Pulmonary Hypertension Drugs Policy Number: Original Effective Date: MM.04.028 10/01/2009 Line(s) of Business: Current Effective Date: HMO; PPO; QUEST Integration 05/22/2015 Section: Prescription Drugs Place(s) of Service: Home I. Description Pulmonary hypertension (PH) is characterized by sustained elevations of pulmonary artery pressure (PAP). PH is defined as a mean PAP greater than 25 mmhg at rest. A mean PAP of 8 to 20 mmhg at rest is considered normal, while a mean PAP of 21 to 24 mmhg at rest has uncertain clinical implications. Drug therapy of PH includes vasodilators (particularly calcium channel blockers and sildenafil), anticoagulants to reduce in situ thrombosis, inotropic and diuretic agents, and oxygen therapy. Lung transplantation and combined heart-lung transplantation have been performed in patients refractory to medical management. II. Criteria/Guidelines A. Drugs that are FDA-approved for the treatment of PH are covered (subject to Limitations and Administrative Guidelines) for the treatment of primary or secondary PH when all of the following criteria are met: 1. Therapy is recommended by a pulmonologist or cardiologist; 2. Right heart catheterization demonstrates a mean PAP of more than 25 mm Hg at rest; 3. Baseline assessment of all of the following is done: a. New York Heart Association Class; b. Echocardiogram. B. Continuation of therapy is covered (subject to Limitations and Administrative Guidelines) for the treatment of primary or secondary PH when all of the following criteria are met: 1. There is documentation of the patient's clinical response to therapy including the following: a. New York Heart Association Class; and b. Echocardiogram. 2. Patient is followed by a pulmonologist or cardiologist.
Pulmonary Hypertension Drugs 2 III. Limitations A. PH drugs are not covered for patients with chronic obstructive pulmonary disease (COPD) and pulmonary arterial hypertension with a FEV1<30. B. More than one drug may be appropriate for the treatment of PH. HMSA reserves the right to approve the least costly treatment. C. Only generic drugs are covered when both brand name and generic drugs are available. D. Sildenafil (Revatio) will be covered at the manufacturer's recommended dose of 20 mg taken orally three times a day. A higher dosage of sildenafil is not known to improve health outcomes and will not be covered. E. Tadalafil (Cialis) and vardenafil (Levitra) are not covered for the treatment of PH. IV. Administrative Guidelines A. Precertification is required for the following drugs for the treatment of PH, including, but not limited to: 1. Injectable/Infused Drugs a. Epoprostenol sodium (Flolan; Veletri) b. Treprostinil (Remodulin) 2. Oral/Inhaled Drugs a. For private lines of business (PPO/HMO) and QUEST, see the CVS Caremark Drug Policy for precertification requirements. B. Precertification is required for the initial three months of treatment. The following documentation from the medical record must be submitted: 1. Current clinical notes; 2. Prescription drug history; 3. Right heart cardiac catheterization results; 4. Echocardiogram; and 5. New York Heart Association Class. New York Heart Association Classification: Class Class I (Mild) Class II (Mild) Class III (Moderate) Class IV (Severe) Patient Symptoms No limitation of physical activity. Ordinary fatigue, palpitation, or dyspnea (shortness of breath). Slight limitation of physical activity. Comfortable at rest, but ordinary physical activity results in fatigue, palpitation, or dyspnea. Marked limitation of physical activity. Comfortable at rest, but less than ordinary activity causes fatigue, palpitation, or dyspnea. Unable to carry out any physical activity without discomfort. Symptoms of cardiac insufficiency at rest. If any physical activity is undertaken, discomfort is increased.
Pulmonary Hypertension Drugs 3 C. To precertify, please complete HMSA's Drug Review Request and mail or fax the form as indicated. D. Precertification is required for continuation of therapy for each additional 12 months. The following documentation must be submitted: 1. Evidence that patient is followed by a pulmonologist or cardiologist; and 2. Recent clinical notes including New York Heart Association Class documenting response to treatment and echocardiogram HCPCS Codes J1325 J3285 Description Injection, epoprostenol, 0.5 mg Injection, treprostinil, 1 mg V. Important Reminder The purpose of this Medical Policy is to provide a guide to coverage. This Medical Policy is not intended to dictate to providers how to practice medicine. Nothing in this Medical Policy is intended to discourage or prohibit providing other medical advice or treatment deemed appropriate by the treating physician. Benefit determinations are subject to applicable member contract language. To the extent there are any conflicts between these guidelines and the contract language, the contract language will control. This Medical Policy has been developed through consideration of the medical necessity criteria under Hawaii's Patients' Bill of Rights and Responsibilities Act (Hawaii Revised Statutes 432E-1.4), generally accepted standards of medical practice and review of medical literature and government approval status. HMSA has determined that services not covered under this Medical Policy will not be medically necessary under Hawaii law in most cases. If a treating physician disagrees with HMSA's determination as to medical necessity in a given case, the physician may request that HMSA reconsider the application of the medical necessity criteria to the case at issue in light of any supporting documentation. VI. References 1. Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. CHEST August 2014; 146(2): 449-475. 2. Barst RJ, Galie N, Naeije R et al. Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J 2006; 28(6):1195-203.
