Table 2 World Health Organization functional class (WHO FC) IV patients in randomised clinical trials of pulmonary arterial hypertension
Investigational drugTrial and publication yearPatients in WHO FC IV at baseline %
EpoprostenolBarst et al. [20]; 199627
Iloprost#Olschewski et al. [39]; 200242
TreprostinilSimonneau et al. [40]; 20027
BosentanChannick et al. [41] (Study 351); 20010
Rubin et al. [42] (BREATHE-1); 20028
AmbrisentanGaliè et al. [43] (ARIES-1); 20087
Galiè et al. [43] (ARIES-2); 20082
SitaxentanBarst et al. [44] (STRIDE-1); 20041
Barst et al. [45] (STRIDE-2); 20064
SildenafilGaliè et al. [46] (SUPER-1); 20053
TadalafilGaliè et al. [47] (PHIRST-1); 20092
  • BREATHE: Bosentan Randomized Trial of Endothelin Antagonist Therapy; ARIES: Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Study; STRIDE: Sitaxsentan To Relieve Impaired Exercise; SUPER: Sildenafil Use in Pulmonary Arterial Hypertension; PHIRST: Pulmonary Arterial Hypertension and Response to Tadalafil. #: patients were stratified by WHO FC prior to being randomised.