Table 6– Prognostic algorithms in pulmonary arterial hypertension (PAH)
Study populationRecruitment periodVariablesEnd-pointValidation
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 patients6MWD, sex, COSurvival up to 3 yrs post-diagnosisProspective 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 yrsPra, mean Ppa, CISurvival at number of yrs post-diagnosisProspective 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 yrsAge, aetiology, sex, renal insufficiency, SBP, HR, WHO FC, 6MWD, BNP or NT-pro-BNP, Pra, PVR, ipresence of pericardial effusion, % pred DL,CO1-yr survivalProspective validation in newly diagnosed PAH patients from REVEAL
Scottish composite ScoreWHO 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 yrsAge, aetiology, sex, 6MWD, Pra and COSurvival at number of yrs post-diagnosisRetrospective 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.