Pulmonary Hypertension Drugs 4 3. Barst RJ, Rubin LJ, Long WA et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 334(5):296-302. 4. Chen YF, Jowett S, Barton P et al. Clinical and cost-effectiveness of epoprostenol, iloprost, bosentan, sitaxentan and sildenafil for pulmonary arterial hypertension within their licensed indications: a systematic review and economic evaluation. Health Technol Assess 2009;13(49):1-320. 5. Galiè N, Brundage BH, Ghofrani HA et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119(22):2894-903. 6. Galiè N, Ghofrani HA, Torbicki A et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353(20):2148-57. 7. Galiè N, Manes A, Negro L et al. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009; 30(4):394-403. 8. Hoeper MM, Markevych I, Spiekerkoetter E et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005; 26(5):858-63. 9. Jing ZC, Jiang X, Wu BX et al. Vardenafil treatment for patients with pulmonary arterial hypertension: a multicenter, open-label study. Heart 2009; 95(18):1531-6. 10. Macchia A, Marchioli R, Tognoni G et al. Systematic review of trials using vasodilators in pulmonary arterial hypertension: why a new approach is needed. Am Heart J 2010; 159(2):245-57. 11. 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. Circulation 2009; 119(16):2250-94. 12. McLaughlin VV, Genthner DE, Panella MM et al. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998; 338(5):273-7. 13. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106(12):1477-82. 14. Oudiz RJ, Schilz RJ, Barst RJ et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004; 126(2):420-7. 15. Ryerson CJ, Nayer S, Swiston JR et al. Pharmacotherapy in pulmonary arterial hypertension: a systematic review and meta-analysis. Respiratory Res 2010; 11:12. 16. Simonneau G, Robbins IM, Beghetti M et al. Updated classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54(1 suppl):s43-54. 17. Simonneau G, Rubin LJ, Galie N et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008; 149(8):521-30. 18. The Canadian Agency for Drugs and Technologies in Health (CADTH). Therapeutic Review Report. Drugs for pulmonary arterial hypertension: comparative efficacy, safety, and costeffectiveness recommendations report. March 2015. Available online at: https://www.cadth.ca/sites/default/files/pdf/tr0006_pah_recs_report.pdf. 19. The Canadian Agency for Drugs and Technologies in Health (CADTH). Rapid Response Report: Summary with Critical Appraisal. Drug therapy for chronic thromboembolic pulmonary
Pulmonary Hypertension Drugs 5 hypertension: a review of the comparative clinical effectiveness. September 9, 2014. Available online at: https://www.cadth.ca/sites/default/files/pdf/htis/dec- 2014/RC0580%20Drug%20for%20CTEPH%20Final.pdf. 20. Flolan (epoprostenol sodium) [prescribing information]. Research Triangle Park, NC: GlaxoSmithKline; April 2015. Available online at: https://www.gsksource.com/pharma/content/dam/glaxosmithkline/us/en/prescribing_inform ation/flolan/pdf/flolan.pdf. 21. Veletri (epoprostenol) [prescribing information]. South San Francisco, CA: Actelion Pharmaceuticals US, Inc; June 2012. Available online at: https://www.veletri.com/pdf/veletri_full_prescribing_information_2nd_gen.pdf. 22. Remodulin (treprostinil) [prescribing information]. Research Triangle Park, CA: United Therapeutics Corp; December 2014. Available online at: https://www.remodulin.com/downloads/remodulin-prescribinginformation.pdf. 23. Wilkins MR, Paul GA, Strange JW et al. Sildenafil versus endothelin receptor antagonists for pulmonary hypertension (SERAPH) study. Am J Respir Crit Care Med 2005; 171(11):1292-7.