What are examples of prostacyclins (Prostacyclin Analogues) used to treat Pulmonary Arterial Hypertension (PAH)?

From the Guidelines

Prostacyclins, such as epoprostenol, iloprost, treprostinil, and selexipag, are crucial medications for treating pulmonary arterial hypertension (PAH), with specific administration routes and dosages, including intravenous infusion, inhaled, subcutaneous, and oral formulations. These medications work by promoting vasodilation of pulmonary vessels, inhibiting platelet aggregation, and reducing vascular smooth muscle proliferation, as outlined in the Chest guideline and expert panel report 1. The dosages for these medications are as follows:

• Epoprostenol: 2 ng/kg/min via intravenous infusion, increased as tolerated 1
• Iloprost: 2.5 or 5.0 mg, 6-9 inhalations per day 1
• Treprostinil:
  • Oral: 0.25 mg twice daily or 0.125 mg three times daily, increased by 0.125 mg twice daily every 3-4 days 1
  • Inhaled: 18–54 mg (3-9 inhalations) four times daily 1
  • Subcutaneous or intravenous infusion: 1.25 ng/kg/min, increased by 1.25 ng/kg/min per week based on clinical response, and after week 4, increased by 2.5 ng/kg/min per week based on clinical response 1
• Selexipag: 200 mg twice daily, increased as tolerated to a maximum dose of 1,600 mg twice daily 1. Prostacyclins are often reserved for advanced PAH (WHO Functional Class III or IV) or when patients fail to respond to other therapies, and require careful dose titration and monitoring for side effects such as headache, flushing, jaw pain, diarrhea, and hypotension, as well as specialized training for administration and care of delivery systems to prevent serious complications like infection or interruption of therapy.

From the FDA Drug Label

Epoprostenol for injection is a prostacyclin vasodilator indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity. Treprostinil is a prostacyclin vasodilator indicated for: Treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to diminish symptoms associated with exercise. Treprostinil injection is a prostacyclin vasodilator indicated for: Treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to diminish symptoms associated with exercise.

Examples of prostacyclins for PAH include:

• Epoprostenol (IV) 2
• Treprostinil (SQ) 3
• Treprostinil (IV) 4

From the Research

Prostacyclins for PAH

• The following are examples of prostacyclins used for the treatment of Pulmonary Arterial Hypertension (PAH):
  • Treprostinil 5, 6, 7
  • Epoprostenol 8, 7, 9
  • Iloprost 7, 9
  • Beraprost 9

Characteristics of Prostacyclins

• Prostacyclins are a class of drugs that display vasodilatory effects in the pulmonary vasculature, as well as antiplatelet aggregation properties 6
• They can be administered through various routes, including continuous intravenous infusions, continuous subcutaneous infusions, and intermittent nebulization therapy 8

Clinical Use of Prostacyclins

• Prostacyclins, such as epoprostenol and treprostinil, are considered the gold standard for the treatment of advanced PAH 8
• They have been shown to improve exercise capacity and survival in patients with PAH 7
• The use of prostacyclins in combination with other PAH drugs is an area of ongoing research 9