Parameter | Value |
WHO group 1 subgroup | |
APAH-CTD | +1 |
APAH-PoPH | +2 |
FPAH | +2 |
Demographics and comorbidities | |
Renal insufficiency | +1 |
Males aged >60 years | +2 |
NYHA/WHO functional class | |
I | −2 |
III | +1 |
IV | +2 |
Vital signs | |
Systolic blood pressure <110 mmHg | +1 |
Heart rate >92 beats·min−1 | +1 |
6-min walking distance | |
≥440 m | −1 |
<165 m | +1 |
BNP# | |
<50 pg·mL−1 | −2 |
>180 pg·mL−1 | +1 |
Echocardiogram | |
Pericardial effusion | +1 |
Pulmonary function test | |
DLCO ≥80% predicted | −1 |
DLCO ≤32% predicted | +1 |
Right heart catheterisation | |
Mean RAP >20 mmHg within 1 year | +1 |
PVR >32 Wood units | +2 |
REVEAL: Registry to Evaluate Early and Long-term PAH Disease Management; PAH: pulmonary arterial hypertension; WHO: World Health Organization; APAH: associated with PAH; CTD: connective tissue disease; PoPH: portopulmonary hypertension; FPAH: familial PAH; NYHA: New York Heart Association; BNP: brain natriuretic peptide; DLCO: diffusing capacity of the lung for carbon monoxide; RAP: right atrial pressure; PVR: pulmonary vascular resistance. #: if N-terminal proBNP is available and BNP is not, listed cut-points are replaced with <300 pg·mL−1 and >1500 pg·mL−1.