Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - Centre de référence des maladies pulmonaires rares, Université de Lyon, Université Lyon I, UCBL-INRA-ENVL-EPHE, UMR754, IFR128, Lyon, France.
Eur Respir J. 2010 Jan;35(1):105-11. doi: 10.1183/09031936.00038709. Epub 2009 Jul 30.
This study aims to describe the haemodynamic and survival characteristics of patients with pulmonary hypertension in the recently individualised syndrome of combined pulmonary fibrosis and emphysema. A retrospective multicentre study was conducted in 40 patients (38 males; age 68+/-9 yrs; 39 smokers) with combined pulmonary fibrosis and emphysema, and pulmonary hypertension at right heart catheterisation. Dyspnoea was functional class II in 15%, III in 55% and IV in 30%. 6-min walk distance was 244+/-126 m. Forced vital capacity was 86+/-18%, forced expiratory volume in 1 s 78+/-19%, and carbon monoxide diffusion transfer coefficient 28+/-16% of predicted. Room air arterial oxygen tension was 7.5+/-1.6 kPa (56+/-12 mmHg). Mean pulmonary artery pressure was 40+/-9 mmHg, cardiac index 2.5+/-0.7 L x min(-1) x m(-2) and pulmonary vascular resistance 521+/-205 dyn x s x cm(-5). 1-yr survival was 60%. Higher pulmonary vascular resistance, higher heart rate, lower cardiac index and lower carbon monoxide diffusion transfer were associated with shorter survival. Patients with combined pulmonary fibrosis and emphysema syndrome and pulmonary hypertension confirmed by right heart catheterisation have a dismal prognosis despite moderately altered lung volumes and flows and moderately severe haemodynamic parameters.
这项研究旨在描述特发性肺纤维化合并肺气肿（CPFE）患者合并肺动脉高压的血流动力学和生存特征。在 40 名 CPFE 合并肺动脉高压的患者（38 名男性；年龄 68+/-9 岁；39 名吸烟者）中进行了一项回顾性多中心研究。右心导管检查显示，呼吸困难为 II 级（15%）、III 级（55%）和 IV 级（30%）。6 分钟步行距离为 244+/-126m。用力肺活量为 86+/-18%，1 秒用力呼气量为 78+/-19%，一氧化碳弥散转移系数为预测值的 28+/-16%。空气动脉血氧分压为 7.5+/-1.6 kPa（56+/-12 mmHg）。平均肺动脉压为 40+/-9 mmHg，心指数为 2.5+/-0.7 L x min(-1) x m(-2)，肺血管阻力为 521+/-205 dyn x s x cm(-5)。1 年生存率为 60%。较高的肺血管阻力、较高的心率、较低的心指数和较低的一氧化碳弥散转移与较短的生存时间相关。尽管肺容积和流量中度改变，血流动力学参数中度严重，但经右心导管检查证实的 CPFE 合并肺动脉高压患者预后不佳。
