Pulmonary Hypertension, Drug Management

Restricted Product(s):

• epoprostenol sodium (Flolan®) continuous intravenous infusion for administration by a healthcare professional
• epoprostenol (Veletri®) continuous intravenous infusion for administration by a healthcare professional
• treprostinil sodium (Remodulin®) continuous subcutaneous infusion or intravenous infusion for administration by a healthcare professional
• iloprost (Ventavis®) inhalation via nebulizer
• sotatercept-crsk (Winrevair™) subcutaneous injection for administration by a healthcare professional
• treprostinil (Tyvaso®) via inhalation system

FDA Approved Use:

• Epoprostenol sodium (Flolan^®)
  • For treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity
• Epoprostenol (Veletri®) 
  • For treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity
• Treprostinil sodium (Remodulin®)
  • For treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to diminish symptoms associated with exercise
  • For patients who require transition from epoprostenol, to reduce the rate of clinical deterioration
• Iloprost (Ventavis®)
  • Treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve a composite endpoint consisting of exercise tolerance, symptoms (NYHA Class), and lack of deterioration
• Sotatercept-crsk (Winrevair™)
  • For treatment of adults with pulmonary arterial hypertension (PAH; WHO Group 1) to increase exercise capacity, improve WHO functional class (FC) and reduce the risk of clinical worsening events
• Treprostinil (Tyvaso®) 
  • For treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to improve exercise ability
  • For treatment of pulmonary hypertension associated with interstitial lung disease (PH-ILD; WHO Group 3) to improve exercise ability

Criteria for Medical Necessity:

The restricted product(s) may be considered medically necessary when the following criteria are met:

Initial Criteria for Approval:

1. The requested agent is epoprostenol sodium (Flolan), epoprostenol (Veletri), or iloprost (Ventavis); AND
   1. The patient has a diagnosis of pulmonary arterial hypertension (PAH; WHO Group 1) [medical record documentation required];OR
2. The requested agent is treprostinil sodium (Remodulin); AND
   1. The patient has a diagnosis of pulmonary arterial hypertension (PAH; WHO Group 1) [medical record documentation required]; AND
   2. If the requested agent is brand Remodulin, ONE of the following:
      1. The patient has tried and had an inadequate response to generic treprostinil sodium [medical record documentation required]; OR
      2. The patient has an intolerance, FDA labeled contraindication, or hypersensitivity to generic treprostinil sodium that is not expected to occur with the requested agent [medical record documentation required]; OR
3. The requested agent is treprostinil (Tyvaso); AND
   1. The patient has a diagnosis of pulmonary arterial hypertension (PAH; WHO Group 1) [medical record documentation required]; OR
   2. The patient has a diagnosis of pulmonary hypertension associated with interstitial lung disease (PH-ILD; WHO Group 3) [medical record documentation required]; OR
4. The requested agent is sotatercept-crsk (Winrevair); AND
   1. The patient is 18 years of age or older; AND
   2. The patient has a diagnosis of pulmonary arterial hypertension (PAH; WHO Group 1) (PAH/WHO Group 1) [medical record documentation required]; AND
   3. The patient has been stable on background PAH therapy for at least 90 days (i.e., monotherapy or combination therapy of the following drug classes: ERA, PDE5i, soluble guanylate cyclase stimulator, and/or prostacyclin analogue or receptor agonist); AND
5. The requested quantity does NOT exceed the maximum units allowed for the duration of approval (see table below).

Duration of Approval: 365 days (1 year)

Continuation Criteria for Approval: 

1. The patient was approved through Blue Cross NC initial criteria for approval; OR 
2. The patient would have met initial criteria for approval at the time they started therapy [medical record documentation required]; AND 
3. The patient has documentation of a positive clinical response since initiation of treatment with the requested agent [medical record documentation required]; AND 
4. If the requested agent is brand Remodulin, ONE of the following:
   1. The patient has tried and had an inadequate response to generic treprostinil sodium [medical record documentation required]; OR
   2. The patient has an intolerance, FDA labeled contraindication, or hypersensitivity to generic treprostinil sodium that is not expected to occur with the requested agent [medical record documentation required]; AND
5. The requested quantity does NOT exceed the maximum units allowed for the duration of approval (see table below). 

Duration of Approval: 365 days (1 year)