Pulmonary Hypertension Drugs

Transcription

1 Pulmonary Hypertension Drugs Policy Number: Original Effective Date: MM /01/2009 Line(s) of Business: Current Effective Date: HMO; PPO 05/25/2012 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/Exclusions and Administrative Guidelines) for the treatment of primary or secondary PH when 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/Exclusions and Administrative Guidelines) for the treatment of primary or secondary PH when 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

2 Pulmonary Hypertension Drugs 2 III. Limitations/Exclusions A. PH drugs are not covered for patients with 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 and generic drugs are available. D. Sildenafil (Revatio) will be covered at the manufacturer's recommended dose of 20 mg 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 (i.e., Flolan) b. Trepostinol sodium (i.e., Remodulin) 2. Oral/Inhaled Drugs a. For private lines of business (PPO/HMO) and QUEST, see the Medco 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 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,

3 Pulmonary Hypertension Drugs 3 discomfort is increased. 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. 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. A clinical trial of ambrisentan and tadalafil in pulmonary arterial hypertension associated with systemic schlerosis (ATPAHSS) (NCT ). Sponsored by United Therapeutics. Last updated January 4, Available online at Clinicaltrials.gov. Last accessed March Badesch DB, Abman SH, Simonneau G et al. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest 2007; 131(6):

4 Pulmonary Hypertension Drugs 4 3. Badesch DB, Tapson VF, McGoon MD et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000; 132(6): 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): Barst RJ, McGoon M, McLaughlin V et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41(12): 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): Channick RN, Olschewski H, Seeger W et al. Safety and efficacy of inhaled treprostinil as add-on therapy to bosentan in pulmonary arterial hypertension. J Am Coll Cardiol 2006; 48(7): 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): Effects of the combination of bosentan and sildenafil versus sildenafil monotherapy on pulmonary arterial hypertension (PAH) (Compass 2) (NCT ). Sponsored by Atelion. Last updated March 9, Available online at Clinicaltrials.gov. Last accessed March Galie N, Brundage BH, Ghofrani HA et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119(22): Galie N, Ghofrani HA, Torbicki A et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353(20): Galie N, Humbert M, Vacheiry JL et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind placebo-controlled trial. J Am Coll Cardiol 2002; 39(9): 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): Hoeper MM, Leuchte H, Halank M et al. Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J Oct; 28(4): Hoeper MM, Markevych I, Spiekerkoetter E et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005; 26(5): Humbert M, Sanchez O, Fartoukh M et al. Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study. Eur Respir J 1999; 13(6): Jain M, Varga J. Bosentan for the treatment of systemic sclerosis-associated pulmonary arterial hypertension, pulmonary fibrosis and digital ulcers. Expert Opin Pharmacother 2006; 7(11): 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): Langleben D, Christman BW, Barst RJ et al. Effects of the thromboxane synthetase inhibitor and receptor antagonist terbogrel in patients with primary pulmonary hypertension. Am Heart J 2002; 143(5):E4.

5 Pulmonary Hypertension Drugs 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): 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): 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): McLaughlin VV, Oudiz RJ, Frost A et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006; 174(11): McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106(12): Naeije R, Huez S. Expert opinion on available options treating pulmonary arterial hypertension. Expert Opin Pharmacother 2007; 8(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): Reinhart K, Salerno E, White CM. Pulmonary arterial hypertension: an overview of current pharmacologic treatment. Conn Med 2008; 72(9): Rich S. The current treatment of pulmonary arterial hypertension: time to redefine success. Chest 2006; 130(4): Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99(14): Rubenfire M, McLaughlin VV, Allen RP et al. Transition from IV epoprostenol to subcutaneous treprostinil in pulmonary arterial hypertension: a controlled trial. Chest 2007; 132(3): Rubin LJ, Badesch DB, Barst RJ et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346(12): Rubin LJ, Simonneau G, Hoeper MM et al. Bosentan improves hemodynamics in patients receiving background sildenafil treatment: results from early, a randomized, double-blind, placebo-controlled study in patients with mildly symptomatic pulmonary arterial hypertension [abstract]. Chest 2007; 132(4 suppl S):487S. Available: (accessed April 2009). 33. Ruiz MJ, Escribano P, Delgado JF et al. Efficacy of sildenafil as a rescue therapy for patients with severe pulmonary arterial hypertension and given long-term treatment with prostanoids: 2- year experience. J Heart Lung Transplant 2006; 25(11): Ryerson CJ, Nayer S, Swiston JR et al. Pharmacotherapy in pulmonary arterial hypertension: a systematic review and meta-analysis. Respiratory Res 2010; 11: Simonneau G, Barst RJ, Galie N et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized placebo-controlled trial. Am J Respir Crit Care Med 2002; 165(6): Simonneau G, Robbins IM, Beghetti M et al. Updated classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54(1 suppl):s43-54.

6 Pulmonary Hypertension Drugs 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): The Canadian Agency for Drugs and Technologies in Health (CADTH). Health Technology Assessment Rapid Review: drugs for pulmonary hypertension: a systematic review of the clinical effectiveness of combination therapy. April Available online at: f 39. The efficacy and safety of vardenafil in the treatment of pulmonary arterial hypertension (EVALUATION) (NCT ). Sponsored by Tongji University. Last updated February 11, Available online at Clinicaltrials.gov. Last accessed March Ventavis, package insert. 41. Voswinckel R, Enke B, Reichenberger F et al. Favorable effects of inhaled treprostinil in severe pulmonary hypertension: results from randomized controlled pilot studies. J Am Coll Cardiol 2006; 48(8): 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):