Table 2

Patient Characteristics

Diagnostic SubgroupaPAH excl. CHD (n = 21)PH Group 3 (n = 38)PH Groups 4 and 5 (n = 6)
IPAH/FPAH (n = 212)PAH-CHD (n = 105)
Age at diagnosis, yrs7.8 (4.2–13.0)6.1 (2.3–11.9)10.8 (7.2–14.1)3.7 (1.4–9.7)13.3 (6.1–13.6)
Female133 (62.7)60 (57.1)11 (52.4)23 (60.5)2 (33.3)
Incident patients106 (50)54 (51.4)10 (47.6)17 (44.7)4 (66.7)
WHO functional class
 I28 (13.2)15 (14.3)3 (14.3)6 (15.8)1 (16.7)
 II89 (42.0)52 (49.5)8 (38.1)19 (50.0)2 (33.3)
 III73 (34.4)37 (35.2)6 (28.6)12 (31.6)3 (50.0)
 IV22 (10.4)1 (1.0)4 (19.0)1 (2.6)
Sedation method at HC
 General anesthesia101 (47.9)56 (53.8)12 (57.1)22 (57.9)2 (33.3)
 Procedural sedation110 (52.1)48 (46.2)9 (42.9)16 (42.1)4 (66.7)
Baseline mPAP, mm Hg59 ± 1858 ± 2050 ± 1545 ± 1560 ± 10
Baseline mRAP, mm Hg7 ± 47 ± 47 ± 37 ± 38 ± 3
Baseline PVRi, WU·m215.9 (10.5–22.6)11.9 (8.1–20.3)12.5 (8.6–18.3)9.0 (6.4–11.5)17.3 (13.2–24.0)
Baseline cardiac index, l/min/m23.1 (2.4–4.1)3.3 (2.5–3.9)3.3 (3.0–4.0)3.3 (2.8–4.6)3.4 (1.8–4.1)
AVT agent
 iNO (and O2)152 (71.7)64 (61.0)15 (71.4)32 (84.2)3 (50.0)
 Other60 (28.3)41 (39.0)6 (28.6)6 (15.8)3 (50.0)

Values are median (interquartile range), n (%), or mean ± SD.

aPAH excl. CHD = associated pulmonary arterial hypertension excluding congenital heart disease; AVT = acute vasodilator response testing; HC = heart catheterization; IPAH/FPAH = idiopathic/familial pulmonary arterial hypertension; iNO = inhaled nitric oxide; mRAP = mean right atrial pressure; PAH-CHD = pulmonary arterial hypertension associated with congenital heart disease; PH = pulmonary hypertension; PVRi = pulmonary vascular resistance index; WHO = World Health Organization; WU = Woods units; other abbreviations as in Table 1.

  • Other agents included adenosine, diltiazem, epoprostenol, iloprost, oxygen, and sildenafil.