Table 3. Percentage of New York Heart Association/World Health Organization functional class (NYHA/WHO FC) patients enrolled in randomised controlled trials (RCTs) with monotherapies
Study drugRCT acronym or study groupPatientsSubjects nNYHA/WHO FC IVDuration weeksPrimary end-point
EpoprostenolRubin et al. [22]IPAH23NA8NA
Barst et al. [23]IPAH NYHA/WHO FC III/IV8126%126MWD
Badesch et al. [24]SSc11117%126MWD
TreprostinilSimonneau et al. [25]IPAH, CTD, CHD4697%126MWD
BeraprostALPHABET [26]PAH1300%126MWD
Barst et al. [27]IPAH, CTD, CHD1160%52TTCW
IloprostAIR [28]IPAH, CTD, CTEPH20341%12Composite
BosentanBREATHE-1 [29]IPAH, CTD2138.5%166MWD
AmbrisentanARIES 1 [30]PAH2027%126MWD
ARIES 2 [30]PAH1922.3%126MWD
SildenafilSUPER 1 [31]IPAH, CTD, CHD2773%126MWD
TadalafilPHIRST [32]PAH4051.7%166MWD
  • PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; SSc: systemic sclerosis-associated PAH; CTD: connective tissue diseases-associated PAH; CHD: congenital heart disease-associated PAH; CTEPH: chronic thromboembolic pulmonary hypertension; NA: not applicable; 6MWD: 6-min walk distance; TTCW: time to clinical worsening.