Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, FranceAP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, FranceInserm UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
Simplified REVEAL (Registry to Evaluate Early And Long-term PAH Disease Management) risk score. PAH: pulmonary arterial hypertension; WHO: World Health Organization; NYHA: New York Heart Association; BNP: brain natriuretic peptide; APAH; associated PAH; CTD: connective tissue disease; PoPH: portopulmonary hypertension; FPAH: familial PAH; SBP: systolic blood pressure; HR: heart rate; DLCO: diffusing capacity of the lung for carbon monoxide; mRAP: mean right atrial pressure; PVR: pulmonary vascular resistance. Reproduced from  with permission from the publisher.
1-year survival in the REVEAL (Registry to Evaluate Early And Long-term PAH Disease Management) validation cohort as predicted according to the a) risk prognostic equation and b) simplified risk score. PAH: pulmonary arterial hypertension. Reproduced from  with permission from the publisher.
RA area 18–26 cm2 No or minimal, pericardial effusion
RA area >26 cm2 Pericardial effusion
RAP <8 mmHg CI ≥2.5 L·min−1·m−2 SvO2 >65%
RAP 8–14 mmHg CI 2.0–2.4 L·min−2·m−2 SvO2 60–65%
RAP >14 mmHg CI <2.0 L·min−1·m−2 SvO2 <60%
WHO: World Health Organization; 6MWD: 6-min walking distance; NT-proBNP: N-terminal pro-brain natriuretic peptide; CMR: cardiac magnetic resonance; V′O2: oxygen uptake; V′E/V′CO2: ventilatory equivalents for carbon dioxide; BNP: brain natriuretic peptide; RA: right atrium; RAP: right atrial pressure; CI: cardiac index; SvO2: mixed venous oxygen saturation. #: most of the proposed variables and cut-off values are based on expert opinion. They may provide prognostic information and may be used to guide therapeutic decisions, but application to individual patients must be done carefully. One must also note that most of these variables have been validated mostly for idiopathic pulmonary arterial hypertension and the cut-off levels used above may not necessarily apply to other forms of pulmonary arterial hypertension. Furthermore, the use of approved therapies and their influence on the variables should be considered in the evaluation of the risk. ¶: occasional syncope during brisk or heavy exercise, or occasional orthostatic syncope in an otherwise stable patient. +: repeated episodes of syncope, even with little or regular physical activity. Reproduced from  with permission from the publisher.