Subject: Tyvaso 1650-A SGM P2021a

Drug

Tyvaso  (treprostinil inhalation solution)

Tyvaso DPI  (treprostinil inhalation powder)

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 Indications
             A.  Treatment of Pulmonary arterial hypertension (PAH; WHO
                  Group 1) to improve exercise ability. Studies establishing
                  effectiveness predominately included patients with NYHA
                  Functional Class III symptoms and etiologies of idiopathic
                  or heritable PAH or PAH associated with connective tissue
                  diseases.
             B.  Treatment of Pulmonary hypertension associated with
                  interstitial lung disease (PH-ILD; WHO Group 3) to improve
                  exercise ability. The study establishing effectiveness
                  predominately included patients with etiologies of
                  idiopathic interstitial pneumonia (IIP) inclusive of idiopathic
                  pulmonary fibrosis (IPF), combined pulmonary fibrosis and
                  emphysema (CPFE), and WHO Group 3 connective tissue
                  disease.

                  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 Hypertension (PH)
            Authorization of 12 months may be granted for treatment of
            PH when both of the following criteria are met:
            A.  Member has either of the following:
                 1.  WHO Group 1 class of pulmonary hypertension (refer to
                      Appendix)
                 2.  Pulmonary hypertension associated with interstitial lung
                      disease (WHO Group 3)
            B.  PH 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, PH 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 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.

 
       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
                          4.2.5   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. Tyvaso [package insert]. Research Triangle Park, NC: United Therapeutics Corp.; March 2021.
2. Tyvaso DPI [package insert]. Research Triangle Park, NC: United Therapeutics Corp.; May 2022
3. Chin KM, Rubin LJ. Pulmonary arterial hypertension. J Am Coll Cardiol. 2008;51(16):1527-1538.
4. 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.
5. Badesch DB, Champion HC, Gomez-Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S55-S66.
6. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D34-S41.
7. 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.
8. 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.
9. 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.
10. 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.
11. 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.
12. 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.
13. 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.

July 15, 2022