摘要
目的分析华中地区不同亚型肺动脉高压患者临床表型及血流动力学特征,提高临床诊断及治疗。方法收集2005年1月至2017年4月武汉亚洲心脏病医院收治的556例华中地区肺动脉高压患者的基线资料,按病因学将患者分为特发性肺动脉高压(IPAH)、遗传性肺动脉高压(HPAH)、疾病相关性肺动脉高压(APAH)3组,其中,APAH组分为结缔组织病相关性肺动脉高压(CTD-PAH)、门静脉高压相关性肺动脉高压(portal HT-PAH)、先天性心脏病相关性肺动脉高压(CHD-PAH)3组,比较不同亚型肺动脉高压患者临床表型及血流动力学特征的差异。结果患者年龄中位数为28.2岁,其中女性367例(66.0%)。CHD-PAH组448例,该组患者m PAP较高(F=7.319,P=0.001),但NT-proBNP(F=5.960,P=0.003)较低。IPAH组患者ALT(F=5.833,P=0.007)、SUA(F=6.659,P=0.001)及NTproBNP(F=4.199,P=0.027)较高,心功能Ⅲ/Ⅳ级比例(44.4%)较高(χ~2=23.099,P=0.001)。结论华中地区PAH患者年龄较小且高发于青年女性;CHD-PAH为最常见类型,该型患者mPAP与NT-proBNP的变化趋势不一致;IPAH组患者就诊时肝、肾功能受损,心功能较差。
Objective To analyze clinical phenotypes and hemodynamic characteristics of patients with different subtypes of pulmonary arterial hypertension （PAH）and to improve clinical diagnosis and treatment. Methods From January 2005 to April 2017, SS6 patients with idiopathic pulmonary arterial hypertension, heritable pulmonary arterial hypertension, and accociated pulmonary arterial hypertension （connective tissue disease, portal hypertension and congenital heart disease）were enrolled at Wuhan Asia Heart Hospital.Clinical phenotypes and hemodynamic characteristics were compared among different subtypes groups of PAH. Results Among the 556 cases, there were 367 （66.0%）female and the median age was 28.2 yars.CHD-PAH group （448 cases）had the significantly higher mPAP （F=7.319, P=0.001）. Compared with other groups, NT-proBNP of CHD-PAH group was relatively lower （F=5.960, P=0.003）.IPAH group had higher ALT （F=5.833, P=0.007）, USA （F=6.659, P=0.001） and NT-proBNP （F=4.199, P=0.027）. Moreover,there was a high percentage of cardiac functional class III/IV （44.4%）in IPAH group （χ^2=23.099, P=0.001）. Conclution Patients with pulmonary arterial hypertension in central China are younger and frequently in female than in previous descriptions. CHD-PAH is the most common type. NT-proBNP is not parallel to mPAP. The liver function and kidney function in IPAH ptients are damaged and they are already at the severe condition when seeing doctor for the first time.
作者
杨凯越
张连生
周红梅
李珊珊
张刚成
郑璇
YANG Kai-yue ZHANG Lian-sheng ZHOU Hong-mei LI Shan-shan ZHANG Gang-cheng ZHENG Xuan(College of Medicine, Wuhan University of Science and Technology, Wuhan 430065, Chin)
出处
《中国实用内科杂志》
CAS
CSCD
北大核心
2017年第10期898-902,共5页
Chinese Journal of Practical Internal Medicine
基金
湖北省自然科学基金(2017CFB256)
武汉市卫生和计划生育委员会科研项目(WX16Z10)
关键词
肺动脉高压
临床表型
血流动力学
pulmonary arterial hypertension
clinical phenotype
hemodynamics
