. 2024 Jun 3;24(1):265.

doi: 10.1186/s12890-024-03042-5.

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

Shuai Zhang^#¹, Yishan Li^#¹, Yuanhua Yang², Sugang Gong³, Zhenwen Yang⁴, Cheng Hong⁵, Xiaopei Cui⁶, Jun Wan⁷, Yingqun Ji⁸, Hong Chen⁹, Ling Zhu¹⁰, Chenghong Li¹¹, Zhaozhong Cheng¹², Yunhui Zhang¹³, Qiguang Wang¹⁴, Qin Luo¹⁵, Lu Guo¹⁶, Guofeng Ma¹⁷, Baoshi Han¹⁸, Zhihong Liu¹⁵, Changming Xiong¹⁵, Lan Wang¹⁹, Qiushang Ji²⁰, Fajiu Li¹¹, Shengfeng Wang²¹, Zhu Zhang¹, Yunxia Zhang¹, Dingyi Wang²², Qian Gao¹, Qiang Huang¹, Wanmu Xie²³, Zhenguo Zhai²⁴, Chen Wang^{1

25}

Affiliations

¹ National Center for Respiratory Medicine, State Key Laboratory of Respiratory Health and Multimorbidity, National Clinical Research Center for Respiratory Diseases, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, P.R. China.
² Department of Pulmonary and Critical Care Medicine, Beijing Chaoyang Hospital, Capital Medical University, Beijing, P.R. China.
³ Dpartments of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, P.R. China.
⁴ Department of Cardiology, Tianjin Medical University General Hospital, Tianjin, P.R. China.
⁵ State Key Laboratory of Respiratory Diseases, Guangdong Key Laboratory of Vascular Diseases, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, P.R. China.
⁶ Department of Geriatric Medicine, Qilu Hospital of Shandong University, Jinan, P.R. China.
⁷ Department of Pulmonary and Critical Care Medicine, Beijing Anzhen Hospital, Capital Medical University, Beijing, P.R. China.
⁸ Department of Pulmonary and Critical Care Medicine, East Hospital, Tongji University School of Medicine, Shanghai, China.
⁹ Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Chongqing Medical University, Chongqing, P.R. China.
¹⁰ Department of Respiratory Medicine, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, P.R. China.
¹¹ Department of Pulmonary and Critical Care Medicine, Affiliated Hospital of Jianghan University, Wuhan, P.R. China.
¹² Department of Pulmonary and Critical Care Medicine, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, P.R. China.
¹³ Department of Respiratory Medicine, The First People's Hospital of Yunnan Province, Kunming, P.R. China.
¹⁴ Division of Pulmonary Vascular Disease, General Hospital of Northern Theater of Command, Shenyang, P.R. China.
¹⁵ Center for Pulmonary Vascular Diseases, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, P.R. China.
¹⁶ Department of Pulmonary and Critical Care Medicine, School of Medicine, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, P.R. China.
¹⁷ Department of Respiratory Diseases, Sir Run Run ShawHospital, Zhejiang University School of Medicine, Hangzhou, P.R. China.
¹⁸ Department of Cardiology, Chinese People's Liberation Army General Hospital, Beijing, P.R. China.
¹⁹ Departments of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, P.R. China.
²⁰ Department of Cardiology, Qilu Hospital of Shandong University, Jinan, P.R. China.
²¹ Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing, P.R. China.
²² National Center for Respiratory Medicine, State Key Laboratory of Respiratory Health and Multimorbidity, National Clinical Research Center for Respiratory Diseases, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Data and Project Management Unit, Institute of Clinical Medical Sciences, China-Japan Friendship Hospital, Beijing, P.R. China.
²³ National Center for Respiratory Medicine, State Key Laboratory of Respiratory Health and Multimorbidity, National Clinical Research Center for Respiratory Diseases, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, P.R. China. xiewanmu@126.com.
²⁴ National Center for Respiratory Medicine, State Key Laboratory of Respiratory Health and Multimorbidity, National Clinical Research Center for Respiratory Diseases, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, P.R. China. zhaizhenguo2011@126.com.
²⁵ Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, P.R. China.

^# Contributed equally.

PMID: 38825688
PMCID: PMC11145764
DOI: 10.1186/s12890-024-03042-5

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

Shuai Zhang et al. BMC Pulm Med. 2024.

