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Ten-year survival, trends and risk stratification of newly diagnosed idiopathic pulmonary arterial hypertension in China: insights from a national multicenter prospective registry

Year: 2025
Type: article
Study design: Observational Study
Abstract: BACKGROUND: Data on the 10-year survival of incident IPAH in China is lacking. The changing trend of characteristics and survival of IPAH over the past decade has yet been demonstrated. The 4-stratum risk model needs further validation in different cohorts. METHODS: Incident cases of IPAH identified in the national multicenter prospective registry of PAH from 2009 to 2019 were analyzed. Patients with at least two of the three acquired variables (WHO-FC, 6MWD, NT-pro BNP/BNP) were included for risk stratification. RESULTS: 605 incident patients with IPAH were included (age: 35 ± 12 years; 75.2% females). The 1-, 3-, 5-, and 10-year survival of the whole cohort was 95.4%, 84.3%, 72.3%, and 53.4%, respectively. Aside from traditional prognostic factors of PAH, TG/HDL-c ratio > 1.4 was independently associated with 10-year mortality (HR 1.458). Compared with 2009–2014, patients enrolled in 2015–2019 showed shorter diagnostic delays and less severe diseases at baseline. The 1-year and 3-year survival improved over time, with increased prescription of targeted therapy and initial combination therapy. With the 4-stratum risk model, patients classified into different risk groups showed significantly different survival at both baseline (C-statistic 0.66) and follow-up (C-statistic 0.83) when three variables were available. CONCLUSIONS: The 10-year survival of IPAH patients in China has shown improvement, though further gains are needed. TG/HDL-c > 1.4 was associated with increased10-year mortality. Over the past decade, advances in the management of IPAH have contributed to better survival. The 4-stratum model demonstrated a good performance in Chinese patients with IPAH when three variables were available. TRIAL REGISTRATION: No. NCT01417338 (Registration Date 200908).
DOI: 10.1186/s12890-025-04025-w
Authors: Yifan Wu, Yuling Qian, Ruilin Quan, Yuanhua Yang, Gangcheng Zhang, Caojin Zhang, Zaixin Yu, Weifeng Wu, Shengqing Li, Guangyi Wang, Guangliang Shan, Huijun Han, Changming Xiong, Wei Zhang, Jianguo He
Institutions: Nanchang University, Sir Run Run Shaw Hospital, Zhejiang University, Chinese Academy of Medical Sciences & Peking Union Medical College, Fu Wai Hospital, Capital Medical University, Beijing Chao-Yang Hospital, Capital Medical University, Wuhan Asia Heart Hospital, Guangdong Academy of Medical Sciences, Guangdong Provincial People's Hospital, Central South University, Xiangya Hospital Central South University, Guangxi Medical University, First Affiliated Hospital of GuangXi Medical University, Xijing Hospital, Air Force Medical University, Chinese PLA General Hospital, Institute of Basic Medical Sciences of the Chinese Academy of Medical Sciences
Language: English
Field: Medicine
Open Access status: gold
Estimated APC paid: $3,090
OpenAlex ID: W4416701463
FWCI:
Cites: 38
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