Madonna Rosalinda, Cocco Nino
Institute of Cardiology, Center of Excellence on Aging, "G. d'Annunzio" University, Chieti, Italy.
Curr Drug Targets. 2016;17(7):817-23. doi: 10.2174/1389450116666150722140424.
Pulmonary arterial hypertension (PAH) is a pathophysiological condition characterized by increased pulmonary vascular resistance (PVR), initially due to abnormal pulmonary vasoconstriction in response to endothelial injury. Recent studies confirmed the key role of endothelin (ET)-1 in the vasoconstriction and remodeling of pulmonary microcirculation during PAH. In responders patients, classical treatments for PAH are prostanoids, phosphodiesterase (PDE)-5 inhibitors and endothelin receptor antagonists (ERAs), which target prostaglandin I2, nitric oxide and endothelin pathways, respectively. Randomised, placebo-controlled trials have shown that ERAs improves haemodynamic parameters of the pulmonary circulation, functional capacity and clinical outcome in patients affected by PAH. Here, we will review the definition, classification and pathophysiology of PH. Furthermore, we will provide an up-to-date overview of currently recommended diagnostic and therapeutic work-up in PAH.
肺动脉高压（PAH）是一种病理生理状态，其特征为肺血管阻力（PVR）增加，最初是由于对内皮损伤的异常肺血管收缩所致。最近的研究证实了内皮素（ET）-1在PAH期间肺微循环的血管收缩和重塑中的关键作用。在反应者患者中，PAH的经典治疗方法是前列环素、磷酸二酯酶（PDE）-5抑制剂和内皮素受体拮抗剂（ERA），它们分别靶向前列腺素I2、一氧化氮和内皮素途径。随机、安慰剂对照试验表明，ERA可改善PAH患者的肺循环血流动力学参数、功能能力和临床结局。在此，我们将综述肺动脉高压的定义、分类和病理生理学。此外，我们将提供PAH目前推荐的诊断和治疗检查的最新概述。
