Dept of Medicine, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA, USA
Lung Transplant Program, Inova Fairfax Hospital, Falls Church, VA, USA.
Eur Respir J. 2015 Nov;46(5):1370-7. doi: 10.1183/13993003.01537-2014. Epub 2015 Aug 6.
The clinical course of pulmonary hypertension (PH) in idiopathic pulmonary fibrosis (IPF) is not known except in advanced disease.488 subjects in a placebo-controlled study of ambrisentan in IPF with mild-moderate restriction in lung volume, underwent right heart catheterisation (RHC) at baseline and 117 subjects (24%) had repeated haemodynamic measurements at 48 weeks. The subjects were categorised into a) World Health Organization (WHO) Group 3 PH (PH associated with pulmonary disease), n=68 (14%); b) WHO Group 2 PH (PH associated with left-sided cardiac disease), n=25 (5%); c) no PH but elevated pulmonary artery wedge pressure (PAWP), n=21 (4%); and d) no PH but without elevation of PAWP, n=374 (77%). The WHO Group 3 PH subjects had a lower diffusion capacity, 6MWD and oxygen saturation compared to the subjects with no PH. There was no significant change in mean pulmonary arterial pressure with ambrisenten or placebo after 12 months. Subjects with IPF associated with WHO Group 3 PH had impaired gas exchange and exercise capacity compared to patients without PH. An additional 9% of the subjects had haemodynamic evidence of subclinical left-ventricular dysfunction. Pulmonary artery pressures remained stable over 1 year in the majority of the cohort.
特发性肺纤维化（IPF）患者的肺动脉高压（PH）临床病程除了在疾病晚期外并不明确。488 名在特发性肺纤维化中接受安立生坦安慰剂对照研究的受试者在基线和 48 周时接受了右心导管检查（RHC），其中 117 名受试者（24%）进行了重复血流动力学测量。这些受试者被分为 a）世界卫生组织（WHO）第 3 组 PH（与肺部疾病相关的 PH），n=68（14%）；b）WHO 第 2 组 PH（与左心疾病相关的 PH），n=25（5%）；c）无 PH 但肺动脉楔压升高（PAWP），n=21（4%）；和 d）无 PH 但无 PAWP 升高，n=374（77%）。与无 PH 的受试者相比，WHO 第 3 组 PH 受试者的弥散能力、6MWD 和氧饱和度较低。使用安立生坦或安慰剂 12 个月后，平均肺动脉压没有明显变化。与无 PH 的患者相比，与 IPF 相关的 WHO 第 3 组 PH 患者的气体交换和运动能力受损。另外 9%的患者有亚临床左心室功能障碍的血流动力学证据。在大多数患者中，肺动脉压在 1 年内保持稳定。
