Subject: Orenitram 1643-A SGM P2021

Drug

Orenitram  (treprostinil extended-release tablets)

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
        Orenitram is indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to delay disease progression and to improve exercise capacity. The studies that established                      effectiveness included predominately patients with WHO functional class II-III symptoms and etiologies of idiopathic or heritable PAH (66%) or PAH associated with connective tissue disease (26%).

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

II.    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.     mPAP > 20 mmHg
                  ii.    PCWP ≤ 15 mmHg
                  iii.   PVR ≥ 3 Wood units
             2.  For infants less than one year of age, PAH was confirmed by Doppler echocardiogram if right heart catheterization cannot be performed. 

 

III.  CONTINUATION OF THERAPY
        Authorization of 12 months may be granted for members with an indication listed in Section II 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.


IV.    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
                4.2.5    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:

1. Orenitram [package insert]. Research Triangle Park, NC: United Therapeutics Corp.; October 2019.
2. Chin KM, Rubin LJ. Pulmonary arterial hypertension. J Am Coll Cardiol. 2008;51(16):1527-1538.
3. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573-1619.
4. Badesch DB, Champion HC, Gomez-Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S55-S66.
5. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D34-S41.
6. 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.
7. Barst RJ, Gibbs SR, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S78-S84.
8. Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults. CHEST guideline and expert panel report. Chest. 2014;46(2):449-475.
9. 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.
10. 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.
11. 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.
12. 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.

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.

September 30, 2021