. 2024 Jun 3;24(1):265.

doi: 10.1186/s12890-024-03042-5.

Authors

Affiliations

¹ National Center for Respiratory Medicine, State Key Laboratory of Respiratory Health and Multimorbidity, National Clinical Research Center for Respiratory Diseases, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, P.R. China.
² Department of Pulmonary and Critical Care Medicine, Beijing Chaoyang Hospital, Capital Medical University, Beijing, P.R. China.
³ Dpartments of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, P.R. China.
⁴ Department of Cardiology, Tianjin Medical University General Hospital, Tianjin, P.R. China.
⁵ State Key Laboratory of Respiratory Diseases, Guangdong Key Laboratory of Vascular Diseases, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, P.R. China.
⁶ Department of Geriatric Medicine, Qilu Hospital of Shandong University, Jinan, P.R. China.
⁷ Department of Pulmonary and Critical Care Medicine, Beijing Anzhen Hospital, Capital Medical University, Beijing, P.R. China.
⁸ Department of Pulmonary and Critical Care Medicine, East Hospital, Tongji University School of Medicine, Shanghai, China.
⁹ Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Chongqing Medical University, Chongqing, P.R. China.
¹⁰ Department of Respiratory Medicine, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, P.R. China.
¹¹ Department of Pulmonary and Critical Care Medicine, Affiliated Hospital of Jianghan University, Wuhan, P.R. China.
¹² Department of Pulmonary and Critical Care Medicine, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, P.R. China.
¹³ Department of Respiratory Medicine, The First People's Hospital of Yunnan Province, Kunming, P.R. China.
¹⁴ Division of Pulmonary Vascular Disease, General Hospital of Northern Theater of Command, Shenyang, P.R. China.
¹⁵ Center for Pulmonary Vascular Diseases, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, P.R. China.
¹⁶ Department of Pulmonary and Critical Care Medicine, School of Medicine, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, P.R. China.
¹⁷ Department of Respiratory Diseases, Sir Run Run ShawHospital, Zhejiang University School of Medicine, Hangzhou, P.R. China.
¹⁸ Department of Cardiology, Chinese People's Liberation Army General Hospital, Beijing, P.R. China.
¹⁹ Departments of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, P.R. China.
²⁰ Department of Cardiology, Qilu Hospital of Shandong University, Jinan, P.R. China.
²¹ Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing, P.R. China.
²² National Center for Respiratory Medicine, State Key Laboratory of Respiratory Health and Multimorbidity, National Clinical Research Center for Respiratory Diseases, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Data and Project Management Unit, Institute of Clinical Medical Sciences, China-Japan Friendship Hospital, Beijing, P.R. China.
²³ National Center for Respiratory Medicine, State Key Laboratory of Respiratory Health and Multimorbidity, National Clinical Research Center for Respiratory Diseases, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, P.R. China. xiewanmu@126.com.
²⁴ National Center for Respiratory Medicine, State Key Laboratory of Respiratory Health and Multimorbidity, National Clinical Research Center for Respiratory Diseases, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, P.R. China. zhaizhenguo2011@126.com.
²⁵ Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, P.R. China.

^# Contributed equally.

PMID: 38825688
PMCID: PMC11145764
DOI: 10.1186/s12890-024-03042-5

Abstract

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.

Keywords: Chronic thromboembolic pulmonary hypertension; Multimodality treatment; Prognosis; Real-world; Registry.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

**Fig. 1**
Design and flowchart of the study CTEPH: chronic thromboembolic pulmonary hypertension; VTE: venous thromboembolism; CTEPD: chronic thromboembolic pulmonary disease; VQ: ventilation/perfusion; CTPA: computer tomography pulmonary angiography; MRPA: magnetic resonance pulmonary angiography; PA: pulmonary angiograph; RHC, right heart catheterization; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; WHO-FC: WHO functional class; 6MWD: 6-minute walking distance; CMR: cardiac magnetic resonance; CPET: cardiopulmonary exercise testing; MDT: multidisciplinary team; PEA: pulmonary endarterectomy; BPA: balloon pulmonary angioplasty; PAH: pulmonary arterial hypertension

See this image and copyright information in PMC

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

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

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