Treatment tested | Study name | [Ref.] | Background therapy | Primary end-point | Duration | Patients n |
Ambrisentan | ATHENA-1 | [29] | Sildenafil or tadalafil | PVR | 24 weeks | 38 |
Bosentan | EARLY | [21] | None or sildenafil (16%) | PVR#, Δ6MWD (ns) | 24 weeks | 185 |
Bosentan | COMPASS-2 | [30] | Sildenafil | Morbidity/mortality | Up to 8 years | 334 |
Iloprost | STEP | [19] | Bosentan | Δ6MWD (ns) | 12 weeks | 67 |
Inhaled iloprost | VISION | [31] | Sildenafil | Δ6MWD | 16 weeks | 67 |
Iloprost | COMBI | [20] | Bosentan | Δ6MWD (ns) | 12 weeks | 40 |
Imatinib | [32] | Bosentan and/or sildenafil and/or prostanoids | Δ6MWD (ns) | 24 weeks | 59 | |
Macitentan | SERAPHIN | [28] | None, PDE-5i or inhaled iloprost | Morbidity/mortality# | 144 weeks¶ | 742 |
Riociguat | PATENT | [14] | None, bosentan or prostanoids | Δ6MWD# | 12 weeks | 443 |
Selexipag | [22] | Bosentan and/or sildenafil | PVR# | 17 weeks | 43 | |
Selexipag | GRIPHON | [33] | None, PDE-5i or ERAs | Morbidity/mortality | Up to 4.3 years | 1156 |
Sildenafil | [34] | Bosentan | Δ6MWD (ns) | 12 weeks | 104 | |
Sildenafil | PACES | [17] | Epoprostenol | Δ6MWD# | 16 weeks | 264 |
Sildenafil | [23] | Bosentan | Δ6MWD# | 12 weeks | 20 | |
Tadalafil | PHIRST | [16] | None or bosentan (54%) | Δ6MWD# | 16 weeks | 405 |
Inhaled trepostinil | TRIUMPH | [18] | Bosentan or sildenafil | Δ6MWD# | 12 weeks | 235 |
Trepostinil | FREEDOM-C1 oral | [25] | Bosentan and/or sildenafil | Δ6MWD (ns) | 16 weeks | 354 |
Trepostinil | FREEDOM-C2 oral | [26] | Bosentan and/or sildenafil | Δ6MWD (ns) | 16 weeks | 310 |
PVR: pulmonary vascular resistance; Δ6MWD: change in 6-min walking distance; ns: nonsignificant; PDE-5i: phosphodiesterase type-5 inhibitor; ERAs: endothelin receptor antagonists. #: statistically significant; ¶: event-driven study. Data from [10].