| Subjects | 19 |
| Age years (range) | 39.4±14.2 (18.1–63.1) |
| Females | 17 (89) |
| Idiopathic/heritable/anorexigen-associated PAH | 9/10/0 |
| BMPR2 mutation carrier n/n tested (%) | 10/13 (77) |
| NYHA functional class III/IV | 8 (42)/11 (58) |
| 6-min walk distance m | 215±174 |
| Haemodynamics | |
| Right atrial pressure mmHg | 12.2±5.2 |
| Mean pulmonary arterial pressure mmHg | 67.7±15.8 |
| Pulmonary capillary wedge pressure mmHg | 8.3±3.4 |
| Cardiac output L·min−1 | 2.83±0.77 |
| Cardiac index L·min−1·m−2 | 1.64±0.34 |
| Pulmonary vascular resistance dyn·s·cm−5 | 1807±722 |
| Mean blood pressure mmHg | 91.7±12.2 |
| Heart rate beats per min | 92.3±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.