Oral PAH – NC Standard

Restricted Product(s):

Restriction applies to brand and generic products

• Adcirca® (Tadalafil, Alyq^TM (tadalafil))
• Adempas® (Riociguat)
• Letairis® (Ambrisentan)
• Liqrev® (Sildenafil)
• Opsumit® (Macitentan)
• Opsynvi® (Macitentan-tadalafil)
• Orenitram® (Treprostinil)
• Revatio® (Sildenafil)
• Tadliq® (Tadalafil)
• Tracleer® (Bosentan)
• Uptravi® (Selexipag)

FDA Approved Use:

• Adcirca
  • For the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise ability.
• Adempas
  • For the treatment of persistent/recurrent Chronic Thromboembolic Pulmonary Hypertension (CTEPH) (WHO Group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and WHO functional class.
  • For Pulmonary Arterial Hypertension (PAH) (WHO Group 1) to improve exercise capacity, improve WHO functional class and to delay clinical worsening.
• Letairis
  • For the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1):
    • To improve exercise ability and delay clinical worsening.
    • In combination with tadalafil to reduce the risks of disease progression and hospitalization for worsening PAH, and to improve exercise ability.
• Liqrev
  • For the treatment of pulmonary arterial hypertension (PAH, WHO Group I) in adults to improve exercise ability and delay clinical worsening.
• Opsumit
  • For the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression. Disease progression included: death, initiation of intravenous (IV) or subcutaneous prostanoids, or clinical worsening of PAH (decreased 6-minute walk distance, worsened PAH symptoms and need for additional PAH treatment). OPSUMIT also reduced hospitalization for PAH.
• Opsynvi
  • For the chronic treatment of adults with pulmonary arterial hypertension (PAH, WHO Group I and WHO Functional Class (FC) II–III).
• Orenitram
  • For the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity.
• Revatio
  • In adults, for the treatment of pulmonary arterial hypertension (PAH) (WHO Group I) to improve exercise ability and delay clinical worsening.
  • In pediatric patients aged 1 year and older, for the treatment of pulmonary arterial hypertension (PAH) (WHO Group I) to improve exercise ability and, in pediatric patients too young to perform standard exercise testing, pulmonary hemodynamics thought to underly improvements in exercise.
• Tadliq
  • For the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise ability.
• Tracleer
  • For the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise ability and to decrease clinical worsening:
    • in adults to improve exercise ability and to decrease clinical worsening. Studies establishing effectiveness included predominantly patients with WHO Functional Class II-IV symptoms and etiologies of idiopathic or heritable PAH (60%), PAH associated with connective tissue diseases (21%), and PAH associated with congenital heart disease with left-to-right shunts (18%).
    • in pediatric patients aged 3 years and older with idiopathic or congenital PAH to improve pulmonary vascular resistance (PVR), which is expected to result in an improvement in exercise ability.
• Uptravi
  • For the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression and reduce the risk of hospitalization for PAH.

Criteria for Approval of Adcirca, Liqrev, Revatio, and Tadliq:

1. The requested medication is being used for treatment of pulmonary arterial hypertension (PAH/WHO Group 1); AND
2. The patient’s symptoms are Functional Class 2 through 4; AND
3. The patient is being managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., pulmonologist, cardiologist); AND
4. The patient is not taking requested medication in combination with another Phosphodiesterase-5 Enzyme Inhibitor (e.g., Tadalafil, Sildenafil); AND
5. If the request is for the following brand products: Adcirca or Revatio:
   1. The patient has tried and failed or has a clinical contraindication/intolerance to the generic equivalent of the requested product; AND
6. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies

Criteria for Approval of Letairis, Opsumit, and Tracleer:

1. The requested medication is being used for treatment of pulmonary arterial hypertension (PAH/WHO Group 1); AND
2. The patient’s symptoms are Functional Class 2 through 4; AND
3. The patient is being managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., pulmonologist, cardiologist); AND
4. The patient is not taking requested medication in combination with another Endothelin Receptor Antagonist (e.g., Bosentan, Ambrisentan, Macitentan); AND
5. If the request is for the following brand products: Letairis, Tracleer 62.5mg or 125mg:
   1. The patient has tried and failed or has a clinical contraindication/intolerance to the generic equivalent of the requested product; AND
6. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies

Criteria for Approval of Orenitram or Uptravi:

1. 1. The requested medication is being used for treatment of pulmonary arterial hypertension (PAH/WHO Group 1); AND
2. The patient’s symptoms are Functional Class 2 through 4; AND
3. The patient is being managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., pulmonologist, cardiologist); AND
4. The patient is not taking the requested medication in combination with a prostanoid/prostacyclin analogue (e.g., Epoprostenol, Iloprost, Treprostinil, Selexipag); AND
5. If the request is for the following brand products (Orenitram or Uptravi):
   1. The patient has tried and failed or has a clinical contraindication/intolerance to the generic equivalent of the requested product; AND
6. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies

Criteria for Approval of Adempas:

1. The requested medication is being used for treatment of pulmonary arterial hypertension documented by right heart catheterization or echocardiogram (PAH/WHO Group 1); OR
2. The requested medication is being used for treatment of Chronic Thromboembolic Pulmonary Hypertension (CTEPH) either after surgical treatment or inoperable CTEPH (CTEPH/WHO Group 4); AND
3. The patient’s symptoms are Functional Class 2 through 4; AND
4. The patient is being managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., pulmonologist, cardiologist); AND
5. Adempas will not be used in combination with a phosphodiesterase-5 inhibitor (e.g., sildenafil, tadalafil); AND
6. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.

Criteria for Approval of Opsynvi:

1. The requested medication is being used for treatment of pulmonary arterial hypertension (PAH/WHO Group 1); AND
2. The patient’s symptoms are Functional Class 2 through 3; AND
3. The patient is not pregnant or planning to become pregnant while on therapy with the requested agent; AND
4. The patient is not taking requested medication in combination with another Endothelin Receptor Antagonist or Phosphodiesterase-5 Enzyme Inhibitor (e.g., Bosentan, Ambrisentan, Macitentan, Tadalafil, Sildenafil); AND
5. The patient is being managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., pulmonologist, cardiologist); AND
6. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.

Duration of Approval: 365 days (1 year)

Quantity Limitations:

Quantity limitations apply to brand and associated generic products.