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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).

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:

• WHO Class II to Class IV symptoms
• Baseline serum aminotransferase (ALT and AST) <3 x upper limits of normal prior to initiation of treatment monthly and thereafter.
• Pregnancy must be excluded before the start of treatment and prevented thereafter by the use of a reliable method of contraception. Hormonal contraceptives, including oral, injectable, transdermal and implantable contraceptives should not be used as a sole means of contraception because these may not be effective in patients receiving bosentan (Tracleer®). Pregnancy testing is required prior to initiation of therapy and monthly thereafter.

Treprostinil (Remodulin®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with NYHA Class II through Class IV symptoms.

Treprostinil (Tyvaso®)  is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with NYHA Class III symptoms.

Iloprost (Ventavis®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with NYHA Class III or IV symptoms (added 10-27-2005).

Sildenafil (Revatio®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with WHO Group I symptoms (added 10-27-2005).

Tadalafil (Adcirca®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with WHO Group I symptoms.

Ambrisentan (Letairis®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients that meet the following criteria:

• WHO Class II of Class III symptoms
• Baseline serum amniotransferase (ALT and AST) <3 x upper limits of normal prior to initiation of treatment and monthly thereafter.
• Pregnancy must be excluded before the initiation of treatment and prevented thereafter by the use of at least two reliable methods of contraception unless the patient is unable to become pregnant. In women who can become pregnant, pregnancy tests should be obtained monthly.

None

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

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.

FDA website

American Hospital Formulary Service

Letairis® Prescribing Information

Adcirca® Prescribing Information

Tracleer® Prescribing Information

Tyvaso® Prescribing Information

The code(s) listed below are ONLY covered if the procedure is performed according to the "Policy" section of this document.

Covered Codes

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