肺纤维化合并肺气肿综合征并发肺动脉高压患者的临床特征分析  

Clinical Characteristics of Patients With Combined Pulmonary Fibrosis and Emphysema Complicating With Pulmonary Arterial Hypertension

作　　者：曾绮娴[1] 罗勤[1] 赵青[1] 赵智慧[1] 杨涛[1] 柳志红[1] 熊长明 ZENG Qixian;LUO Qin;ZHAO Qing;ZHAO Zhihui;YANG Tao;LIU Zhihong;XIONG Changming(Pulmonary Vascular Disease Center,National Center for Cardiovascular Diseases and Fuwai Hospital,CAMS and PUMC,Beijing 100037,China)

机构地区：[1]中国医学科学院,北京协和医学院,国家心血管病中心,阜外医院肺血管病中心,北京市100037

出　　处：《中国循环杂志》2020年第10期1000-1005,共6页Chinese Circulation Journal

基　　金：首都卫生发展科研专项课题(2016-2-4036);中国医学科学院医学与健康科技创新工程项目(2016-I2M-3-006)。

摘　　要：目的:探讨肺纤维化合并肺气肿综合征(CPFE)并发肺动脉高压患者的临床、肺功能、影像学及血流动力学特点。方法:选取2017~2019年中国医学科学院阜外医院肺血管病中心收治的12例临床诊断为CPFE并发肺动脉高压患者为研究对象,收集患者临床基本资料及肺功能、影像学及血流动力学等资料进行分析。结果:12例CPFE并发肺动脉高压患者中,10例为男性,均为长期大量吸烟患者,平均年龄(61.25±8.31)岁。肺功能检查结果显示,患者通气功能基本正常或轻度下降,第一秒用力呼气容积占预计值百分比为(79.60±10.82)%,第一秒用力呼气容积与用力肺活量比值占预计值(95.90±11.14)%,小气道阻力中度增加,弥散功能严重受损,一氧化碳弥散量占预计值(28.8±8.04)%。胸部高分辨CT提示,患者有不同程度肺纤维化、肺气肿,部分患者可见纵隔淋巴结增大及小叶间隔增厚等表现,其中肺纤维化者12例,多分布于双肺下叶(41.7%);肺气肿者11例(91.7%),可局限于双上肺,也可呈双肺弥漫分布。右心导管检查提示,CPFE患者为毛细血管前性肺动脉高压,肺动脉平均压为(43.45±11.05) mmHg(1 mmHg=0.133 kPa),肺血管阻力显著升高为(10.25±5.12)Wood单位,心指数下降为(2.81±0.69)L/(min·m^2)。结论:CPFE并发肺动脉高压多见于长期吸烟的老年男性,肺功能结合胸部高分辨CT及血流动力学检查对临床诊断及病情评估具有重要作用。Objectives:To investigate the clinical characteristics of patients with combined pulmonary fibrosis and emphysema(CPFE)complicating with pulmonary arterial hypertension.Methods:Patients admitted to Fuwai hospital from 2017 to 2019 and diagnosed with CPFE were retrospectively screened.Demographic characteristics,pulmonary function,chest high-resolution CT imaging findings and hemodynamic data were analyzed.Results:A total of 12 CPFE patients with pulmonary arterial hypertension were included in this study.Average age of patients was(61.25±8.31)years old.Ten out of the 12 CPFE patients were male heavy smokers,Spirometry results demonstrated relatively preserved ventilation function,forced expiratory volume in one second(FEV1)was(79.60±10.82)%of the predicted value and FEV1/forced vital capacity(FVC)was(95.90±11.14)%of predicted value.However,the diffusing capacity of the lung for carbon monoxide(DLCO)were remarkably decreased(28.8%±8.04%pred).Pulmonary fibrosis,emphysema,lymph node enlargement and interlobular septal thickening were presented on chest high-resolution CT imaging.In particular,all 12 patients demonstrated pulmonary fibrosis,predominantly in the lower lobes(41.7%).Emphysema was evidenced in 11 patients,locating mainly on the upper lobes or diffusely distributed.Right heart catheterization demonstrated significant pre-capillary pulmonary arterial hypertension(mean pulmonary arterial pressure:[43.45±11.05]mmHg,1 mmHg=0.133 kPa),significantly increased pulmonary vascular resistance([10.25±5.12]Wood unit)and significantly reduced cardiac index([2.81±0.69]L/[min·m2]).Conclusions:CPFE patients with pulmonary arterial hypertension are susceptible among old male population with a heavy smoking history.Pulmonary test in combination with high-resolution chest CT scan and hemodynamic examination are of importance for accurate and early diagnosis of CPFE with pulmonary arterial hypertension.Once diagnosis is confirmed,timely decision making for lung transplantation is required.

关 键 词：肺纤维化 肺气肿 肺动脉高压 血流动力学 

分 类 号：R541[医药卫生—心血管疾病]