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
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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 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.
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.
引文
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[15]LAI Y L,OLSON J W,GILLESPIE M N.Ventilatory dysfunction precedes pulmonary vascular changes in monocrotaline-treated rats[J].J Appl Physiol,1991,70:561-566.
[16]STEINBACHER M,PFLEGER A,SCHWANTZERG,et al.Small airway function before and after cold dry air challenge in pediatric asthma patients during remission[J].Pediatr Pulmonol,2017,52(7):873-879.
[17]SORKNESS R L,BLEECKER E R,BUSSE W W,et al.Lung function in adults with stable but severe asthma:air trapping and incomplete reversal of obstruction with bronchodilation[J].J Applied Physiol,2008,104(2):394-403.
[18]STRANGE G,ROSE M,KERMEEN F,et al.A binational registry of adults with pulmonary arterial hypertension complicating congenital heart disease[J].Intern Med J,2015,45(9):944-950.
[19]GALIE N,MANES A,PALAZZINI M,et al.Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome[J].Drugs,2008,68:1049-1046.
[20]杨国玲,袁平,郭健,等.心肺运动试验参数预测特发性肺动脉高压患者肺血管阻力升高的性别差异[J].同济大学学报(医学版),2018,39(1):64-69.
[21]DILLER G P,GATZOULIS M A.Pulmonary vascular disease in adults with congenital heart disease[J].Circulation,2007,115:1039-1050.
[22]BAUMGARTNER H,BONHOEFFER P,DE GROOTN M,et al.ESC Guidelines for the management of grown-up congenital heart disease(new version 2010)[J].Eur Heart J,2010,31:2915-2957.
[2]RUNO J R,Loyd J E.Primary pulmonary hypertension[J].Lancet,2003,361(9368):1533-1544.
[3]中国医师学会心血管内科医师分会.2015年先天性心脏病相关性肺动脉高压诊治中国专家共识[J].中国介入心脏病学杂志,2015,23(1):61-69.
[4]HOEPER M M,BOGAARD H J,CONDLIFFE ROBIN,et al.Definitions and diagnosis of pulmonary hypertension[J].J Am Coll Cardiol,2013,62(25 Suppl):42-50.
[5]LIU L,LIU W,LIU C H,et al.Study on small airway function in asthmatics with fractional exhaled nitric oxide and impulse oscillometry[J].Clin Respir J,2018,12(2):483-490.
[6]ANDERSON W J,ZAJDA E.Are we overlooking persistent small airways dysfunction in communitymanaged asthma?[J].Ann Allergy Asthma Immunol,2012,109(3):185-189,e2.
[7]GALIE N,MARC H,JEAN-LUC V,et al.2015ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension:the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology(ESC)and the European Respiratory Society(ERS):endorsed by:Association for European Paediatric and Congenital Cardiology(AEPC),International Society for Heart and Lung Transplantation(ISHLT)[J].Eur Heart J,2016,37(1):67-119.
[8]SIMONNEAU G,IVAN M R,MAURICE B,et al.Updated clinical classification of pulmonary hypertension[J].J Am Coll Cardiol,2009,54(1 Suppl):S43-54.
[9]ZHENG J,ZHONG N.Normative values of pulmonary function testing in Chinese adults[J].Chin Med J(Engl),2002,115(1):50-54.
[10]JIANG R,AIZI-SHENG,JIANG X,et al.Intravenousfasudil improves in-hospital mortality of patients with right heart failure in severe pulmonary hypertension[J].Hypertens Res,2015,38(8):539-544.
[11]荆志成,徐希奇,蒋鑫,等.经前臂静脉径路行右心导管检查和肺动脉造影的可行性研究[J].中华心血管病杂志,2009,37(2):142-144.
[12]JING Z C,XU X Q,DAVID B,et al.Pulmonary function testing in patients with pulmonary arterial hypertension[J].Respir Med,2009,103(8):1136-1142.
[13]SPIEKERKOETTER E,FABEL H,HOEPER M M.Effects of inhaled salbutamol in primary pulmonary hypertension[J].Eur Respir J,2002,20:524-528.
[14]INSCORE S C,STENMARK K R,ORTON C,et al.Neonatal calves develop airflow limitation due to chronic hypobaric hypoxia[J].J Appl Physiol,1991,70:384-390.
[15]LAI Y L,OLSON J W,GILLESPIE M N.Ventilatory dysfunction precedes pulmonary vascular changes in monocrotaline-treated rats[J].J Appl Physiol,1991,70:561-566.
[16]STEINBACHER M,PFLEGER A,SCHWANTZERG,et al.Small airway function before and after cold dry air challenge in pediatric asthma patients during remission[J].Pediatr Pulmonol,2017,52(7):873-879.
[17]SORKNESS R L,BLEECKER E R,BUSSE W W,et al.Lung function in adults with stable but severe asthma:air trapping and incomplete reversal of obstruction with bronchodilation[J].J Applied Physiol,2008,104(2):394-403.
[18]STRANGE G,ROSE M,KERMEEN F,et al.A binational registry of adults with pulmonary arterial hypertension complicating congenital heart disease[J].Intern Med J,2015,45(9):944-950.
[19]GALIE N,MANES A,PALAZZINI M,et al.Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome[J].Drugs,2008,68:1049-1046.
[20]杨国玲,袁平,郭健,等.心肺运动试验参数预测特发性肺动脉高压患者肺血管阻力升高的性别差异[J].同济大学学报(医学版),2018,39(1):64-69.
[21]DILLER G P,GATZOULIS M A.Pulmonary vascular disease in adults with congenital heart disease[J].Circulation,2007,115:1039-1050.
[22]BAUMGARTNER H,BONHOEFFER P,DE GROOTN M,et al.ESC Guidelines for the management of grown-up congenital heart disease(new version 2010)[J].Eur Heart J,2010,31:2915-2957.