Characteristics of the patients included in the baseline risk stratification group

NLow riskIntermediate riskHigh riskAll
Subjects n19611162761588
Age years53±1866±1465±1764±16
BMI kg·m226±528±628±728±6
PAH aetiology
 I/D/H-PAH101 (52)760 (68)199 (72)1060 (67)
 CTD-PAH52 (27)234 (21)61 (22)347 (22)
 HIV-PAH4 (2)16 (1)2 (1)22 (1)
 PoPH20 (10)60 (5)9 (3)89 (6)
 CHD-PAH19 (10)46 (4)5 (2)70 (4)
WHO FC class I/II/III/IV15302/47/43/0 (unknown n=8)0/8/78/11 (unknown n=3)0/1/53/43 (unknown n=3)0/11/70/15 (unknown n=4)
6MWD m1262442±100299±109186±103298±126
NT-proBNP ng·L−11003151 (86–351)1404 (597–3042)4006 (2519–6743)1573 (526–3498)
BNP ng·L−124944 (25–96)180 (93–377)549 (418–784)236 (101–523)
 Right atrial pressure mmHg15065±38±413±58±5
 mPAP mmHg158843±1344 ±1250±1245±13
 PAWP mmHg15888±39±410±39±3
 Cardiac index L·min−1·m−214973.1±0.72.3±0.71.6±0.42.3±0.8
 PVR dyn·s·cm−51588547±273743±3721149±522784±431
SvO2 %142072±564±853±863±9
Initial therapy (within 3 months after diagnosis)
 Combination therapy19%14%27%17%

Data are presented as mean±sd, n (%) or median (interquartile range), unless otherwise stated. BMI: body mass index; PAH: pulmonary arterial hypertension; I/D/H: idiopathic, drug-associated or hereditary; CTD: connective tissue disease; PoPH: portopulmonary hypertension; CHD: congenital heart disease; WHO FC: World Health Organization functional class; 6MWD: 6-min walking distance; NT-proBNP: N-terminal fragment of pro-brain natriuretic peptide; BNP: brain natriuretic peptide; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance; SvO2: mixed venous oxygen saturation; CCB: calcium channel blocker; ERA: endothelin receptor antagonist; PDE-5i: phosphodiesterase-5 inhibitor; sGC: stimulator of soluble guanylate cyclase; PCA: prostacyclin analogue.