Study population | Recruitment period | Variables | End-point | Validation | |
French registry equation [4, 5] | Idiopathic, familial and anorexigen-associated PAH n=190, 29% incident and 71% prevalent cases | 2002–2003, follow-up time 3 yrs for all patients | 6MWD, sex, CO | Survival up to 3 yrs post-diagnosis | Prospective validation in PAH cohorts from clinical trials |
Pulmonary Hypertension Connection registry equation [3] | Idiopathic, familial and anorexigen-associated PAH n=282, incident and prevalent cases | 1991–2007, median (interquartile range) follow-up time 3.9 (1.7–7.8) yrs, maximum follow-up time 16.6 yrs | Pra, mean Ppa, CI | Survival at number of yrs post-diagnosis | Prospective validation in PAH cohorts from clinical trials |
REVEAL registry equation and risk score [6, 7] | WHO Group I PAH n=2716, 14% incident and 86% prevalent cases | 2006 onwards, mean follow-up time 1.4 yrs, range 0–2 yrs | Age, aetiology, sex, renal insufficiency, SBP, HR, WHO FC, 6MWD, BNP or NT-pro-BNP, Pra, PVR, ipresence of pericardial effusion, % pred DL,CO | 1-yr survival | Prospective validation in newly diagnosed PAH patients from REVEAL |
Scottish composite Score | WHO Group I PAH (except for CHD-PAH) n=182, all incident cases | 2000–2009, median follow-up time 2.1yrs, range 3 days to 9.4 yrs | Age, aetiology, sex, 6MWD, Pra and CO | Survival at number of yrs post-diagnosis | Retrospective validation in an independent UK idiopathic and heritable PAH cohort |
REVEAL: Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management; 6MWD: 6-min walk distance; CO: cardiac output; Pra: right atrial pressure; Ppa: pulmonary artery pressure; CI: cardiac index; WHO: World Health Organization; SBP: systolic blood pressure; HR: heart rate; FC: functional class; BNP: brain natriuretic peptide; NT-pro-BNP: N-terminal-pro BNP; PVR: pulmonary vascular resistance; DL,CO: diffusing capacity of the lung for carbon monoxide; CHD-PAH: congenital heart disease associated PAH.