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吴琼,姜蓉,宫素岗,等.特发性与先心病相关性肺动脉高压患者小气道功能差异的比较研究[J].同济大学学报（医学版）,2019,40(1):98-104. [点击复制]
WU Qiong,JIANG Rong,GONG Su-gang,et al.Differences in peripheral small airway dysfunction between idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease[J].同济大学学报（医学版）,2019,40(1):98-104. [点击复制]

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特发性与先心病相关性肺动脉高压患者小气道功能差异的比较研究
吴琼,姜蓉,宫素岗,刘锦铭
0 字体:加大+\|默认\|缩小-
(同济大学附属上海市肺科医院肺循环科，上海 200433)

摘要:

目的比较特发性肺动脉高压（idiopathic pulmonary arterial hypertension, IPAH）与先天性心脏病相关性肺动脉高压（pulmonary arterial hypertension associated with congenital heart disease, PAH-CHD）患者小气道功能的差异。方法 50例确诊为IPAH的患者、44例PAH-CHD患者及24位健康人群（对照组），进行常规肺功能检查（pulmonary function test, PFT)及脉冲振荡肺功能（impulse oscillometry lung function, IOS）检查，分析IPAH与PAH-CHD患者肺功能小气道变化特点。结果与健康对照组相比，IPAH和PAH-CHD患者外周小气道阻力参数，如75%肺活量时的最大呼气流量占预计值百分比（MEF25%pred）、50%肺活量时的最大呼气流量占预计值百分比（MEF50%pred）、25%肺活量时的最大呼气流量占预计值百分比（MEF75%pred）、最大呼气中期流速占预计值百分比（MMEF75/25%pred）均降低，ΔR5-R20均升高。与IPAH患者相比，PAH-CHD患者病程更长，确诊年龄更年轻，血流动力学更差，小气道功能损害更严重，PFT检测显示MEF25%pred、MEF50%pred、MEF75%pred、MMEF75/25%pred减退更显著。IOS检查发现PAH-CHD患者小气道阻力参数X5较IPAH患者显著升高[1.4(1.1,2.1)vs1.2(0.8,1.6),P<0.05]。亚组分析发现，PAH-CHD患者有无手术矫正史，对其小气道功能无明显影响。结论与IPAH患者相比，PAH-CHD患者确诊PAH时平均年龄更年轻，血流动力学参数更差，小气道功能损害更明显。

关键词: 特发性肺动脉高压先天性心脏病相关性肺动脉高压脉冲振荡肺功能血流动力学

DOI：10.16118/j.1008-0392.2019.01.019

投稿时间：2018-07-17

基金项目:国家卫生计生委医药卫生科技发展研究中心课题（ZX-01-C2016144）；上海市科学技术委员会自然科学基金（16ZR1429000）；国家自然科学基金青年项目（81700045）

Differences in peripheral small airway dysfunction between idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease

WU Qiong,JIANG Rong,GONG Su-gang,LIU Jin-ming

(Dept. of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University,Shanghai 200433, China)

Abstract:

Objective To investigate the differences in peripheral small airway dysfunction between idiopathic pulmonary arterial hypertension(IPAH) and pulmonary arterial hypertension associated with congenital heart disease(PAH-CHD). Methods Impulse oscillmetory lung function(IOS) and pulmonary function testing(PFT) were performed in 50 IPAH, 44 PAH-CHD patients and 24 healthy controls to explore the differences of peripheral small airway dysfunction among groups. Results Compared with control group, peripheral airway function was impaired in IPAH and PAH-CHD patients with decreased value of MEF25%pred, MEF50%pred, MEF75%pred, MMEF75/25%pred and increased value of ΔR5-R20. Compared with IPAH patients, PAH-CHD patients were younger and had more serious pulmonary vessel resistance and mean pulmonary arterial pressure. PAH-CHD patients had significantly impaired peripheral small airway function with decreased MEF25 %pred, MEF50%pred, MEF75%pred, MMEF75/25, as evaluated by PFT, and increased X5 values as measured by IOS (all P< 0.05). Subgroup analysis revealed that there were no significant differences in peripheral small dysfunction between PAH-CHD patients with or without operation. Conclusion Compared with IPAH, PAH-CHD patients are younger, but have worse hemodynamics and peripheral airway function.

Key words: idiopathic pulmonary arterial hypertension pulmonary arterial hypertension associated with congenital heart disease impulse oscillometry lung function hemodynamics.