Barst [7] | Sitbon [8] | McLaughlin [9] | |
Drug | Epoprostenol | Epoprostenol | Epoprostenol |
Design | Open-label, uncontrolled, historical control group | Single-centre series (France), matched control cohort | Single-centre series (USA) versus predicted survival (NIH registry) |
Patients | 18 IPAH | 178 IPAH | 162 IPAH |
End-points | 6MWD, haemodynamics, death | NYHA, 6MWD, PVR, death | NYHA, haemodynamics, death |
Baseline CI L·min−1·m−2 | 1.8±0.6 | 1.96±0.56 | 1.82±0.57 |
Baseline 6MWD m | 264±160 | 240±146 | |
NYHA FC II/III–V % | 6/94 | 0/100 | 0/100 |
Dose ng·kg−1·min−1 | 1 yr: 18±11 | 3 months: 14±4 | 17 months: 35±30 |
2 yrs: 37±21 | 1 yr: 21±7 | 30 months: 52±35 | |
3 yrs: 53±30 | 41 months: 33±11 | 43 months: 55±42 | |
Age yrs | 36±13 | 43±13 | 42 |
Data are presented as mean±sd unless otherwise stated. CI: cardiac index; 6MWD: 6-min walk distance; NYHA: New York Heart Association; FC: functional class; NIH: National Institutes of Health; IPAH: idiopathic pulmonary arterial hypertension; PVR: pulmonary vascular resistance.