Treprostinil injection (Remodulin®); Treprostinil

We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the Medical Policy and a health plan or government program (e.g., TennCare), the express terms of the health plan or government program will govern.

POLICY

INDICATIONS

The indications below including FDA-approved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to the prescribed therapy.

FDA-Approved Indications

Treatment of pulmonary arterial hypertension (PAH; World Health Organization [WHO] Group 1) to diminish symptoms associated with exercise. Studies establishing effectiveness included patients with New York Heart Association (NYHA) Functional Class II-IV symptoms and etiologies of idiopathic or heritable PAH, PAH associated with congenital systemic-to-pulmonary shunts, or PAH associated with connective tissue diseases.
In patients with PAH requiring transition from epoprostenol, to diminish the rate of clinical deterioration. Consider the risks and benefits of each drug prior to transition.

All other indications are considered experimental/investigational and not medically necessary.

PRESCRIBER SPECIALTIES

This medication must be prescribed by or in consultation with a pulmonologist or cardiologist.

COVERAGE CRITERIA

Pulmonary Arterial Hypertension (PAH)

Authorization of 12 months may be granted for treatment of PAH when ALL of the following criteria are met:

Member has PAH defined as WHO Group 1 class of pulmonary hypertension (refer to Appendix).
PAH was confirmed by either of the following:

Pretreatment right heart catheterization with all of the following results:

Mean pulmonary arterial pressure (mPAP) > 20 mmHg
Pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg
Pulmonary vascular resistance (PVR) > 2 Wood units. For pediatric members, pulmonary vascular resistance index (PVRI) > 3 Wood units x m²is also acceptable.

For infants less than one year of age, PAH was confirmed by Doppler echocardiogram if right heart catheterization cannot be performed.

CONTINUATION OF THERAPY

Authorization of 12 months may be granted for members with an indication listed in the coverage criteria section who are currently receiving the requested medication through a paid pharmacy or medical benefit, and who are experiencing benefit from therapy as evidenced by disease stability or disease improvement.

APPENDIX

WHO Classification of Pulmonary Hypertension (PH)

Note: Patients with heritable PAH or PAH associated with drugs and toxins might be long-term responders to calcium channel blockers.

Group 1: Pulmonary Arterial Hypertension (PAH)

Idiopathic

Long-term responders to calcium channel blockers

Heritable
Associated with drugs and toxins

Associated with:

Connective tissue disease
Human immunodeficiency virus (HIV) infection
Portal hypertension
Congenital heart disease
Schistosomiasis

PAH with features of venous/capillary (pulmonary veno-occlusive disease [PVOD]/pulmonary capillary hemangiomatosis [PCH]) involvement
Persistent PH of the newborn

Group 2: PH associated with Left Heart Disease

Heart failure:

With preserved ejection fraction
With reduced or mildly reduced ejection fraction
Cardiomyopathies with specific etiologies (i.e., hypertrophic, amyloid, Fabry disease, and Chagas disease)

Valvular heart disease:

Aortic valve disease
Mitral valve disease
Mixed valvular disease

Congenital/acquired cardiovascular conditions leading to post-capillary PH

Group 3: PH associated with Lung Diseases and/or Hypoxia

Chronic obstructive pulmonary disease (COPD) and/or emphysema

Interstitial lung disease
Combined pulmonary fibrosis and emphysema

Other parenchymal lung diseases (i.e., parenchymal lung diseases not included in Group 5)
Nonparenchymal restrictive diseases:

Hypoventilation syndromes
Pneumonectomy

Hypoxia without lung disease (e.g., high altitude)

Developmental lung diseases

Group 4: PH associated with Pulmonary Artery Obstructions

Chronic thromboembolic PH
Other pulmonary artery obstructions:

Sarcomas (high-or intermediate-grade or angiosarcoma)

Other malignant tumors (e.g., renal carcinoma, uterine carcinoma, germ-cell tumors of the testis)

Non-malignant tumors (e.g., uterine leiomyoma)

Arteritis without connective tissue disease

Congenital pulmonary artery stenoses

Hydatidosis

Group 5: PH with Unclear and/or Multifactorial Mechanisms

Hematological disorders, including inherited and acquired chronic hemolytic anemia and chronic myeloproliferative disorders
- Systemic disorders: Sarcoidosis, pulmonary Langerhans cell histiocytosis, and neurofibromatosis type 1
- Metabolic disorders, including glycogen storage diseases and Gaucher disease
- Chronic renal failure with or without hemodialysis

Pulmonary tumor thrombotic microangiopathy
Fibrosing mediastinitis
- Complex congenital heart disease

APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS

BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.

ADDITIONAL INFORMATION

For appropriate chemotherapy regimens, dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) published by the National Comprehensive Cancer Network®, Drugdex Evaluations of Micromedex Solutions at Truven Health, or The American Hospital Formulary Service Drug Information).

REFERENCES

Remodulin [package insert]. Research Triangle Park, NC: United Therapeutics Corp.; October 2023.
Treprostinil [package insert]. Princeton, NJ: Sandoz, Inc.; April 2023.
Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019;53(1):1801913; doi:10.1183/13993003.01913-2018.
Kovacs G, Bartolome S, Denton CP, et al. Definition, classification and diagnosis of pulmonary hypertension. Eur Respir J. 2024;64(4):2401324. doi: 10.1183/13993003.01324-2024.
Chin KM, Gaine SP, Gerges C, et al. Treatment algorithm for pulmonary arterial hypertension. Eur Respir J. 2024;64(4):2401325. doi: 10.1183/13993003.01325-2024.
Ivy D, Rosenzweig EB, Abman SH, et al. Embracing the challenges of neonatal and pediatric pulmonary hypertension. Eur Respir J. 2024;64(4):2401345. doi: 10.1183/13993003.01345-2024.

ORIGINAL EFFECTIVE DATE: 6/1/2002

MOST RECENT REVIEW DATE: 11/11/2025

ID_CHS

Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.

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