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(M EF25%pred)、50%肺活量时的最大呼气流量占预计值百分比(M EF50%pred)、25%肺活量时的最大呼气流量占预计值百分比(M EF75%pred)、最大呼气中期流速占预计值百分比(M M EF75/25%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M EF25%pred、M EF50%pred、M EF75%pred、M M EF75/25%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。
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 MEF 25%pred,MEF 50%pred,MEF 75%pred,MMEF 75/25%pred and increased value ofΔR5-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 MEF 25%pred,MEF 50%pred,MEF 75%pred,MMEF 75/25,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.
作者
吴琼
姜蓉
宫素岗
刘锦铭
WU Qiong;JIANG Rong;GONG Su-gang;LIU Jin-ming(Dept.of Cardiopulmonary Circulation,Shanghai Pulmonary Hospital,Tongji University,Shanghai 200433,China)
出处
《同济大学学报（医学版）》
CAS
2019年第1期98-104,共7页
Journal of Tongji University(Medical Science)
基金
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)
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
idiopathic pulmonary arterial hypertension
pulmonary arterial hypertension associated with congenital heart disease
impulse oscillometry lung function
hemodynamics
