Pulmonary arterial hypertension leads to significant impairment in haemodynamics, right heart function, exercise capacity, quality of life and survival. Current therapies have mechanisms of action involving signalling via one of four pathways: endothelin-1, nitric oxide, prostacyclin and bone morphogenetic protein/activin signalling. Efficacy has generally been greater with therapeutic combinations and with parenteral therapy compared with monotherapy or nonparenteral therapies, and maximal medical therapy is now four-drug therapy. Lung transplantation remains an option for selected patients with an inadequate response to therapies.

中文翻译：

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肺动脉高压的治疗流程

肺动脉高压导致血流动力学、右心功能、运动能力、生活质量和生存率的严重损害。目前的疗法具有作用机制，涉及通过以下四种途径之一进行信号传导：内皮素-1、一氧化氮、前列环素和骨形态发生蛋白/激活素信号传导。与单药治疗或非肠外治疗相比，联合治疗和肠外治疗的疗效通常更高，现在最大程度的药物治疗是四药治疗。肺移植仍然是对治疗反应不足的特定患者的一种选择。