TABLE 1

Patient characteristics

BaselineStudy enrolmentp-value
Male/female3/29
Age years28.5±10.333.0±10.1<0.01
Body mass index kg·m−220.7±3.019.8±2.60.09
WHO functional class# (I/II/III/IV)III (0/6/14/12)II (7/25/0/0)<0.01
PAH-specific therapy
 Intravenous epoprostenol1 (3)26 (81)<0.01
 Beraprost11 (34)5 (16)0.18
 Endothelin receptor antagonist9 (28)30 (94)<0.01
 Phosphodiesterase type 5 inhibitor6 (19)21 (66)<0.01
 Soluble guanylate cyclase stimulator0 (0)1 (3)
 Combination therapy
  Dual therapy2 (6)13 (41)<0.01
  Triple therapy4 (13)19 (59)<0.01
Laboratory parameters
BNP pg·mL−1334.5 (8.7–1687.6)10.3 (5.8–131.7)<0.01
Respiratory parameters
 Vital capacity % predicted89.9±17.293.7±14.40.03
 FEV1/FVC %84.1±7.382.5±7.20.24
DLCO % predicted58.5±12.666.3±10.8<0.01
Pulmonary haemodynamic parameters
 PAWP mmHg8.3±3.47.9±3.20.57
 mPAP mmHg61.8±18.328.7±12.2<0.01
 Cardiac index L·min−1·m−22.17±0.663.59±0.89<0.01
 PVR Wood units18.3±8.04.3±2.9<0.01
Exercise capacity
6MWD m287.3±117.1449.7±69.2<0.01

Data are presented as n, mean±sd, n (%) or median (range), unless otherwise stated. WHO: World Health Organization; PAH: pulmonary arterial hypertension; BNP: brain natriuretic peptide; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide; PAWP: pulmonary artery wedge pressure; mPAP: mean pulmonary arterial pressure; PVR: pulmonary vascular resistance; 6MWD: 6-min walk distance. #: median (n).