不同治疗时代特发性和家族性肺动脉高压临床特征和生存率的比较  

Comparison of clinical characteristics and survival on patients with idiopathic pulmonary arterial hypertension and familial pulmonary arterial hypertension during conventional therapy era and targeted therapy era

作　　者：徐希奇[1] 孙明利[2] 蒋鑫[1] 张锐[2] 赵勤华[2] 王勇[3] 孙凯[1] 王晓建[1] 彭富华[1] 郑璐[2] 文莉[1] 荆志成[1] 

机构地区：[1]中国医学科学院北京协和医学院阜外心血管病医院心血管疾病国家重点实验室血栓性疾病诊治中心,100037 [2] 同济大学附属上海市肺科医院心肺循环中心 [3] 首都医科大学附属北京世纪坛医院肺循环科

出　　处：《中华心血管病杂志》2014年第6期465-468,共4页Chinese Journal of Cardiology

基　　金：基金项目：国家自然科学基金国际（地区）合作与交流项目（81320108005）

摘　　要：目的 比较传统治疗时代与靶向治疗时代特发性肺动脉高压（IPAH）和家族性肺动脉高压（FPAH）的临床特征和生存率.方法 入选1999年1月至2004年10月阜外心血管病医院所有住院的IPAH及FPAH患者为传统治疗时代研究对象,收集基线资料,并于2005年6月前完成随访.入选2006年9月至2011年8月参加研究医院住院的患者为靶向治疗时代研究对象,于2013年12月前完成随访.研究观察终点均为患者生存状态及治疗药物使用情况.结果 传统治疗时代共入选IPAH和FPAH患者72例,靶向治疗时代共入选375例.两组患者平均年龄分别为（35.9 ±12.2）岁和（34.5 ± 17.4）岁（P=0.67）,均为女性多发,两组间WHO肺动脉高压功能分级和血液动力学参数比较差异均无统计学意义.传统治疗时代90.3％ （65/72）的患者接受钙通道阻滞剂治疗,靶向药物治疗时代绝大多数患者接受一种或一种以上靶向药物治疗,仅3.2％（12/375）的急性肺血管扩张试验阳性患者接受钙通道阻滞剂治疗.传统治疗时代生存时间中位数为30.4个月,靶向治疗时代生存时间中位数为66.2个月.传统治疗时代IPAH及FPAH患者1年、2年、3年和5年生存率分别为68.0％、56.9％、38.9％和20.8％,靶向药物治疗时代均有所改善,分别为89.3％、78.1％、68.2％和53.7％（P均＜0.000 1）.结论 靶向治疗时代IPAH和FPAH患者的生存率较传统治疗时代有明显改善.Objective To compare the clinical characteristics and survival on Chinese patients with idiopathic pulmonary arterial hypertension （IPAH） and familiar pulmonary arterial hypertension （FPAH） during conventional therapy era and targeted therapy era.Methods IPAH and FPAH patients who were referred between Jan 1999 and Oct 2004 in Fuwai Hospital were defined as conventional therapy era group （before 2005 no PAH-specific drug was available in China）.All patients in this group were followed up till Jun 2005.IPAH and FPAH patients who were referred between Sep 2006 and Aug 2011 were defined as targeted therapy era group （new PAH-specific drugs were available in China since 2006） were analyzed.All patients in this group were followed up till Dec 2013.The primary endpoints were death and therapy medicine.Results Seventy-two patients were enrolled in conventional therapy era group,375 were enrolled in targeted therapy era group.The mean age was （35.9 ± 12.2） years and （34.5 ± 17.4） years respectively （P =0.67）,and women was predominant in both groups.There was no difference in WHO functional class and hemodynamic data between the two groups.About 90.3% patients were treated by calcium-channel blockers （CCB） in conventional therapy era group.In targeted therapy era group,almost all patients were treated by at least one PAH-specific drug,only 3.2% patients who had a positive response to acute pulmonary vasodilator testing were treated by CCB.The median survival time was 30.4 months in conventional therapy era group and 66.2 months in targeted therapy era group.The 1-,2-,3-and 5-year survival rates of IPAH and FPAH patients were 68.0%,56.9%,38.9% and 20.8% in conventional therapy era group,and 89.3%,78.1%,68.2% and 53.7% in targeted therapy era group respectively （P 〈 0.000 1）.Conclusion Compared with conventional therapy era,the survival rate of Chinese IPAH and FPAH patients is significantly improved in targeted therapy era.

关 键 词：高血压 肺性 疾病特征 存活率 靶向治疗 

分 类 号：R544.1[医药卫生—心血管疾病]