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.
A. FDA-Approved Indications 1. Revatio/Liqrev/sildenafil is indicated for the treatment of pulmonary arterial hypertension (PAH) (World Health Organization [WHO] Group 1) in adults to improve exercise ability and delay clinical worsening. 2. Revatio/sildenafil is indicated in pediatric patients 1 to 17 years old for the treatment of pulmonary arterial hypertension (PAH) (WHO Group I) to improve exercise ability and, in pediatric patients too young to perform standardized exercise testing, pulmonary hemodynamics thought to underlie improvements in exercise.
B. Compendial Uses 1. Secondary Raynaud’s phenomenon 2. PAH (WHO Group I) in pediatric members less than 1 year of age
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 for the diagnosis of pulmonary arterial hypertension (PAH).
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 either criterion (i) or criterion (ii) below: i. Pretreatment right heart catheterization with all of the following results: a. Mean pulmonary arterial pressure (mPAP) > 20 mmHg b. Pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg c. Pulmonary vascular resistance (PVR) ≥ 3 Wood units in adult members or pulmonary vascular resistance index (PVRI) ≥ 3 Wood units x m2 in pediatric members ii. For infants less than one year of age, PAH was confirmed by Doppler echocardiogram if right heart catheterization cannot be performed.
B. Secondary Raynaud’s phenomenon Authorization of 12 months may be granted for treatment of secondary Raynaud’s phenomenon when the member has had an inadequate response to one of the following medications: 1. Calcium channel blockers 2. Angiotensin II receptor blockers 3. Selective serotonin reuptake inhibitors 4. Alpha blockers 5. Angiotensin-converting enzyme inhibitors 6. Topical nitrates
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:
Revatio [package insert]. Morgantown, WV; Viatris Specialty LLC; January 2023.
Liqrev [package insert]. Farmville, NC: CMP Pharma, Inc.; April 2023.
Sildenafil [package insert]. Piscataway, NJ: Camber Pharmaceuticals, Inc.; February 2024.
Sildenafil for oral suspension [package insert]. East Windsor, NJ: Aurobindo Pharma USA, Inc.; January 2024.
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.
Badesch DB, Champion HC, Gomez-Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S55-S66.
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.
Barst RJ, Ivy DD, Gaitan G, et al. A randomized, double-blind, placebo-controlled, dose-ranging study of oral sildenafil citrate in treatment-naïve children with pulmonary arterial hypertension. 2012;125:324-334.
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.
Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353:2148-2157.
Rubin LJ, Badesch DB, Fleming RT, et al. Long-term treatment with sildenafil citrate in pulmonary arterial hypertension. Chest. 2011;140(5):1274-1283.
IBM Micromedex® DRUGDEX® (electronic version). IBM Watson Health, Greenwood Village, Colorado, USA. Available at: https://www.micromedexsolutions.com. Accessed April 12, 2024.
Lexi-Drugs. Lexicomp. Wolters Kluwer Health, Inc. Riverwoods, IL. Available at: http://online.lexi.com. Accessed April 12, 2024.
Hughes M, Ong VH, Anderson ME, et al. Consensus best practice pathway of the UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis. Rheumatology. 2015;54:2015-2024.
Roustit M, Blaise S, Allanore Y, et al. Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud’s phenomenon: systematic review and meta-analysis of randomized trials. Ann Rheum Dis. 2013;72(10):1696-1699.
Walker KM, Pope J, et al. Treatment of systemic sclerosis complications: what to use when first-line treatment fails – a consensus of systemic sclerosis experts. Semin Arthritis Rheum. 2012;42(1):42-55.
Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327-1339.
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.
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
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.
Barst RJ, Beghetti M, Pulido T, et al. STARTS-2: long-term survival with oral sildenafil monotherapy in treatment-naive pediatric pulmonary arterial hypertension. Circulation. 2014;129(19):1914-23.
Kwan WC, Shavelle DM, Laughrun DR. Pulmonary vascular resistance index: getting the units right and why it matters. Clin Cardiol. 2019;42(3):334-338.
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.
