Hahn Stella S, Makaryus Mina, Talwar Arunabh, Narasimhan Mangala, Zaidi Gulrukh
Northwell Health Division of Pulmonary Critical Care and Sleep Medicine, 410 Lakeville Road, Suite 107, New Hyde Park, NY 11042, USA.
Ther Adv Respir Dis. 2017 Jan;11(1):46-63. doi: 10.1177/1753465816665289. Epub 2016 Sep 19.
Pulmonary arterial hypertension (PAH) is an uncommon, progressive and life threatening disease characterized by a proliferative vasculopathy of the small muscular pulmonary arterioles resulting in elevated pulmonary vascular resistance and eventually right ventricular failure. An increasing understanding of the pathobiology of PAH and its natural history has led to the development of numerous targeted therapies. Despite these advances there is significant progression of disease and the survival rate remains low. This article reviews the agents currently available for the medical management of PAH.
肺动脉高压(PAH)是一种罕见、进行性且危及生命的疾病,其特征是小肌性肺小动脉发生增殖性血管病变,导致肺血管阻力升高,最终引发右心室衰竭。对PAH病理生物学及其自然史的认识不断加深,促使了众多靶向治疗方法的发展。尽管取得了这些进展,但疾病仍有显著进展,生存率仍然很低。本文综述了目前可用于PAH药物治疗的药物。