RT Journal Article SR Electronic T1 Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 1700889 DO 10.1183/13993003.00889-2017 VO 50 IS 2 A1 Athénaïs Boucly A1 Jason Weatherald A1 Laurent Savale A1 Xavier Jaïs A1 Vincent Cottin A1 Grégoire Prevot A1 François Picard A1 Pascal de Groote A1 Mitja Jevnikar A1 Emmanuel Bergot A1 Ari Chaouat A1 Céline Chabanne A1 Arnaud Bourdin A1 Florence Parent A1 David Montani A1 Gérald Simonneau A1 Marc Humbert A1 Olivier Sitbon YR 2017 UL http://erj.ersjournals.com/content/50/2/1700889.abstract AB 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.Simplified risk assessment using the number of low-risk criteria predicts prognosis at baseline and follow-up in PAH http://ow.ly/KMsj30cPNbm