The management pattern and outcomes of chronic thromboembolic pulmonary hypertension: rationale and design for a Chinese real-world study

作者全名：Zhang, Shuai; Li, Yishan; Yang, Yuanhua; Gong, Sugang; Yang, Zhenwen; Hong, Cheng; Cui, Xiaopei; Wan, Jun; Ji, Yingqun; Chen, Hong; Zhu, Ling; Li, Chenghong; Cheng, Zhaozhong; Zhang, Yunhui; Wang, Qiguang; Luo, Qin; Guo, Lu; Ma, Guofeng; Han, Baoshi; Liu, Zhihong; Xiong, Changming; Wang, Lan; Ji, Qiushang; Li, Fajiu; Wang, Shengfeng; Zhang, Zhu; Zhang, Yunxia; Wang, Dingyi; Gao, Qian; Huang, Qiang; Xie, Wanmu; Zhai, Zhenguo; Wang, Chen

作者地址：[Zhang, Shuai; Li, Yishan; Zhang, Zhu; Zhang, Yunxia; Gao, Qian; Huang, Qiang; Xie, Wanmu; Zhai, Zhenguo; Wang, Chen] Chinese Acad Med Sci, China Japan Friendship Hosp, Natl Ctr Resp Med, Dept Pulm & Crit Care Med,Ctr Resp Med,State Key L, Beijing, Peoples R China; [Yang, Yuanhua] Capital Med Univ, Beijing Chaoyang Hosp, Dept Pulm & Crit Care Med, Beijing, Peoples R China; [Gong, Sugang] Tongji Univ, Shanghai Pulm Hosp, Dpat Pulm Circulat, Sch Med, Shanghai, Peoples R China; [Yang, Zhenwen] Tianjin Med Univ, Gen Hosp, Dept Cardiol, Tianjin, Peoples R China; [Hong, Cheng] Guangzhou Med Univ, Guangzhou Inst Resp Hlth, State Key Lab Resp Dis, Guangdong Key Lab Vasc Dis,Affiliated Hosp 1, Guangzhou, Peoples R China; [Cui, Xiaopei] Shandong Univ, Dept Geriatr Med, Qilu Hosp, Jinan, Peoples R China; [Wan, Jun] Capital Med Univ, Beijing Anzhen Hosp, Dept Pulm & Crit Care Med, Beijing, Peoples R China; [Ji, Yingqun] Tongji Univ, East Hosp, Dept Pulm & Crit Care Med, Sch Med, Shanghai, Peoples R China; [Chen, Hong] Chongqing Med Univ, Dept Pulm & Crit Care Med, Affiliated Hosp 1, Chongqing, Peoples R China; [Zhu, Ling] Shandong Univ, Dept Resp Med, Shandong Prov Hosp, Jinan, Peoples R China; [Li, Chenghong; Li, Fajiu] Jianghan Univ, Affiliated Hosp, Dept Pulm & Crit Care Med, Wuhan, Peoples R China; [Cheng, Zhaozhong] Qingdao Univ, Dept Pulm & Crit Care Med, Affiliated Hosp, Qingdao, Shandong Provin, Peoples R China; [Zhang, Yunhui] First Peoples Hosp Yunnan Prov, Dept Resp Med, Kunming, Peoples R China; [Wang, Qiguang] Gen Hosp Northern Theater Command, Div Pulm Vasc Dis, Shenyang, Peoples R China; [Luo, Qin; Liu, Zhihong; Xiong, Changming] Chinese Acad Med Sci & Peking Union Med Coll, Ctr Pulm Vasc Dis, State Key Lab Cardiovasc Dis, Fuwai Hosp,Natl Ctr Cardiovasc Dis, Beijing, Peoples R China; [Guo, Lu] Univ Elect Sci & Technol China, Sichuan Prov Peoples Hosp, Sch Med, Dept Pulm & Crit Care Med, Chengdu, Peoples R China; [Ma, Guofeng] Zhejiang Univ, Dept Resp Dis, Sch Med, Sir Run Run Shaw Hosp, Hangzhou, Peoples R China; [Han, Baoshi] Chinese Peoples Liberat Army Gen Hosp, Dept Cardiol, Beijing, Peoples R China; [Wang, Lan] Tongji Univ, Sch Med, Shanghai Pulm Hosp, Dept Pulm Circulat, Shanghai, Peoples R China; [Ji, Qiushang] Shandong Univ, Qilu Hosp, Dept Cardiol, Jinan, Peoples R China; [Wang, Shengfeng] Peking Univ, Sch Publ Hlth, Dept Epidemiol & Biostat, Beijing, Peoples R China; [Wang, Dingyi] Chinese Acad Med Sci, China Japan Friendship Hosp, Inst Resp Med, Inst Clin Med Sci,Natl Ctr Resp Med,State Key Lab, Beijing, Peoples R China; [Wang, Chen] Chinese Acad Med Sci, Peking Union Med Coll, Beijing, Peoples R China

