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Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

Open access
Boucly, Athénaïs, Weatherald, Jason, Savale, Laurent, Jaïs, Xavier, Cottin, Vincent, Prevot, Grégoire, Picard, François, de Groote, Pascal, Jevnikar, Mitja, Bergot, Emmanuel, Chaouat, Ari, Chabanne, Céline, Bourdin, Arnaud, Parent, Florence, Montani, David, Simonneau, Gérald, Humbert, Marc, Sitbon, Olivier·2017·European Respiratory Journal

Abstract

Current European guidelines recommend periodic risk assessment for patients with pulmonary arterial hypertension (PAH). The aim of our study was to determine the association between the number of low-risk criteria achieved within 1 year of diagnosis and long-term prognosis. Incident patients with idiopathic, heritable and drug-induced PAH between 2006 and 2016 were analysed. The number of low-risk criteria present at diagnosis and at first re-evaluation were assessed: World Health Organization (WHO)/New York Heart Association (NYHA) functional class I or II, 6-min walking distance (6MWD) >440 m, right atrial pressure <8 mmHg and cardiac index ≥2.5 L·min−1·m−2. 1017 patients were included (mean age 57 years, 59% female, 75% idiopathic PAH). After a median follow-up of 34 months, 238 (23%) patients had died. Each of the four low-risk criteria independently predicted transplant-free survival at first re-evaluation. The number of low-risk criteria present at diagnosis (p<0.001) and at first re-evaluation (p<0.001) discriminated the risk of death or lung transplantation. In addition, in a subgroup of 603 patients with brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP) measurements, the number of three noninvasive criteria (WHO/NYHA functional class, 6MWD and BNP/NT-proBNP) present at first re-evaluation discriminated prognostic groups (p<0.001). A simplified risk assessment tool that quantifies the number of low-risk criteria present accurately predicted transplant-free survival in PAH.

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Metadata

Authors
Boucly, A., Weatherald, J., Savale, L., Jaïs, X., Cottin, V., Prevot, G., Picard, F., de Groote, P., Jevnikar, M., Bergot, E., Chaouat, A., Chabanne, C., Bourdin, A., Parent, F., Montani, D., Simonneau, G., Humbert, M., & Sitbon, O.
Venue
European Respiratory Journal
Published
2017-08
Volume
50
Issue
2
Pages
1700889
ISSN
  • 0903-1936 (ISSN-L)
  • 1399-3003
OpenAlex
W2744074505
Cited by
697
References
21
FWCI
48.40
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. https://app.cochat.ai/papers/10.1183/13993003.00889-2017
The paper
Boucly, A., Weatherald, J., Savale, L., Jaïs, X., Cottin, V., Prevot, G., Picard, F., de Groote, P., Jevnikar, M., Bergot, E., Chaouat, A., Chabanne, C., Bourdin, A., Parent, F., Montani, D., Simonneau, G., Humbert, M., & Sitbon, O. (2017). Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. European Respiratory Journal. https://doi.org/10.1183/13993003.00889-2017

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.