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Risk prediction in medically treated chronic thromboembolic pulmonary hypertension

Authors
  • Quan, Ruilin1
  • Yang, Yuanhua2
  • Yang, Zhenwen3
  • Tian, Hongyan4
  • Li, Shengqing5
  • Shen, Jieyan6
  • Ji, Yingqun7
  • Zhang, Gangcheng8
  • Zhang, Caojin9
  • Wang, Guangyi10
  • Liu, Yuhao11
  • Cheng, Zhaozhong12
  • Yu, Zaixin13
  • Song, Zhiyuan14
  • Zheng, Zeqi15
  • Cui, Wei16
  • Chen, Yucheng17
  • Liu, Shuang18
  • Chen, Xiaoxi1
  • Qian, Yuling1
  • And 3 more
  • 1 Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Xicheng District, Beijing, 100037, China , Beijing (China)
  • 2 Capital Medical University, Beijing, 100043, China , Beijing (China)
  • 3 Cardiovascular, Tianjin Medical University General Hospital, Tianjin, 300052, China , Tianjin (China)
  • 4 Medical College of Xi’an Jiaotong University, Xi’an, 710061, China , Xi’an (China)
  • 5 Fourth Military Medical University, Xi’an, 710032, China , Xi’an (China)
  • 6 Shanghai Jiaotong University School of Medicine, Shanghai, 200240, China , Shanghai (China)
  • 7 First Affiliated Hospital of Dalian Medical University, Dalian, 116011, China , Dalian (China)
  • 8 Wuhan Asia Heart Hospital, Wuhan, 430022, China , Wuhan (China)
  • 9 Guangdong Academy of Medical Sciences, Guangzhou, 510080, China , Guangzhou (China)
  • 10 Medical School of Chinese PLA, Beijing, 100039, China , Beijing (China)
  • 11 Central China Fuwai Hospital of Zhengzhou University, Zhengzhou, 450003, China , Zhengzhou (China)
  • 12 The Affiliated Hospital of Qingdao University, Qingdao, 266000, China , Qingdao (China)
  • 13 Central South University, Changsha, 410008, China , Changsha (China)
  • 14 Third Military Medical University (Army Medical University), Chongqing, 400038, China , Chongqing (China)
  • 15 The First Affiliated Hospital of Nanchang University, Nanchang, 330006, China , Nanchang (China)
  • 16 Second Hospital of Hebei Medical University, Shijiazhuang, 050000, China , Shijiazhuang (China)
  • 17 Sichuan University, Chengdu, 610041, China , Chengdu (China)
  • 18 Capital Medical University, Beijing, 100029, China , Beijing (China)
  • 19 Chinese Academy of Medical Sciences, Beijing, 100037, China , Beijing (China)
Type
Published Article
Journal
BMC pulmonary medicine
Publication Date
Apr 20, 2021
Volume
21
Issue
1
Identifiers
DOI: 10.1186/s12890-021-01495-6
Source
Springer Nature
Keywords
License
Green

Abstract

BackgroundAt present, there is no generally accepted comprehensive prognostic risk prediction model for medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients.MethodsConsecutive medically treated CTEPH patients were enrolled in a national multicenter prospective registry study from August 2009 to July 2018. A multivariable Cox proportional hazards model was utilized to derive the prognostic model, and a simplified risk score was created thereafter. Model performance was evaluated in terms of discrimination and calibration, and compared to the Swedish/COMPERA risk stratification method. Internal and external validation were conducted to validate the model performance.ResultsA total of 432 patients were enrolled. During a median follow-up time of 38.73 months (IQR: 20.79, 66.10), 94 patients (21.8%) died. The 1-, 3-, and 5-year survival estimates were 95.5%, 83.7%, and 70.9%, respectively. The final model included the following variables: the Swedish/COMPERA risk stratum (low-, intermediate- or high-risk stratum), pulmonary vascular resistance (PVR, ≤ or > 1600 dyn·s/cm5), total bilirubin (TBIL, ≤ or > 38 µmol/L) and chronic kidney disease (CKD, no or yes). Compared with the Swedish/COMPERA risk stratification method alone, both the derived model [C-index: 0.715; net reclassification improvement (NRI): 0.300; integrated discriminatory index (IDI): 0.095] and the risk score (C-index: 0.713; NRI: 0.300; IDI: 0.093) showed improved discriminatory power. The performance was validated in a validation cohort of 84 patients (C-index = 0.707 for the model and 0.721 for the risk score).ConclusionsA novel risk stratification strategy can serve as a useful tool for determining prognosis and guide management for medically treated CTEPH patients.Trial registration: ClinicalTrials.gov (Identifier: NCT01417338).

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