Table 2—

Haemodynamic, lung function and exercise data at pulmonary arterial hypertension(PAH) diagnosis

SScPAHIPAHp-value
Subjects n811
ra mmHg8 (3–15)/610 (4–19)/90.30
Ppa,sys mmHg73 (35–101)/8100 (62–128)/110.15
pa mmHg45 (25–71)/860 (43–76)/90.08
PCWP mmHg7 (4–12)/87 (5–12)/90.57
PVR dyn·s·cm−5654 (227–3278)/6920 (346–1587)/90.41
CI L·min·m22.4 (1.1–3.6)/62.5 (1.3–3.2)/90.69
FEV1 % pred88 (77–97)80 (66–86)0.15
TLC % pred88 (81–115)/693 (78–115)/90.78
VC % pred97 (84–115)/789 (58–110)/80.34
TL,CO % pred40 (26–58)/768 (39–89)/100.01
TL,CO/VA %39 (26–69)/758.5 (52–87)/100.01
Pa,O2mmHg73 (58–88)65.5 (52–80)0.46
6MWD m278 (0–382)400 (100–570)0.15
Smoking history (never/current/former) n0/0/30/0/3
Therapy at time of death
Prostacycline n710
ERA n11
PDE-5 inhibitor n1
ABS n2
  • Data are presented as median (range)/n or median (range), unless otherwise stated. SScPAH: systemic sclerosis-associated PAH; IPAH: idiopathic PAH; ra: mean right atrial pressure; Ppa,sys: systolic pulmonary artery pressure; pa: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance; CI: cardiac index; FEV1; forced expiratory volume in 1 s; % pred: % predicted; TLC: total long capacity; VC: vital capacity; TL,CO: transfer factor of the lung for carbon monoxide; VA: alveolar volume; Pa,O2: arterial oxygen tension; 6MWD: 6-min walk distance; ERA: endothelin receptor antagonist; PDE-5: phosphodiesterase 5; ABS: atrial balloon septostomy.