不同类型肺动脉高压患者临床特征和血流动力学的比较分析

摘要

目的:比较不同类型肺动脉高压患者的人口学特征、临床特征以及血流动力学方面的差异.方法:选取2007-05至2010-10由我院牵头的全国31家三级甲等医院经右心导管检查、符合美国心脏学院基金会/美国心脏学会颁布的肺动脉压专家共识确诊标准的、4种不同类型的成人肺动脉高压患者551例,女性386例(70.1％),其中特发性肺动脉高压150例(IPAH组)、先天性心脏病相关肺动脉高压273例(CHD-PAH组)、结缔组织病相关脉动脉高压64例(CTD-PAH组)和慢性血栓塞性肺动脉高压64例(CTEPH组),收集并比较分析不同类型肺动高压患者的人口学、功能分级和血流动力学指标的差异.结果:551例患者中CHD-PAH组患者病例数最多(273例,49.5％)；CHD-PAH组年龄最小,体质指数最低,而CTEPH组则年龄大.CTD-PAH组的女男比例最高.551例患者以乏力(76.4％)和呼吸困难(722％)为最常见的症状；CTD-PAH组的患者6分钟步行距离(6 MWD)最短,CHD-PAH组的患者6 MWD最长.CHD-PAH组的患者世界卫生组织肺动脉高压功能分级(WHO-FC)以Ⅱ级患者最多.CHD-PAH组患者肺动脉平均压和肺血管阻力均显著高于其它3组的患者,差异有统计学意义(P ＜0.01).结论:CTD-PAH好发于中青年女性,多数IPAH患者确诊时病情较重,就诊较晚；CHD-PAH最常见,其肺动脉压力和肺血管阻力最高,而WHO-FC和运动耐量较好,提示先天性心脏病相关性肺动脉高压患者心功能与肺动脉压力和肺血管阻力并不一致.%Objective:To analyze the demography,clinical features and hemodynamics in patients with different types of pulmonary arterial hypertension (PAH).Methods:We retrospectively studied 551 PAH patients who were treated at 31 medical centers in China from May 2007 to October 2010.The diagnosis was confirmed by right heart catheterization,and 386 (70.1％) patients with female gender.According to the standard of American Heart Association,there were 4 types of PAH including idiopathic PAH (IPAH group),n=150,PAH associated with congenital heart disease (CHD-PAH group),n=273,PAH associated with connective tissue disease (CTD-PAH group),n=64,and chronic thromboembolie PAH (CTEPH),n=64.The demography,WHO functional classification (WHO-FC) and hemodynamics were collected and compared among different PAH groups.Results:CHD-PAH group had the largest number of patients (273/551,49.5％),with the youngest age and lowest BMI.While CTEPH group was opposite from CHD-PAH group in age and BMI,it had the highest ratio of female/male gender.Overall,fatigue (76.4％) and breathlessness (72.2％) were the most common symptoms in PAH patients.CTD-PAH group had the shortest 6 minutes walking distance (6MWD),and CHD-PAH group had the longest 6MWD.There were more patients with WHO-FC Ⅱ in CHD-PAH group,and the mean pulmonary arterial pressure (mPAP),pulmonary vascular resistance (PVR) in CHD-PAH group was obviously higher than those in the other 3 groups,P ＜ 0.01 respectively.Conclusion:With confirmed diagnosis,more IPAH patients were already at the severe condition.CHD-PAH was the most common type and it more happened in young and middle aged female gender,it had the highest mPAP and PVP,while rather good WHO-FC,which implies that the cardiac function may not be parallel to mPAP and PVR in those patients.