Subject: Adempas 1641-A SGM P2024

Drug

ADEMPAS  (riociguat)

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.  Pulmonary Arterial Hypertension (PAH)
               Adempas is indicated for the treatment of adults with pulmonary arterial hypertension (PAH),
               (World Health Organization [WHO] Group 1), to improve exercise capacity, WHO functional
               class and to delay clinical worsening. Efficacy was shown in patients on Adempas
               monotherapy or in combination with endothelin receptor antagonists or prostanoids. Studies
               establishing effectiveness included predominately patients with WHO functional class II-III
               and etiologies of idiopathic or heritable PAH or PAH associated with connective tissue
               diseases.
          B.  Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
               Adempas is indicated for the treatment of adults with persistent/recurrent chronic
               thromboembolic pulmonary hypertension (CTEPH), (WHO Group 4) after surgical
               treatment, or inoperable CTEPH to improve exercise capacity and WHO functional class.

          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

          A.  Pulmonary arterial hypertension (PAH)
               Authorization of 12 months may be granted for treatment of PAH when ALL of the following
               criteria are met:
               1.  Member has PAH defined as WHO Group 1 class of pulmonary hypertension (refer to
                    Appendix).
               2.  PAH was confirmed by right heart catheterization with all of the following pretreatment
                    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

          B.  Chronic thromboembolic pulmonary hypertension (CTEPH)
               Authorization of 12 months may be granted for treatment of CTEPH when ALL of the
               following criteria are met:
               1.  Member has CTEPH defined as WHO Group 4 class of pulmonary hypertension (refer to
                    Appendix).
               2.  Member meets either criterion (i) or criterion (ii) below:
                    i.     Recurrent or persistent CTEPH after pulmonary endarterectomy (PEA)
                    ii.    Inoperable CTEPH with diagnosis confirmed by BOTH of the following (a. and b.):
                           a.  Computed tomography (CT)/magnetic resonance imaging (MRI) angiography or
                                pulmonary angiography
                           b.  Pretreatment right heart catheterization with all of the following results:
                                1.  mPAP > 20 mmHg
                                2.  PCWP ≤ 15 mmHg
                                3.  PVR ≥ 3 Wood units


   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. Adempas [package insert]. Whippany, NJ: Bayer HealthCare Pharmaceuticals, Inc.; January 2023.
2. 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.
3. Badesch DB, Champion HC, Gomez-Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S55-S66.
4. 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.
5. Jaff MR, McMurtry MS, Archer SL, et al. Management of massive and submassive pulmonary embolism, iliofemoral deep vein thrombosis, and chronic thromboembolic pulmonary hypertension: a scientific statement from the American Heart Association. Circulation. 2011;123(16):1788-1830.
6. Fedullo P, Kerr KM, Kim NH, Auger WR. Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011;183(12):1605-1613.
7. Jenkins D, Mayer E, Screaton N, Madani M. State-of-the-art chronic thromboembolic pulmonary hypertension diagnosis and management. Eur Respir Rev. 2012;21(123):32-39.
8. 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.
9. 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
10. 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