Subject: Uptravi 1645-A SGM P2024
Policy:
I. 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 Indication
Uptravi is indicated for the treatment of pulmonary arterial hypertension (PAH, World Health
Organization [WHO] Group I) to delay disease progression and reduce the risk of
hospitalization for PAH. Effectiveness of Uptravi tablets was established in a long-term study in
PAH patients with WHO Functional Class II-III symptoms. Patients had idiopathic and heritable
PAH, PAH associated with connective tissue disease, PAH associated with congenital heart
disease with repaired shunts.
All other indications are considered experimental/investigational and not medically necessary.
II. PRESCRIBER SPECIALTIES
This medication must be prescribed by or in consultation with a pulmonologist or cardiologist.
III. CRITERIA FOR INITIAL APPROVAL
Pulmonary arterial hypertension (PAH)
Authorization of 12 months may be granted for treatment of PAH when ALL of the following
criteria are met:
A. Member has PAH defined as WHO Group 1 class of pulmonary hypertension (refer to
Appendix).
B. PAH was confirmed by either criterion (1) or criterion (2) below:
1. Pretreatment right heart catheterization with all of the following results:
i. Mean pulmonary arterial pressure (mPAP) > 20 mmHg
ii. Pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg
iii. Pulmonary vascular resistance (PVR) ≥ 3 Wood units in adult members or
pulmonary vascular resistance index (PVRI) ≥ 3 Wood units x m2 in pediatric
members
2. For infants less than one year of age, PAH was confirmed by Doppler echocardiogram if
right heart catheterization cannot be performed.
IV. CONTINUATION OF THERAPY
Authorization of 12 months may be granted for members with an indication listed in Section III
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.
V. APPENDIX
WHO Classification of Pulmonary Hypertension (PH)
1 Pulmonary arterial hypertension (PAH)
1.1 Idiopathic PAH
1.2 Heritable PAH
1.3 Drug- and toxin-induced PAH
1.4 PAH associated with:
1.4.1 Connective tissue disease
1.4.2 Human immunodeficiency virus (HIV) infection
1.4.3 Portal hypertension
1.4.4 Congenital heart disease
1.4.5 Schistosomiasis
1.5 PAH long-term responders to calcium channel blockers
1.6 PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease
[PVOD]/pulmonary capillary hemangiomatosis [PCH]) involvement
1.7 Persistent PH of the newborn syndrome
2 PH due to left heart disease
2.1 PH due to heart failure with preserved left ventricular ejection fraction (LVEF)
2.2 PH due to heart failure with reduced LVEF
2.3 Valvular heart disease
2.4 Congenital/acquired cardiovascular conditions leading to post-capillary PH
3 PH due to lung diseases and/or hypoxia
3.1 Obstructive lung disease
3.2 Restrictive lung disease
3.3 Other lung disease with mixed restrictive/obstructive pattern
3.4 Hypoxia without lung disease
3.5 Developmental lung disorders
4 PH due to pulmonary artery obstructions
4.1 Chronic thromboembolic PH
4.2 Other pulmonary artery obstructions
4.2.1 Sarcoma (high or intermediate grade) or angiosarcoma
4.2.2 Other malignant tumors
Renal carcinoma
Uterine carcinoma
Germ cell tumors of the testis
Other tumors
4.2.3 Non-malignant tumors
Uterine leiomyoma
4.2.4 Arteritis without connective tissue disease
4.2.5 Congenital pulmonary artery stenosis
4.2.6 Parasites
Hydatidosis
5 PH with unclear and/or multifactorial mechanisms
5.1 Hematologic disorders: Chronic hemolytic anemia, myeloproliferative disorders
5.2 Systemic and metabolic disorders: Pulmonary Langerhans cell histiocytosis, Gaucher
disease, glycogen storage disease, neurofibromatosis, sarcoidosis
5.3 Others: Chronic renal failure with or without hemodialysis, fibrosing mediastinitis
5.4 Complex congenital heart disease
Place of Service:
Outpatient
The above policy is based on the following references:
- Uptravi [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; July 2022.
- Sitbon O, Channick R, Chin K, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373:2522-33.
- McLaughlin V, et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. J Am Coll Cardiol. 2009;53:1573-1619.
- Klinger JR, Elliott CG, Levine DJ, et al. Therapy for pulmonary arterial hypertension in adults: update of the CHEST guideline and expert panel report. Chest. 2019;155(3):565-586.
- Galie N, McLaughlin VV, Rubin LJ, Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J. 2019;53(1):1802148. doi: 10.1183/13993003.02148-2018
- 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
- Abman SH, Hansmann G, Archer SL, et al. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132(21):2037-99.
Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.
October 30, 2024
