Pharmacy Prior Authorization Criteria

Dojolvi (triheptanoin)- PA, NF

Indications for Prior Authorization

Dojolvi (triheptanoin)

• For diagnosis of Long-Chain Fatty Acid Oxidation Disorders (LC-FAOD)
  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).

Criteria

Dojolvi

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)

• Diagnosis of a long-chain fatty acid oxidation disorder (LC-FAOD) has been confirmed by at least two of the following: [B]
• Disease specific elevation of acyl-carnitines on a newborn blood spot or in plasma
• Low enzyme activity in cultured fibroblasts
• One or more known pathogenic mutations in CPT2, ACADVL, HADHA, or HADHB
AND
• Not used with any other medium-chain triglyceride (MCT) product
AND
• Prescribed by or in consultation with a clinical specialist knowledgeable in appropriate disease-related dietary management (e.g., geneticist, cardiologist, gastroenterologist, etc.)

Dojolvi

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)

• Prescriber attests to continued need of therapy [A]
AND
• Not used with any other medium-chain triglyceride (MCT) product
AND
• Prescribed by or in consultation with a clinical specialist knowledgeable in appropriate disease-related dietary management (e.g., geneticist, cardiologist, gastroenterologist, etc.)

Dojolvi

Non Formulary

Length of Approval: 12 Month(s)

• Submission of medical records (e.g., chart notes) confirming diagnosis of a long-chain fatty acid oxidation disorder (LC-FAOD) has been confirmed by at least two of the following: [B]
• Disease specific elevation of acyl-carnitines on a newborn blood spot or in plasma
• Low enzyme activity in cultured fibroblasts
• One or more known pathogenic mutations in CPT2, ACADVL, HADHA, or HADHB
AND
• Submission of medical records (e.g., chart notes) or absence of paid claims confirming requested medication is not used with any other medium-chain triglyceride (MCT) product
AND
• Prescribed by or in consultation with a clinical specialist knowledgeable in appropriate disease-related dietary management (e.g., geneticist, cardiologist, gastroenterologist, etc.)

P & T Revisions

2026-08-28, 2025-10-02, 2024-09-04, 2023-09-05, 2022-09-14, 2021-08-05, 2020-09-16

References

1. Dojolvi (triheptanoin) prescribing information. Ultragenyx Pharmaceutical Inc. Novato, CA. Accessed August 2026.

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End Notes

1. This reauthorization criteria was recommended by the clinical consultant since LA-FAODs are progressive even with therapy. Patients will need lifelong therapy even though positive clinical response may not be seen.
2. The efficacy of triheptanoin as a source of calories and fatty acids was evaluated in Study 3 (NCT01379625). The study enrolled 32 adult and pediatric patients with a confirmed diagnosis of LC-FAOD and evidence of at least one significant episode of rhabdomyolysis and at least two of the following diagnostic criteria: disease specific elevation of acylcarnitines on a newborn blood spot or in plasma, low enzyme activity in cultured fibroblasts, or one or more known pathogenic mutations in CPT2, ACADVL, HADHA, or HADHB. (1)

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Revision History

• 2026-08-28: 2026 Annual Review. No criteria changes. Reference and background updates.
• 2025-10-02: 2025 Annual Review. Added drug specific NF criteria.
• 2024-09-04: 2024 Annual Review. Updated references.
• 2023-09-05: Annual Review
• 2022-09-14: Annual Review
• 2021-08-05: Annual Review
• 2020-09-16: New Program

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