Subject: riociguat-Adempas 1641-A SGM P2023

Drug

Adempas  (riociguat)

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


  III.  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 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. Adempas [package insert]. Whippany, NJ: Bayer HealthCare Pharmaceuticals, Inc.; January 2023.
2. Riociguat [package insert]. Telangana, India: MSN Laboratories Private Limited: September 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. 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. Jaff MR, McMurty 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.
10. Fedullo P, Kerr KM, Kim NH, Auger WR. Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011;183(12):1605-1613.
11. 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.
12. 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.
13. 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.
14. 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.

October 14, 2023