Shanghai Pulmonary Hospital, Tongji University, Shanghai, China.
Respir Med. 2009 Aug;103(8):1136-42. doi: 10.1016/j.rmed.2009.03.009. Epub 2009 Apr 28.
Although previous studies have shown that peripheral airway obstruction can occur in idiopathic PAH (IPAH), pulmonary function tests have not been well-studied in patients with PAH associated with congenital heart disease (CHD-PAH) and connective tissue disease (CTD-PAH).
A multicenter prospective study was performed in PAH patients in China. Pulmonary function tests were evaluated in 190 PAH patients.
Total lung capacity (TLC), residual volume (RV) and total airway resistance (Rtot) were similar in PAH patients and controls. However, measures of airflow, including vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1s (FEV(1)), FEV(1)/FVC and MEF(50) were decreased in PAH group. Single-breath diffusion capacity for carbon monoxide (DL(CO)) was also decreased in PAH patients. Expiratory flow-volume curves showed reduction and a curvilinear appearance in patients with PAH. Similar changes were observed among the various subgroups of IPAH, CHD-PAH, and CTD-PAH patients. More CTD-PAH patients had abnormal DL(CO).
Airway obstruction is common in IPAH, CHD-PAH and CTD-PAH patients. CTD-PAH patients have lower DL(CO). Hemodynamics, serum markers and exercise capacity parameters did not correlate well with pulmonary function indices.
虽然先前的研究表明特发性肺动脉高压（IPAH）可出现外周气道阻塞，但肺动脉高压伴先天性心脏病（CHD-PAH）和结缔组织疾病（CTD-PAH）患者的肺功能检查尚未得到充分研究。
在中国进行了一项多中心前瞻性研究，共评估了 190 例肺动脉高压患者的肺功能。
肺动脉高压患者的肺总量（TLC）、残气量（RV）和总气道阻力（Rtot）与对照组相似，但包括肺活量（VC）、用力肺活量（FVC）、1 秒用力呼气量（FEV1）、FEV1/FVC 和 MEF50 等气流受限指标均降低。一氧化碳弥散量（DLCO）也在肺动脉高压患者中降低。用力呼气流量-容积曲线显示肺动脉高压患者存在流量减少和曲线呈线性外观。在 IPAH、CHD-PAH 和 CTD-PAH 患者的各个亚组中也观察到了类似的变化。更多的 CTD-PAH 患者存在 DLCO 异常。
气道阻塞在 IPAH、CHD-PAH 和 CTD-PAH 患者中很常见。CTD-PAH 患者的 DLCO 较低。血流动力学、血清标志物和运动能力参数与肺功能指标相关性不佳。
