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, 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

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.

Conflict of interest statement

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

Copyright ©ERS 2017.

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