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DESCRIPTIONPulmonary arterial hypertension (PAH) is an abnormally high blood pressure in the arteries between the heart and lungs. PAH significantly reduces the ability of patients to exert themselves physically without becoming short of breath. PAH significantly shortens the life span of patients because it leads to heart failure. Epoprostenol (Flolan®), bosentan (Tracleer®), treprostinil (Remodulin®) (Tyvaso®), piloprost (Ventavis®), ambrisentan (Letairis®), tadalafil (Adcirca®), and sildenafil (Revatio®) are all FDA approved for treating patients with PAH (revised 8/10/2009).
Epoprostenol (Flolan®) is indicated for the treatment of primary pulmonary hypertension in patients with New York Heart Association (NYHA) Class III or Class IV symptoms. Flolan is a prostacyclin that has two major pharmacological actions. It directly dilates the pulmonary and systemic arterial vascular beds and inhibits platelet aggregation. Flolan decreases pulmonary arterial pressure through these mechanisms, which may lead to prolonged survival.
Bosentan (Tracleer®) is FDA approved for the treatment of pulmonary arterial hypertension (WHO Group I) in patients with WHO Class II to IV symptoms to improve exercise capacity and decrease the rate of clinical worsening. Tracleer is an endothelin receptor antagonist. Endothelin is produced endogenously in high concentrations in the lungs of patients with PAH, which suggests that endothelin is capable of causing PAH. There is a black box warning on Tracleer due to liver toxicity and the drug s potential to damage a fetus. Liver enzyme levels must be measured before initiation of treatment and monthly thereafter to avoid liver injury. Tracleer must not be prescribed to pregnant women because of its potential to cause birth defects. Female patients of childbearing age must take measures to prevent pregnancy and take a monthly pregnancy test.
Treprostinil (Tyvaso®) is indicated for the treatment of PAH (WHO Group I) in patients with NYHA Class III symptoms, to increase walk distance. It is intended for inhalation administration via the Tyvaso Inhalation System.
Treprostinil (Remodulin®) is indicated for the treatment of PAH in patients with NYHA Class II through Class IV symptoms to diminish symptoms associated with exercise. Remodulin is a prostacyclin that works similarly to Flolan. It causes direct vasodilation of pulmonary and systemic arterial vascular beds and inhibits platelet aggregation. This decreases pulmonary arterial pressure and may prolong survival in PAH patients.
Iloprost (Ventavis®) is indicated for the treatment of PAH (WHO Group I) in patients with NYHA Class III or IV symptoms. Ventavis is a synthetic analogue of prostacyclin PGI2. It dilates systemic and pulmonary arterial vascular beds and affects platelet aggregation. It is intended for inhalation administration only via either of two pulmonary drug delivery devices: the I-neb® AAD® System or the Prodose® AAD® System.
Sildenafil (Revatio®) and tadalafil (Adcirca®) are indicated for the treatment of PAH (WHO Group I) to improve exercise ability. Revatio and Adcirca are inhibitors of cGMP specific phosphodiesterase type-5 (PDE5) in the smooth muscle of the pulmonary vasculature, where PDE5 is responsible for degradation of cGMP. They, therefore, increase cGMP within pulmonary vascular smooth muscle cells resulting in relaxation. In PAH patients, this can lead to vasodilation of the pulmonary vascular bed and, to a lesser degree, vasodilatation in the systemic circulation.
Ambrisentan (Letairis®) is indicated for the treatment of PAH in patients with WHO Class II or III symptoms to improve exercise capacity and delay clinical worsening. Letairis® is an endothelin receptor antagonist that is selective for the endothelin type A (ETA) receptor. Activation of the ETA receptor by endothelin, a small peptide hormone, leads to vasoconstriction and cell proliferation. Because of the risks of liver injury and birth defects, Letairis® is available only through a special restricted distribution program called the Letairis® Education and Access Program (LEAP).
Riociguat (Adempas®) is a soluble guanylate cyclase stimulator 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 and indicated for the treatment of adults with pulmonary arterial hypertension WHO Group 1 to improve exercise capacity, improve WHO functional class and to delay clinical worsening.
Macitentan (Opsumit®)is an endothelin receptor antagonist indicated for the treatment of pulmonary arterial hypertension WHO Group 1 to delay disease progression.
POLICYPrior authorization is required.
Epoprostenol (Flolan®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with NYHA Class III or Class IV symptoms (revised 10-27-2005).
Bosentan (Tracleer®) therapy is considered medically necessary for the treatment of pulmonary arterial hypertension in patients that meet the following criteria:
Treprostinil (Remodulin®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with NYHA Class II through Class IV symptoms.
Macitentan (Opsumit®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients that meet the following criteria:
Riociguat (Adempas®) is not covered.
The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.
POLICY HISTORY10/15/2002: Approved by Pharmacy & Therapeutics (P & T) Committee
10/27/2005: Description section updated. Policy section updated: changed preferred provider to Accredo. Phone # 1-800-235-8498 changed to 1-866-240-3373. Fax # changed from 1-888-355-6682 to 1-800-711-3526. Sources updated: Ventavis® and Revatio® added.
11/4/2005: Code Reference section updated, HCPCS codes Q4077, S0090 added; the drug Iloprost (Ventavis®) was added to the descriptor for code J3490
11/2005: Approved by Pharmacy and Therapeutics (P&T) Committee
2/6/2006: Code Reference table updated; codes J3285 and Q4080 added, deletion date added to codes Q4077 and S0114
3/14/2006: Coding updated. HCPCS 2006 revisions added to policy.
3/22/2006: Policy reviewed, no changes
7/16/2007: Ambrisentan (Letairis®) added to policy as medically necessary
12/19/2007: Coding updated per 2008 CPT/HCPCS revisions
01/01/2009: Accredo preferred provider information removed. BCBSMS information added.
8/10/2009: Tadalafil (Adcirca®) information added to the Policy Description section, Policy Statement section updated to include Tadalafil (Adcirca®) medically necessary information, and Source section updated to include Tadalafil (Adcirca®) Prescribing Information as a reference, Added Tadalafil (Adcirca®) to unclassified code J3490 under Covered table, Removed deleted HCPCS codes Q4077 and S0114 from Covered table. Added ICD-9 Diagnosis codes 416.1 and 416.9 to covered table.
9/4/2009: Policy updated with the addition of Treprostinil (Tyvaso®). Description section and policy statement section updated to include Treprostinil (Tyvaso®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with NYHA Class III symptoms. Sources section updated with addition of Tracleer® and Tyvaso® Prescribing Information as sources. Code reference section updated with Tyvaso® added to HCPC code J3285
12/15/2009: Coding Section revised for 2010 CPT4 and HCPCS revisions
02/28/2011: Added new HCPCS code J7686 to the Code Reference section.
03/18/2014: Policy updated to include Macitentan (Opsumit®) and Riociguat (Adempas®) in the policy description and policy statement. Removed deleted HCPCS code Q4080 from the Code Reference section.
SOURCE(S)Facts and Comparisons
American Hospital Formulary Service
Letairis® Prescribing Information
Adcirca® Prescribing Information
Tracleer® Prescribing Information
Tyvaso® Prescribing Information
Opsumit® Prescribing Information
Adempas® Prescribing Information
CODE REFERENCEThis may not be a comprehensive list of procedure codes applicable to this policy.
The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.