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Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

Athénaïs Boucly, Jason Weatherald, Laurent Savale, Xavier Jaïs, Vincent Cottin, Grégoire Prevot, François Picard, Pascal de Groote, Mitja Jevnikar, Emmanuel Bergot, Ari Chaouat, Céline Chabanne, Arnaud Bourdin, Florence Parent, David Montani, Gérald Simonneau, Marc Humbert, Olivier Sitbon
European Respiratory Journal 2017 50: 1700889; DOI: 10.1183/13993003.00889-2017
Athénaïs Boucly
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Jason Weatherald
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
4Department of Medicine, Division of Respirology, University of Calgary, Calgary, AB, Canada
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  • ORCID record for Jason Weatherald
Laurent Savale
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Xavier Jaïs
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Vincent Cottin
5Université Lyon-1, Hospices Civils de Lyon, Centre de Référence des Maladies Pulmonaires Rares, Centre de Compétences de l'Hypertension Pulmonaire, Hôpital Louis Pradel, Lyon, France
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Grégoire Prevot
6Service de Pneumologie, Hôpital Larrey, Toulouse, France
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François Picard
7Service de Cardiologie, Hôpital Haut-Lévêque, Pessac, France
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Pascal de Groote
8Hôpital Cardiologique de Lille, Centre de Compétences de l'Hypertension Pulmonaire, Lille, France
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Mitja Jevnikar
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Emmanuel Bergot
9Hôpital Côte de Nacre, Centre de Compétences Basse Normandie de l'Hypertension Pulmonaire, Université de Caen-Basse Normandie, Caen, France
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Ari Chaouat
10CHU Nancy, Pôle des Spécialités Médicales, Département de Pneumologie, Vandoeuvre-lès-Nancy, France
11Université de Lorraine, INGRES, EA 7298, Vandoeuvre-lès-Nancy, France
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Céline Chabanne
12CHU de Rennes, Service de Cardiologie et Maladies Vasculaires, INSERM U1099, Rennes, France
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Arnaud Bourdin
13Service de Pneumologie, Université de Montpellier; INSERM U1046, UMR 9214, Hôpital Arnaud de Villeneuve, Montpellier, France
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Florence Parent
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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David Montani
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Gérald Simonneau
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Marc Humbert
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Olivier Sitbon
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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  • For correspondence: olivier.sitbon@bct.aphp.fr
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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.

Abstract

Simplified risk assessment using the number of low-risk criteria predicts prognosis at baseline and follow-up in PAH http://ow.ly/KMsj30cPNbm

Footnotes

  • This article has supplementary material available from erj.ersjournals.com

  • Support statement: This work was supported in part by the Assistance Publique-Hôpitaux de Paris (PHRC EFORT: ClinicalTrials.gov Identifier NCT01185730), INSERM, Université Paris-Sud and Agence Nationale de la Recherche (Département Hospitalo-Universitaire Thorax Innovation; LabEx LERMIT, ANR-10-LABX-0033; and RHU BIO-ART LUNG 2020, ANR-15-RHUS-0002). J. Weatherald is the recipient of a joint European Respiratory Society/Canadian Thoracic Society Long-Term Research Fellowship (LTRF 2015 – 4780). Funding information for this article has been deposited with the Crossref Funder Registry.

  • Conflict of interest: Disclosures can be found alongside this article at erj.ersjournals.com

  • Received May 1, 2017.
  • Accepted May 29, 2017.
  • Copyright ©ERS 2017
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Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension
Athénaïs Boucly, Jason Weatherald, Laurent Savale, Xavier Jaïs, Vincent Cottin, Grégoire Prevot, François Picard, Pascal de Groote, Mitja Jevnikar, Emmanuel Bergot, Ari Chaouat, Céline Chabanne, Arnaud Bourdin, Florence Parent, David Montani, Gérald Simonneau, Marc Humbert, Olivier Sitbon
European Respiratory Journal Aug 2017, 50 (2) 1700889; DOI: 10.1183/13993003.00889-2017

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Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension
Athénaïs Boucly, Jason Weatherald, Laurent Savale, Xavier Jaïs, Vincent Cottin, Grégoire Prevot, François Picard, Pascal de Groote, Mitja Jevnikar, Emmanuel Bergot, Ari Chaouat, Céline Chabanne, Arnaud Bourdin, Florence Parent, David Montani, Gérald Simonneau, Marc Humbert, Olivier Sitbon
European Respiratory Journal Aug 2017, 50 (2) 1700889; DOI: 10.1183/13993003.00889-2017
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