{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T08:58:05Z","timestamp":1792400285414,"version":"4.4.0"},"reference-count":24,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2022,3,15]],"date-time":"2022-03-15T00:00:00Z","timestamp":1647302400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and objective<\/jats:title>\n                    <jats:p>Nationally representative reports on the characteristics and long\u2010term survival of pulmonary arterial hypertension (PAH) from developing countries are scarce. The applicability of the current main risk stratifications and the longitudinal changes in goal\u2010oriented treatments have yet to be elucidated in real\u2010world settings. Therefore, we aimed to provide insights into the characteristics, goal\u2010oriented treatments and survival of PAH in China and to explore the applicability of the main risk stratifications in our independent cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>PAH patients were consecutively enrolled from a national prospective multicentre registry. Data on baseline, follow\u2010up re\u2010evaluation and therapeutic changes were collected.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 2031 patients were enrolled, with congenital heart disease (CHD)\u2010PAH (45.2%) being the most common aetiology. The mean age was 35\u2009\u00b1\u200912\u2009years, and 76.2% were females. At baseline, approximately 20% of the patients with intermediate or high risk received combination treatment. At follow\u2010up, approximately half of the re\u2010evaluated patients did not achieve low\u2010risk profiles, and even among patients who received combination therapy at baseline, 4% of them still worsened. The rate of combination therapy increased significantly from 6.7% before 2015 to 35.5% thereafter. The main risk assessment tools demonstrated good performance for predicting survival both at baseline and at follow\u2010up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Chinese PAH patients show both similar and distinct features compared to other countries. Current main risk stratifications can significantly discriminate patients at different risk levels. There were still many patients not achieving low\u2010risk profiles at follow\u2010up, indicating more aggressive treatment should be implemented to optimize the goal\u2010oriented treatment strategy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/resp.14247","type":"journal-article","created":{"date-parts":[[2022,3,15]],"date-time":"2022-03-15T23:42:45Z","timestamp":1647387765000},"page":"517-528","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":49,"title":["Characteristics, goal\u2010oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5194-0420","authenticated-orcid":false,"given":"Ruilin","family":"Quan","sequence":"first","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gangcheng","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Cardiology Wuhan Asia Heart Hospital  Wuhan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zaixin","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Xiangya Hospital Central South University  Changsha China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caojin","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Guangdong Provincial People's Hospital Guangdong Academy of Medical Sciences  Guangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhenwen","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Cardiology Tianjin Medical University General Hospital  Tianjin China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongyan","family":"Tian","sequence":"additional","affiliation":[{"name":"Department of Peripheral Vascular Diseases The First Affiliated Hospital of Xi'an Jiaotong University  Xi'an China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuanhua","family":"Yang","sequence":"additional","affiliation":[{"name":"Respiratory and Critical Care Medicine, Beijing Institute of Respiratory Medicine, Beijing Chao\u2010Yang Hospital Capital Medical University  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weifeng","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Cardiology The First Affiliated Hospital of Guangxi Medical University  Nanning China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yucheng","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, West China Hospital Sichuan University  Chengdu China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuhao","family":"Liu","sequence":"additional","affiliation":[{"name":"Heart Centre of Henan Provincial People's Hospital, Central China Fuwai Hospital Central China Fuwai Hospital of Zhengzhou University  Zhengzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xianyang","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease General Hospital of Northern Theater Command  Shenyang China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shengqing","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Xijing Hospital Fourth Military Medical University  Xi'an China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jieyan","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Renji Hospital Shanghai Jiaotong University School of Medicine  Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zeqi","family":"Zheng","sequence":"additional","affiliation":[{"name":"Department of Cardiology The First Affiliated Hospital of Nanchang University  Nanchang China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiulong","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine The People's Hospital of Gaozhou  Maoming China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guangyi","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Cardiology Chinese PLA General Hospital  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qian","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH) Chinese Academy of Medical Sciences and Peking Union Medical College  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daxin","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Zhongshan Hospital, Fudan University Shanghai Institute of Cardiovascular Disease  Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yingqun","family":"Ji","sequence":"additional","affiliation":[{"name":"Department of Respiratory First Affiliated Hospital of Dalian Medical University  Dalian China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5627-2457","authenticated-orcid":false,"given":"Tao","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoxi","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuling","family":"Qian","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yangyi","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qing","family":"Gu","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Changming","family":"Xiong","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guangliang","family":"Shan","sequence":"additional","affiliation":[{"name":"Institute of Basic Medical Sciences Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0521-895X","authenticated-orcid":false,"given":"Jianguo","family":"He","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College State 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