Abstract

Background: The 2015 European pulmonary hypertension (PH) guidelines recommend a risk stratification strategy for pulmonary arterial hypertension (PAH). We aimed to investigate the validation and potential prognostic information in Chinese patients.Methods: The risk assessment variables proposed by the PH guidelines were performed by using the WHO function class, 6-min walking distance, brain natriuretic peptide or its N-terminal fragment, right arterial pressure, cardiac index, mixed venous saturation, right atrium area, pericardial effusion, peak oxygen consumption, and ventilatory equivalents for carbon dioxide. An abbreviated version also was applied.Results: A total of 392 patients with idiopathic PAH (IPAH) were enrolled between 2009 and 2018. After a median interval of 13 months, re-evaluation assessments were available for 386 subjects. The PAH guidelines risk tool may effectively discriminate three risk groups and mortality (p < 0.001) both at the baseline and re-evaluation. Meanwhile, its simplified risk version was valid for baseline and accurately predicted the risk of death in all the risk groups (p < 0.001). At the time of re-evaluation, the percentage of low-risk group has an increase, but a greater proportion achieved the high-risk group and a lesser proportion maintained in the intermediate-risk group.Conclusion: The 2015 European PH guidelines and its simplified version risk stratification assessment present an effective discrimination of different risk groups and accurate mortality estimates in Chinese patients with IPAH. Changes of risk proportion at re-evaluation implicated that natural treatment decisions may not be consistently with goal-oriented treatment strategy.

Highlights

  • The assessment of the prognosis of patients has been considered as an important section in patients with pulmonary arterial hypertension (PAH); different baseline and follow-up variables have been utilized individually or combined to predict outcome

  • A total of 260 (67%) patients were in the WHO function class (FC) III or IV, whereas 34% patients were in the WHO FC I-II

  • The main findings of this study can be demonstrated as follows: [1] the 2015 European pulmonary hypertension (PH) guidelines risk stratification effectively discriminated a low, intermediate, and high risk at baseline and re-evaluation assessments; [2] accurately predicted the risk of death in patients with idiopathic PAH (IPAH); [3] its simplified version risk strategy was valid for baseline; and [4] the percentage of the low-risk group has an increase at re-evaluation, but a greater proportion of patients achieved the high-risk group and a lesser proportion maintained in the intermediate-risk group

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Summary

Introduction

The assessment of the prognosis of patients has been considered as an important section in patients with pulmonary arterial hypertension (PAH); different baseline and follow-up variables have been utilized individually or combined to predict outcome. The COMPERA study used an abbreviated version risk analysis including six variables such as the WHO function class (FC), 6-min walk distance (6MWD), brain natriuretic peptide (BNP) or N-terminal proBNP (NTproBNP), right arterial pressure (RAP), cardiac index (CI), and mixed venous oxygen saturation (SVO2), not capturing disease progression, syncope, echocardiography, and cardiopulmonary exercise testing (CPET) data. These findings confirm and extend previous study by Kylhammar et al [4], who used the same subset of parameters [plus right atrial area and the presence/absence of pericardial effusion (PE)]. We aimed to investigate the validation and potential prognostic information in Chinese patients

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