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.