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Eur Respir J. 2017 Aug 3;50(2). pii: 1700889. doi: 10.1183/13993003.00889-2017. Print 2017 Aug.

Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension.

Author information

1
Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
2
AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
3
INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
4
Department of Medicine, Division of Respirology, University of Calgary, Calgary, AB, Canada.
5
Université 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.
6
Service de Pneumologie, Hôpital Larrey, Toulouse, France.
7
Service de Cardiologie, Hôpital Haut-Lévêque, Pessac, France.
8
Hôpital Cardiologique de Lille, Centre de Compétences de l'Hypertension Pulmonaire, Lille, France.
9
Hôpital Côte de Nacre, Centre de Compétences Basse Normandie de l'Hypertension Pulmonaire, Université de Caen-Basse Normandie, Caen, France.
10
CHU Nancy, Pôle des Spécialités Médicales, Département de Pneumologie, Vandoeuvre-lès-Nancy, France.
11
Université de Lorraine, INGRES, EA 7298, Vandoeuvre-lès-Nancy, France.
12
CHU de Rennes, Service de Cardiologie et Maladies Vasculaires, INSERM U1099, Rennes, France.
13
Service de Pneumologie, Université de Montpellier; INSERM U1046, UMR 9214, Hôpital Arnaud de Villeneuve, Montpellier, France.
14
Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France olivier.sitbon@bct.aphp.fr.

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-21017 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.

PMID:
28775050
DOI:
10.1183/13993003.00889-2017
[Indexed for MEDLINE]

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