通信作者：Xie, WM; Zhai, ZG (通讯作者)，Chinese Acad Med Sci, China Japan Friendship Hosp, Natl Ctr Resp Med, Dept Pulm & Crit Care Med,Ctr Resp Med,State Key L, Beijing, Peoples R China.

来源：BMC PULMONARY MEDICINE

ESI学科分类：CLINICAL MEDICINE

WOS号：WOS:001237438600001

JCR分区：Q2

影响因子：2.6

年份：2024

卷号：24

期号：1

开始页：　

结束页：　

文献类型：Article

关键词：Chronic thromboembolic pulmonary hypertension; Registry; Real-world; Multimodality treatment; Prognosis

摘要：Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disorder with substantial morbidity and mortality, also a disease underdiagnosed and undertreated. It is potentially curable by pulmonary endarterectomy (PEA) in patients with surgically accessible thrombi. Balloon pulmonary angioplasty (BPA) and targeted medical therapy are options for patients with distal lesions or persistent/recurrent pulmonary hypertension after PEA. There is an urgent need to increase the awareness of CTEPH. Qualified CTEPH centers are still quite limited. Baseline characteristics, management pattern and clinical outcome of CTEPH in China needs to be reported.Methods and design The CHinese reAl-world study to iNvestigate the manaGEment pattern and outcomes of chronic thromboembolic pulmonary hypertension (CHANGE) study is designed to provide the multimodality treatment pattern and clinical outcomes of CTEPH in China. Consecutive patients who are >= 14 year-old and diagnosed with CTEPH are enrolled. The diagnosis of CTEPH is confirmed in right heart catheterization and imaging examinations. The multimodality therapeutic strategy, which consists of PEA, BPA and targeted medical therapy, is made by a multidisciplinary team. The blood sample and tissue from PEA are stored in the central biobank for further research. The patients receive regular follow-up every 3 or 6 months for at least 3 years. The primary outcomes include all-cause mortality and changes in functional and hemodynamic parameters from baseline. The secondary outcomes include the proportion of patients experiencing lung transplantation, the proportion of patients experiencing heart and lung transplantation, and changes in health-related quality of life. Up to 31 December 2023, the study has enrolled 1500 eligible patients from 18 expert centers.Conclusions As a real-world study, the CHANGE study is expected to increase our understanding of CTEPH, and to fill the gap between guidelines and the clinical practice in the diagnosis, assessment and treatment of patients with CTEPH.Registration Number in ClinicalTrials.gov NCT05311072.

基金机构：National Key Research and Development Program of China [2023YFC2507200]; CAMS Innovation Fund for Medical Sciences (CIFMS) [2021-I2M-1-049]; National High Level Hospital Clinical Research Funding [2022-NHLHCRF-LX-01-01-02]

基金资助正文：This work was supported by The National Key Research and Development Program of China (No.2023YFC2507200), CAMS Innovation Fund for Medical Sciences (CIFMS) (No.2021-I2M-1-049) and National High Level Hospital Clinical Research Funding (No.2022-NHLHCRF-LX-01-01-02).