{Reference Type}: Journal Article
{Title}: Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension.
{Author}: Boucly A;Weatherald J;Savale L;Jaïs X;Cottin V;Prevot G;Picard F;de Groote P;Jevnikar M;Bergot E;Chaouat A;Chabanne C;Bourdin A;Parent F;Montani D;Simonneau G;Humbert M;Sitbon O;
{Journal}: Eur Respir J
{Volume}: 50
{Issue}: 2
{Year}: 08 2017
{Factor}: 33.795
{DOI}: 10.1183/13993003.00889-2017
{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.