Table 2.

Major clinical characteristics of the three subgroups within the validation cohort (n = 197) at time of diagnosis.

	Subgroup I	Subgroup II	Subgroup V	All patients
n	96	70	31	197
Age, years	65 [55–74]	40 [29–49]	45 [30–63]	54 [39–67]
Gender: female	56 (58%)	58 (83%)	18 (58%)	132 (67%)
Vasoresponse	7 (16.3%)	8 (21.6%)	2 (12.5%)	17 (17.7%)
Treatments
Phosphodiesterase 5 inhibitors (PDE5i)	23 (29.5%)	10 (18.5%)	8 (29.6%)	41 (25.8%)
Endothelin receptor antagonist (ERA)	3 (3.85%)	5 (9.26%)	1 (3.70%)	9 (5.66%)
PDE5i & ERA combination	48 (61.5%)	29 (53.7%)	17 (63.0%)	94 (59.1%)
Prostacyclin agonist	1 (1.28%)	2 (3.70%)	1 (3.70%)	4 (2.52%)
Calcium channel blockers	3 (3.85%)	8 (14.8%)	0 (0.00%)	11 (6.92%)
WHO functional class
I	4 (4.2%)	11 (15.7%)	2 (6.5%)	17 (8.6%)
II	22 (22.9%)	26 (37.1%)	15 (48.4%)	63 (32.0%)
III	60 (62.5%)	32 (45.7%)	12 (38.7%)	104 (52.8%)
IV	10 (10.4%)	1 (1.4%)	2 (6.5%)	13 (6.6%)
6-minute walking distance, m	306 (152)	419 (123)	409 (120)	360 (148)
NT-proBNP, ng/l	492 [196; 1327]	188 [90.0; 400]	266 [128; 499]	303 [128; 1092]
Forced expiratory volume [% predicted]	84 (21)	90 (19)	90 (17)	87 (20)
Forced vital capacity [% predicted]	94 (21)	98 (20)	99 (15)	96 (20)
Transfer factor of lung for carbon monoxide [% predicted]	92 (15)	98 (17)	96 (11)	95 (15)
Diagnostic Right Heart Catheter Study
Mean pulmonary artery pressure, mmHg	50 [43; 57]	48 [42; 58]	49 [41; 57]	49 [42; 57]
Mean right atrial pressure, mmHg	9 [7; 12]	7 [5; 10]	6 [3; 7]	8 [5; 12]
Mean pulmonary Arterial wedge pressure, mmHg	10 (3)	8 (4)	8 (4)	9 (4)
Cardiac Index, l/min/m	2.0 [1.7; 2.5]	2.1 [1.7; 2.5]	2.0 [1.8; 2.4]	2.0 [1.7; 2.5]
Pulmonary vascular resistance, Wood Units	11 [7; 14]	11 [9; 15]	12 [10; 14]	11 [8; 15]

Intervals describe first and third quartiles. Parentheses describe standard deviation (SD).