20.4 : 肺动脉高压的治疗：前列环素受体激动剂

Prostacyclin receptor agonists are vital in managing pulmonary arterial hypertension.

They mimic prostaglandin I₂  and bind to its receptor, IPR, on the smooth muscle cells. This triggers the adenylyl cyclase pathway, ultimately leading to smooth muscle relaxation and vasodilation.

Notable prostacyclin receptor agonists include epoprostenol, treprostinil, iloprost, beraprost, and selexipag.

Epoprostenol is administered via continuous intravenous infusion and has a short half-life.

Treprostinil can be administered via multiple routes, including continuous intravenous and subcutaneous infusions, inhalation, and oral.

Iloprost, available as an inhaled formulation, causes vasodilation in the lung's circulatory system.

Beraprost, the first orally available prostaglandin I₂ analog, showed no benefit during long-term trials.

Selexipag, a selective IPR agonist, is rapidly absorbed and has a longer half-life.

Possible adverse effects of these agonists include musculoskeletal pain, headaches, abdominal discomfort, and systemic hypotension.