Table 1– Patients’ baseline characteristics
Subjects19
Age years (range)39.4±14.2 (18.1–63.1)
Females17 (89)
Idiopathic/heritable/anorexigen-associated PAH9/10/0
BMPR2 mutation carrier n/n tested (%)10/13 (77)
NYHA functional class III/IV8 (42)/11 (58)
6-min walk distance m215±174
Haemodynamics
 Right atrial pressure mmHg12.2±5.2
 Mean pulmonary arterial pressure mmHg67.7±15.8
 Pulmonary capillary wedge pressure mmHg8.3±3.4
 Cardiac output L·min−12.83±0.77
 Cardiac index L·min−1·m−21.64±0.34
 Pulmonary vascular resistance dyn·s·cm−51807±722
 Mean blood pressure mmHg91.7±12.2
 Heart rate beats per min92.3±10.7
 Mixed venous oxygen saturation %50.1±9.0
  • Data are presented as n, mean±sd or n (%), unless otherwise stated. PAH: pulmonary arterial hypertension; BMPR2: bone morphogenetic protein receptor type II; NYHA: New York Heart Association.