Drug | Study acronym | Parameters that improved | [Ref.] | |||||
Exercise capacity | FC | Haemodynamics | TTCW | Signs and symptoms | Survival | |||
Endothelin pathway | ||||||||
Bosentan | Study 351 BREATHE-1 | ↑ | ↑ | ↑ | ↑ | ↑ | NT | [4, 5] |
Sitaxsentan# | STRIDE-1 and 2 | ↑ | ↑ | ↑ | ↓ | ↓ | NT | [6, 7] |
Ambrisentan | ARIES-1 and 2 | ↑ | ↑ | ↑ | ↑ | ↑ | NT | [8] |
NO pathway | ||||||||
Sildenafil | SUPER-1 | ↑ | ↑ | ↑ | ↓ | ↓ | NT | [9] |
Tadalafil | PHIRST | ↑ | ↓ | ↑ | ↑ | ↓ | NT | [10] |
Prostacyclin pathway | ||||||||
Intravenous epoprostenol | ↑ | ↑ | ↑ | NT | NT | ↑ | [11, 12] | |
Inhaled iloprost | AIR | ↑ | ↑ | ↑ | ↑¶ | ↑ | NT | [13] |
Subcutaneous treprostinil | ↑ | NT | ↑ | ↑ | ↑ | NT | [14] | |
Inhaled treprostinil | TRIUMPH | ↑ | ↓ | NT | ↓ | ↓ | NT | [15] |
Oral beraprost | ALPHABET | ↑ | ↑ | ↓ | ↑ | ↑ | NT | [16, 17] |
FC: functional class; TTCW: time to clinical worsening; NO: nitric oxide; NT: not tested; ↑: parameter improved; ↓: parameter did not improve. #: withdrawn from market in 2010 due to two cases of fatal hepatic failure linked to its use; ¶: TTCW was defined as a combination of ≥10% improvement in walking distance combined with an absence of clinical deterioration.