply Limits

Automated approval may be available in some programs. UnitedHealthcare may approve initial and re-authorization based solely on previous claim/medication history, diagnosis codes (ICD-10), and/or claim logic; use of these automated processes varies by program and therapeutic class. Supply or quantity limits may apply—verify program-specific limits before dispensing.

Automated approval triggers: prior claim/medication history and diagnosis codes (ICD-10) may allow auto-approval.
Supply limits: program-specific supply/quantity limits may be enforced.

Documentation Required

Required Documentation

Submit medical records to support the request. For initial authorization include documentation confirming LC-FAOD diagnosis with at least two of the following: disease-specific elevation of acylcarnitines on newborn blood spot or plasma, low enzyme activity in cultured fibroblasts, or genetic testing showing pathogenic mutation(s) in LC-FAOD–associated genes (e.g., CPT2, ACADVL, HADHA, HADHB). If not diagnosed by newborn screening, include history of clinical manifestations (e.g., rhabdomyolysis). For reauthorization include documentation of positive clinical response to Dojolvi therapy.

Required diagnostic documentation: ≥2 of the listed diagnostic findings for LC-FAOD (acylcarnitine elevation, low enzyme activity, pathogenic genetic test).
If newborn screening absent: provide clinical history consistent with LC-FAOD (e.g., rhabdomyolysis).
Reauthorization documentation: evidence of clinical benefit (e.g., improved cardiac measures, reduced rhabdomyolysis).

Denial Risk

Triggers for Potential Denial

Common reasons for denial include missing or inadequate diagnostic confirmation (fewer than two required diagnostic criteria), concomitant use of other MCT products, prescriber not meeting specialty requirement (not a board-certified medical geneticist experienced in LC-FAOD), dosing exceeding 35% of total daily caloric intake, and lack of documented disease-related dietary management or lack of clinical response on reauthorization.

Missing diagnostic confirmation (fewer than two of the required diagnostic criteria).
Concomitant use of other MCT products with Dojolvi.
Prescriber not a board-certified medical geneticist experienced in LC-FAOD.
Prescribed dose exceeds 35% of patient’s total prescribed daily caloric intake (DCI).
Insufficient documentation of disease-related dietary management or lack of documented clinical benefit at reauthorization.

Key Definitions

inv-10: LC-FAOD — definition of long-chain fatty acid oxidation disorders, typical diagnostic approach.

DefinitionLong-chain fatty acid oxidation disorders (LC-FAOD) are inherited defects in enzymes of long-chain fatty acid metabolism; Dojolvi is indicated for pediatric and adult patients with molecularly confirmed LC-FAOD.

Typical diagnostic criteriaDiagnosis is confirmed with at least two of: disease-specific elevation of acylcarnitines on newborn blood spot or in plasma; low enzyme activity in cultured fibroblasts; genetic testing demonstrating one or more pathogenic mutations in genes associated with LC-FAOD (e.g., CPT2, ACADVL, HADHA, HADHB).

Clinical presentation noteIf not diagnosed by newborn screening, documentation of clinical manifestations (e.g., rhabdomyolysis) supports the diagnosis.

Prescriber expectationManagement and prescribing should be by a board-certified medical geneticist experienced in LC-FAOD treatment as required by the policy.

Clinical Background

Dojolvi (triheptanoin) is indicated as a source of calories and fatty acids for the treatment of pediatric and adult patients with molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD). LC-FAOD are inherited defects in long-chain fatty acid metabolism and may be identified by disease-specific elevations of acylcarnitines, low enzyme activity in cultured fibroblasts, or pathogenic mutations in genes such as CPT2, ACADVL, HADHA, or HADHB.