Table 7. Prostacyclin pathway in PAH—summary of clinical studies.
| Prostanoids | Study | Background | Primary endpoint | Secondary endpoints | Duration (weeks) | Patients | |
|---|---|---|---|---|---|---|---|
| Epoprostenol (Flolan) | Rubin, et al. | None | 6MWD | – | 12 | 23 | |
| Barst, et al. | None | 6MWD | Survival | 12 | 81 | ||
| Badesch, et al. | None | 6MWD | – | 12 | 111 | ||
| Treprostinil (Remodulin) | SC | None | 6MWD | – | 12 | 470 | |
| Tyvaso | TRIUMPH (Inhaled) | Bosentan or sildenafil | 6MWD | – | 12 | 235 | |
| Orenitram | FREEDOM-M (po) | None | 6MWD | – | 16 | 185 | |
| FREEDOM-C1 (po) | Bosentan and/or sildenafil | 6MWD (ns) | – | 16 | 354 | ||
| FREEDOM-C2 (po) | Bosentan and/or sildenafil | 6MWD (ns) | – | 16 | 310 | ||
| Iloprost (Ventavis) | AIR | None | 6MWD and FC | – | 12 | 203 | |
| STEP | Bosentan | 6MWD | TTCW | 12 | 67 | ||
| COMBI | Bosentan | 6MWD (ns) | – | 12 | 40 | ||
| Beraprost | ALPHABET | None | 6MWD | – | 12 | 130 | |
| Barst, et al. | None | CW (ns) | – | 52 | 116 | ||
| Prostacyclin IP-receptor agonists | |||||||
| Selexipag (Uptravi) | GRIPHON | Bosentan and/or sildenafil | Death from any cause, PAH complication | 6MWD (ns) | 36 months | 1,156 | |
6MWD, 6-minute walk distance; PAH, pulmonary arterial hypertension.