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Simplified risk stratification for pulmonary arterial hypertension associated with connective tissue disease.

  • Li, Xiaodi1
  • Sun, Xiaoxuan1
  • Huang, Yingheng1
  • Wang, Yuanyuan1
  • Yang, Xiaoman1
  • Wang, Jingya1
  • Zhang, Ning1
  • Gu, Lei1
  • Zhang, Miaojia2
  • Wang, Qiang3
  • 1 Department of Rheumatology, The first affiliated hospital of Nanjing Medical University, Nanjing, China. , (China)
  • 2 Department of Rheumatology, The first affiliated hospital of Nanjing Medical University, Nanjing, China. [email protected] , (China)
  • 3 Department of Rheumatology, The first affiliated hospital of Nanjing Medical University, Nanjing, China. [email protected] , (China)
Published Article
Clinical Rheumatology
Publication Date
Dec 01, 2019
DOI: 10.1007/s10067-019-04690-3
PMID: 31385084


To explore the long-term prognostic value of a simplified risk assessment strategy based on the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines in Chinese patients with connective tissue disease (CTD) associated with pulmonary arterial hypertension (PAH). We identified 50 CTD-PAH patients diagnosed by right heart catheterization. A retrospective chart review was completed to assess their clinical presentation and laboratory test results. A simplified version of the risk stratification model proposed by the 2015 ESC/ERS PH guidelines was applied, which included the WHO functional class, the 6-minute walking distance test, N-terminal pro-B-type natriuretic peptide plasma levels, pericardial effusion, right atrial pressure (RAP), cardiac index (CI), and mixed venous oxygen saturation (SvO2). The risk grades were defined as follows: low risk = at least 3 low-risk variables and no high-risk variables; high risk = at least 2 high-risk variables, including SvO2 or CI; and intermediate risk = when the above definitions of low or high risk were not fulfilled. The study endpoint was 3-year all-cause mortality. Twenty patients were defined as a low-risk group, while 30 were classified into a combined intermediate-high-risk group at the baseline assessment. All 20 patients in the low-risk group remained in the low-risk group at follow-up, 20 patients in the intermediate-high-risk group were downgraded to the low-risk group, and eight patients remained in the intermediate-high-risk group at the follow-up assessment. Patients in the intermediate-high-risk group exhibited higher 3-year mortality than the low-risk group at baseline (26% vs 14%, P = 0.0384). Compared with patients who remained in the intermediate-high-risk group, patients who were downgraded to the low-risk group showed lower 3-year mortality (P = 0.0281). A simplified risk stratification model based on the 2015 ESC/ERS PH guidelines helped to identify CTD-PAH patients with poor long-term prognosis , which was useful in evaluating the severity and treatment response of patients with CTD-PAH.Key Point•This study showed that the simplified version of the 2015 ESC/ERS risk stratification model could help identify Chinese CTD-PAH patients with poor prognosis at diagnosis and after treatment initiation.

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