RT Journal Article SR Electronic T1 Predicting survival in pulmonary arterial hypertension in the UK JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 604 OP 611 DO 10.1183/09031936.00196611 VO 40 IS 3 A1 Lee, Wai-Ting Nicola A1 Ling, Yi A1 Sheares, Karen K. A1 Pepke-Zaba, Joanna A1 Peacock, Andrew John A1 Johnson, Martin Keith YR 2012 UL http://erj.ersjournals.com/content/40/3/604.abstract AB Contemporary prognostic equations in pulmonary arterial hypertension (PAH) derived from US and French cohorts may not perform as well in the UK as a locally derived scoring scheme. The aim of the study was to develop and validate a UK risk score to predict prognosis in PAH.Baseline mortality predictors identified by multivariate Cox analysis in 182 incident PAH patients were used to derive the Scottish composite score (SCS). Its prognostic performance in an independent UK cohort was compared with the French registry and Pulmonary Hypertension Connection (PHC) registry equations using Brier scores (BS).The SCS based on age, sex, aetiology, right atrial pressure, cardiac output and 6-min walk distance predicted survival in the validation cohort (hazard ratio (HR) 1.7 per point increase; p<0.001) and provided further prognostic stratification in World Health Organization (WHO) functional class III patients (HR 1.8 per point increase; p<0.001). It was more accurate than the French registry equation in predicting 1-yr survival (BS: 0.092 versus 0.146; p=0.001) and 2-yr survival (0.131 versus 0.255; p<0.001). There was no significant difference in BS between the SCS and PHC registry equation.The SCS predicts survival and can be used to supplement WHO functional class in prognostication.