First author [ref.] | Study acronym | Patients n | Study period weeks | Primary end-point | Deaths n | |
Study drug | Comparator | |||||
Rubin [7] | 23 | 8 | Haemodynamics# | Epoprostenol, n=1 | Conventional therapy, n=3 | |
Barst [8] | 81 | 12 | Survival and change in 6MWD# | Epoprostenol, n=0 | Conventional therapy, n=8 | |
Badesch [9] | 111 | 12 | Change in 6MWD | Epoprostenol, n=4 | Conventional therapy, n=5 | |
Simonneau [10] | 470 | 12 | Change in 6MWD | Treprostinil, n=9 | Placebo, n=10 | |
Hiremath [11] | TRUST | 44 | 12 | Change in 6MWD | Treprostinil, n=3 | Placebo, n=5 |
Galiè [12] | ALPHABET | 130 | 12 | Change in 6MWD | Beraprost, n=1 | Placebo, n=1 |
Barst [13] | 116 | 36 | Disease progression: death, transplantation, epoprostenol rescue or 25% decrease in peak oxygen consumption | Beraprost, n=1 | Placebo, n=2 | |
Olschewski [14] | AIR | 203 | 12 | Improved by one NYHA FC and 10% increase in 6MWD | Iloprost, n=1 | Placebo, n=4 |
McLaughlin [15] | STEP | 67 | 12 | Change in 6MWD, NHYA FC, Borg dyspnoea index# | Iloprost/bosentan, n=0 | Placebo/bosentan, n=0 |
Hoeper [16] | COMBI | 40 | 12 | Change in 6MWD | Iloprost/bosentan, n=0 | Placebo/bosentan, n=0 |
Channick [17] | 32 | 12 | Change in 6MWD | Bosentan, n=0 | Placebo, n=0 | |
Rubin [18] | BREATHE-1 | 213 | 16 | Change in 6MWD | Bosentan, n=4¶ | Placebo, n=2 |
Galiè [19] | EARLY | 185 | 24 | Change in PVR and 6MWD | Bosentan, n=1 | Placebo, n=1 |
Galiè [20] | BREATHE-5 | 54 | 16 | Change in PVR | Bosentan, n=0 | Placebo, n=0 |
Humbert [21] | BREATHE-2 | 33 | 16 | Change in total pulmonary resistance | Epoprostenol plus bosentan, n=3+ | Epoprostenol plus placebo, n=0 |
Wilkins [22] | SERAPH | 26 | 16 | Changes in right ventricular mass | Bosentan, n=0 | Sildenafil, n=1 |
Galiè [23] | ARIES | 394 | 12 | Change in 6MWD | Ambrisentan, n=4 | Placebo, n=6 |
Barst [24] | STRIDE-1 | 178 | 12 | Change in peak oxygen consumption | Sitaxentan, n=1 | Placebo, n=0 |
Barst [25] | STRIDE-2 | 247 | 18 | Change in 6MWD | Sitaxentan, n=0 | Placebo, n=2 Bosentan, n=0 |
Sandoval [26] | STRIDE-4 | 98 | 18 | Change in 6MWD | Sitaxentan, n=0 | Placebo, n=0 |
Sastry [27] | 22 | 12 | Change in 6MWD | Sildenafil, n=0 | Placebo, n=1 | |
Galiè [28] | SUPER-1 | 278 | 12 | Change in 6MWD | Sildenafil, n=3 | Placebo, n=1 |
Singh [29] | 20 | 8 | Change in 6MWD | Sildenafil, n=0 | Placebo, n=0 | |
Simonneau [30] | PACES | 267 | 16 | Change in 6MWD | Sildenafil, n=0 | Placebo, n=7 |
Galiè [31] | PHIRST | 405 | 16 | Change in 6MWD | Tadalafil, n=2 | Placebo, n=1 |
Jing [32] | EVALUATION | 66 | 12 (+12 open label) | Change in 6MWD | Vardenafil, n=1 | Placebo, n=2 |
Langleben [33] | 71 | 12 | Change in 6MWD | Terbogrel, n=1 | Placebo, n=0 | |
Ghofrani [34] | 59 | 24 | Change in 6MWD | Imatinib, n=3 | Placebo, n=3 | |
Hoeper [35] | IMPRES | 202 | 24 | Change in 6MWD | Imatinib, n=5 | Placebo, n=5 |
6MWD: 6-min walk distance; NYHA FC: New York Heart Association functional class; PVR: pulmonary vascular resistance. #: primary end-point not specified; ¶: three patients were in the post 12-week treatment period; +: there was one death after withdrawal from study.