靶向治疗时代第Ⅰ大类肺动脉高压患者临床资料及预后分析  

Analysis of clinical data and prognosis of pulmonary arterial hypertension in the era of targeted therapy

作　　者：李珊珊[1] 颜梦欢 邱丘[1] 周红梅[1] 张刚成[1] LI Shan-shan;YAN Meng-huan;QIU Qiu;ZHOU Hong-mei;ZHANG Gang-cheng(Congenital Heart Disease Ward,Wuhan Asia Heart Hospital,Wuhan 430022,China)

机构地区：[1]武汉亚洲心脏病医院先天性心脏病中心,湖北省武汉市430022

出　　处：《中国心血管病研究》2020年第7期626-631,共6页Chinese Journal of Cardiovascular Research

基　　金：湖北省卫生健康委项目(WJ2019H242);武汉市卫生局计生委项目(WX16Z10)。

摘　　要：目的分析中国肺血管扩张药物上市后第Ⅰ大类肺动脉高压(PAH)患者的临床资料及预后.方法回顾性分析2012年1月至2019年3月武汉亚洲心脏病医院收治的750例确诊的第Ⅰ大类PAH患者,收集患者基线资料、实验室检查、影像学和心导管检查等,平均随访(2.3±1.7)年,评价第Ⅰ大类PAH的治疗及预后,对比分析中国靶向肺血管扩张药上市前后患者临床资料及预后.结果第Ⅰ大类PAH患者平均确诊年龄(30.39±17.54)岁,女性占65.6%.心功能Ⅲ或Ⅳ级29.2%,6MWD(422±96)米.第Ⅰ大类PAH中IPAH 12.4%、HPAH 0.7%、CTD-PAH 4.8%和portalHT-PAH 0.5%及CHD-PAH 81.6%.不同亚类PAH性别(χ^2=24.551,P<0.05)、年龄(F=3.973,P=0.003)、BMI(F=11.005,P<0.05)、6MWD(F=2.537,P=0.041)、SPO2(F=4.129,P=0.003)、尿酸(F=7.654,P<0.05)、总胆红素(F=3.737,P=0.005)、SvO2(F=3.298,P=0.011)、PVRI(F=11.132,P<0.05)、心排指数(F=3.506,P=0.008)、右心室/左心室舒张末内径(F=14.909,P<0.05)差异有统计学意义.PAH中基础治疗仅占16.3%,心功能Ⅲ~Ⅳ级联合应用两种肺血管扩张药占51.6%.平均随访时间为(2.3±1.7)年,终点事件135例,其中死亡30例.IPAH、HPAH、CTD-PAH和CHD-PAH死亡者分别占8.6%、60.0%、11.1%、2.5%.PAH第1、3年生存率分别为98.8%和96.0%,较非靶向药物治疗时期分别提高30.8%、57.1%.结论靶向治疗时代我国第Ⅰ大类PAH患者早期生存率较前改善,但低龄患者药物选择种类少,早期诊断率有待提高,需提高标准靶向治疗率.ObjectiveTo analyze the clinical data and prognosis of pulmonary arterial hypertension(PAH)in the cra of pulmonary vasodilator released in China.Methods A retrospective and singal center(Wuhan Asia Heart Hospital)cohort study collected data of 750 patients with PAH between January 2012 to March 2019 were conducted.Baseline data,laboratory examination,imaging and cardiac cathcterization were collected and followed up for an average of(2.3±1.7)years.The treatment and prognosis of PAH were evaluated.The clinical data and prognosis of patients before and after China's targeted pulmonary vasodilators were compared.Results The average age of diagnosis of PAH was(30.39±17.54)years old and 65.6%was woman,with WHO-FCⅢ-Ⅳ29.2%and 6MWD(422±96)m.Among the first PAHs,there were IPAH 12.4%,HPAH 0.7%,CTD-PAH 4.8%,portal HT-PAH 0.5%and CHD-PAH 81.6%.There were significant differences in PAH gender(χ^2=24.551,P<0.05),age(F=3.973,P=0.003),BMI(F=11.005,P<0.05),6MWD(F=2.537,P=0.041),SpO2(F=4.129,P=0.003),uric acid(F=7.654,P<0.05),TBIL(F=3.737,P=0.005),SvO2(F=3.298,P=0.011),PVRI(F=11.132,P<0.05),cardiac index(F=3.506,P=0.008)and right/left end diastolic diameter(F=14.909,P<0.05)among the subtypesPAH.For the PAH patients,only 16.3%were treated with basic therapy and 51.6%were treated with two kinds of pulmonary vasodilators.The mean follow-up time was(2.3±1.7)years.There were 135 end-point events,of which 30 died and IPAH,HPAH,CTD-PAH and CHD-PAD accounted for 8.6%,60.0%,11.1%and 2.5%respectively.The 1-year and 3-year survival rates of PAH were 98.8%and 96.0%respectively,which were 30.8%and 57.1%higher than those of non targeted drug therapy.Conclusion In the era of targeted therapy,the early survival rate o f the PAH patients in China is better than before.The early diagnosis rate should be improved and the standard targeted treatment rate needs also to be improved.

关 键 词：肺动脉高压 靶向治疗 肺血管扩张药 

分 类 号：R544.1.6[医药卫生—心血管疾病]