Subject: Uptravi 1645-A SGM P2023
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 patients or pulmonary vascular resistance
index (PVRI) ≥ 3 Wood units x m2 in pediatric
patients
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
1 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 diseases
1.4.2 HIV infection
1.4.3 Portal hypertension
1.4.4 Congenital heart diseases
1.4.5 Schistosomiasis
1.5 PAH long-term responders to calcium channel blockers
1.6 PAH with overt features of venous/capillaries (PVOD/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 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 obstruction
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 tumours of the testis
Other tumours
4.2.3 Non-malignant tumours
Uterine leiomyoma
4.2.4 Arteritis without connective tissue disease
4.2.3 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.
- Rubin LJ; American College of Chest Physicians. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126(1 Suppl):7S-10S.
- 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 Guidelines 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: 1802148; DOI: 10.1183/13993003.02148-2018. Published 24 January 2019.
- Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53:1801913; 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 14, 2023
