Table 1.

Demographic, hemodynamic, and functional characteristics at bosentan initiation

	All patients* (N = 86)	Patients with IPAH/HPAH (N = 36)		Patients with PAH-CHD (N = 48)
Treatment at bosentan initiation		Monotherapy (n = 11)	Plus prostanoid (n = 25)	Monotherapy (n = 30)	Plus prostanoid (n = 18)
Male:Female	37:49	6:5	14:11	9:21	6:12
Age
At bosentan initiation	11 ± 5 [0–18]	10 ± 6 [1–16]	11 ± 4 [4–16]	10 ± 6 [0–18]	12 ± 4 [6–18]
At pulmonary arterial hypertension diagnosis	5 ± 5 [0–16]	8 ± 5 [1–15]	5 ± 5 [0–16]	3 ± 4 [0–13]	6 ± 4 [1–15]
Weight at bosentan initiation	34 ± 16 [5–89]	35 ± 17 [8–62]	37 ± 17 [14–89]	30 ± 16 [5–64]	37 ± 15 [17–57]
Hemodynamic parameters**
Mean pulmonary artery pressure (mmHg)	63 ± 20 (n = 67)	58 ± 18 (n = 10)	65 ± 26 (n = 17)	60 ± 16 (n = 26)	75 ± 20 (n = 12)
Mean pulmonary capillary wedge pressure (mmHg)	9 ± 3 (n = 67)	9 ± 2 (n = 10)	8 ± 3 (n = 17)	9 ± 4 (n = 26)	9 ± 3 (n = 12)
Mean right atrial pressure (mmHg)	7 ± 4 (n = 67)	5 ± 2 (n = 10)	7 ± 4 (n = 17)	7 ± 4 (n = 26)	9 ± 3 (n = 12)
Cardiac index (L/min/m2)	3.6 ± 1.4 (n = 62)	3.7 ± 1.3 (n = 10)	3.8 ± 1.2 (n = 17)	3.3 ± 1.3 (n = 23)	4.4 ± 2.0 (n = 10)
Pulmonary vascular resistance index (U.m2)	20 ± 14 (n = 66)	16 ± 13 (n = 10)	20 ± 17 (n = 17)	22 ± 14 (n = 25)	21 ± 12 (n = 12)
WHO functional class: I: II: III: IV	6: 36: 33: 6	0: 4: 6: 1	3: 8: 10: 2	2: 16: 9: 2	1: 8: 7: 0

Values are number (percentage) of patients or mean ± standard deviation [range].

^*Two male patients with PAH associated with connective tissue disease were included in the all-patient group: one patient had bosentan monotherapy, one had pre-existing prostanoid treatment at baseline, they were 8 and 10 year old at initiation and also at diagnosis, they weighted 22 and 43 kg, PAPm was 38 and 54 mmHg.

^**Most recent right heart catheterization (RHC) within 3 months of bosentan initiation.