Wilken E, Bennji S, Symons G, Williams P G, Allwood B
Division of Pulmonology, Department of Medicine, Tygerberg Hospital and University of Stellenbosch, Cape Town, South Africa.
Division of Pulmonology, Department of Medicine, University of Cape Town, South Africa.
Afr J Thorac Crit Care Med. 2019 Apr 12;25(1). doi: 10.7196/SARJ.2019.v25i1.236. eCollection 2019.
Pulmonary hypertension (PH) has traditionally been considered a rare disease with a uniformly poor prognosis. However, this was prior to the introduction of advanced therapies for this condition, and more recent registries in the treatment era have shown 5-year survival rates of up to 65%. Prior to 2000, there was only one licensed therapy for pulmonary arterial hypertension (PAH); less than 20 years later, the US Food and Drug Administration has approved 14 different medications for PAH. This review aims to summarise for the general pulmonologist the evidence for the current internationally available advanced therapies for PAH (World Health Organization Group I disease), which is characterised haemodynamically by the presence of precapillary PH in the absence of another cause. The benefit of these agents, either alone or in combinations, is now undisputed and their use is advocated in all current international guidelines for PAH. The improvement in survival of patients with PAH over the concurrent timeline emphasises the importance both of the availability and usage of effective therapies and of patients being seen in specialist centres, where physicians are familiar with using these therapies.
肺动脉高压（PH）传统上被认为是一种罕见疾病，预后普遍较差。然而，这是在针对该病症引入先进疗法之前的情况，而治疗时代的最新登记数据显示，5年生存率高达65%。2000年之前，肺动脉高压（PAH）仅有一种获批疗法；不到20年后，美国食品药品监督管理局已批准了14种不同的PAH治疗药物。本综述旨在为普通肺科医生总结目前国际上可获得的PAH先进疗法（世界卫生组织第一组疾病）的证据，该疾病在血流动力学上的特征是在无其他病因的情况下存在毛细血管前性PH。这些药物单独使用或联合使用的益处现在已无可争议，并且在所有当前的PAH国际指南中都提倡使用。PAH患者在同一时期生存率的提高强调了有效疗法的可及性和使用以及患者在专科中心接受诊治的重要性，在专科中心医生熟悉使用这些疗法。
