Simplified risk stratification for pulmonary arterial hypertension associated with connective tissue disease
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).
•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.
KeywordsConnective tissue disease Prognosis Pulmonary arterial hypertension Risk stratification
Pulmonary arterial hypertension
Connective tissue disease
Idiopathic pulmonary arterial hypertension
European Society of Cardiology
European Respiratory Society
6-minute walking distance
N-terminal pro-B-type natriuretic peptide
Pulmonary vascular resistance
Right heart catheterization
Mean pulmonary arterial pressure
Pulmonary arterial wedge pressure
Interstitial lung disease
High-resolution computer tomography
Systematic lupus erythematosus
American Rheumatism Association
Primary Sjogren’s syndrome
Mixed connective tissue disease
Undifferentiated connective tissue disease
Mixed venous oxygen saturation
Right atrial pressure
Thanks to all patients involved in this retrospective study.
This study was supported by the National Natural Science Foundation of China (NSFC) (81671615, 81701610, and 81302575), a project funded by the Priority Academic Program Development of Jiangsu Higher Education Institutions (PAPD), Young Medical People Project in Jiangsu Province, and Medical Talent of Empowering Medicine through Science and Education Program.
Compliance with ethical standards
This study was approved by the Medical Ethics Committee of the First Affiliated Hospital of Nanjing Medical University and it has been performed in accordance with the ethical standard laid down in the 1964 Declaration of Helsinki.
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