Class | First author | Year | Drug | N | FC % change II/III/IV or mean | RCT | Main findings |
---|---|---|---|---|---|---|---|
ERA | Galiè [20] | 2006 | Bosentan | 54 | 0/100/0 | Yes | ↓PVRi, ↓mPAP,↑6MWD |
 | Zuckerman [40] | 2011 | Ambrisentan | 17 | NR | No | ↑6MWD |
 | Blok [28] | 2017 | Macitentan | 40 | 52/48/0 | No | ↓FC, ↓NT-proBNP, ↔6MWD |
 | Herbert [29] | 2017 | Macitentan | 15 | NR | No | ↑6MWD, ↑SaO2 |
 | Galie [41] | 2017 | Macitentan | 226 | 60/40/0 | Yes | ↔6MWD, ↓NT-proBNP, ↓PVRi |
PDE5i | Mukhopadhyay [7] | 2006 | Sildenafil | 16 | 69/31/0 | No | ↑6MWD, ↑SaO2, ↓mPAP, ↓PVR |
 | Chau [42] | 2007 | Sildenafil | 7 | 3.3 ± 0.7 | No | ↓FC, ↑SaO2, ↓mPAP, ↓PVR |
 | Garg [10] | 2007 | Sildenafil | 21 | 46/54/0 | No | ↓FC, ↑6MWD, ↑SaO2, ↓mPAP, ↓PVRi |
 | Tay [11] | 2011 | Sildenafil | 12 | 0/100/0 | No | ↓FC, ↑6MWD, ↑HRQOL |
 | Zhang [12] | 2011 | Sildenafil | 84 | 52/39/8 | No | ↑6MWD, ↑SaO2, ↓mPAP, ↓PVRi |
 | Mukhopadhyay [14] | 2011 | Tadalafil | 28 | 79/21/0 | Yes | ↓FC, ↑6MWD, ↓PVR |
 | Sun [13] | 2013 | Sildenafil | 29 | NR | No | ↓FC, ↑6MWD, ↓PVR |
Prostanoid | Rosenzweig [32] | 1999 | Epoprostenol (intravenous) | 20 | 15/50/35 | No | ↓mPAP, ↑CI, ↓PVRi,↑SvO2 |
 | Fernandes [33] | 2003 | Epoprostenol (intravenous) | 8 | 0/25/75 | No | ↑6MWD |
 | Thomas [43] | 2013 | Epoprostenol (intravenous) & Treprostinil (subcutaneous) | 9 | 12/88/0 | No | ↓mPAP, ↑SvO2 |
 | Skoro-Sajer [44] | 2018 | Treprostinil (subcutaneous) | 22 | 0/47/53 | No | ↓FC, ↓PVR, ↓NT-proBNP, ↑6MWD |
 | Yang [36] | 2012 | Iloprost (nebulised) | 12 | 0/75/25 | No | ↓FC, ↑6MWD, ↑SaO2, |
 | Cha [37] | 2013 | Iloprost (nebulised) | 13 | 0/69/31 | No | ↓FC, ↑6MWD, ↑HRQoL |
 | Chon [38] | 2017 | Iloprost (nebulised) | 11 | 0/64/36 | No | ↓FC, ↓mPAP, ↓PVR, ↑SaO2, ↑6MWD |
Combination | Iversen [45] | 2010 | Bosentan +/− Sildenafil | 21 | 43/48/5 | Yes | ↔6MWD, ↑SaO2 |
 | D’Alto [46] | 2012 | Bosentan & Sildenafil | 32 | 2.9 ± 0.3 | No | ↓FC,↑6MWD, ↓mPAP, ↓PVR, ↓NT-proBNP |
 | Sitbon [30] | 2020 | Macitentan & Tadalafil | 46 | 63% improved FC | No | ↓PVR, ↓NT-proBNP |