TY - JOUR T1 - Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00889-2017 VL - 50 IS - 2 SP - 1700889 AU - Athénaïs Boucly AU - Jason Weatherald AU - Laurent Savale AU - Xavier Jaïs AU - Vincent Cottin AU - Grégoire Prevot AU - François Picard AU - Pascal de Groote AU - Mitja Jevnikar AU - Emmanuel Bergot AU - Ari Chaouat AU - Céline Chabanne AU - Arnaud Bourdin AU - Florence Parent AU - David Montani AU - Gérald Simonneau AU - Marc Humbert AU - Olivier Sitbon Y1 - 2017/08/01 UR - http://erj.ersjournals.com/content/50/2/1700889.abstract N2 - 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 ER -