Subject: bosentan-Tracleer 1649-A SGM P2024

Drug

TRACLEER  (bosentan)

bosentan

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
          Indicated for the treatment of pulmonary arterial hypertension (PAH) (World Health
          Organization [WHO] Group 1):
          A.  In adults to improve exercise ability and to decrease clinical worsening. Studies establishing
               effectiveness included predominantly patients with WHO Functional Class II-IV symptoms
               and etiologies of idiopathic or heritable PAH, PAH associated with connective tissue
               diseases, and PAH associated with congenital heart disease with left-to-right shunts.
          B.  In pediatric patients aged 3 years and older with idiopathic or congenital PAH to improve
               pulmonary vascular resistance (PVR), which is expected to result in an improvement in
               exercise ability.

          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:

1. Tracleer [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; February 2024.
2. Bosentan [package insert]. Pennington, NJ: Zydus Pharmaceuticals (USA) Inc. February 2024.
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. Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014;146(2):449-475.
6. 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.
7. 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.
8. 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
9. 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

 

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