Subject: ambrisentan-Letairis 1646-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
Indicated for the treatment of pulmonary arterial hypertension (PAH) (World Health Organization [WHO] Group 1):
A.  To improve exercise ability and delay clinical worsening.
B.  In combination with tadalafil to reduce the risks of disease progression and hospitalization for
     worsening PAH, and to improve exercise ability.

Studies establishing effectiveness included predominantly patients with WHO Functional Class II-III symptoms and etiologies of idiopathic or heritable PAH or PAH associated with connective tissue diseases.

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
          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. Letairis [package insert]. Foster City, CA: Gilead Sciences, Inc.; August 2019.
2. Ambrisentan [package insert]. Cranbury, NJ: Sun Pharmaceutical Industries, Inc.; January 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