Evidence-based monotherapy treatment algorithm for drugs that target the prostacyclin pathway

RecommendationEvidencePAH severity
Recommended  (class I)Data derived from multiple randomised clinical trials or meta-analyses, or from a single randomised clinical trial or large nonrandomised studiesSelexipag oral#Epoprostenol i.v.; iloprost inhaled+; treprostinil s.c. and inhaled§; selexipag oral#Epoprostenol i.v.
Should be considered  (class IIa)Consensus of opinion of the experts and/or small studies, retrospective studies and registriesIloprost i.v.+; treprostinil i.v.§
May be considered  (class IIb)Data derived from a single randomised clinical trial or large nonrandomised studiesBeraprost oralƒ; treprostinil oralIloprost inhaled and i.v.; treprostinil s.c., i.v. and inhaled
  • Recommendation IA for prostanoid use in sequential combination therapy for inadequate clinical response at maximal therapy [3]. WHO: World Health Organization; FC: functional class. #: not approved at the time of publication; : approved for continuous i.v. administration for pulmonary arterial hypertension (PAH) WHO FC III–IV by the US Food and Drug Administration (FDA) in 1995; +: approved for aerosol administration for PAH WHO FC III in the European Union and Australia in 2003, and PAH WHO FC III–IV by the FDA in 2004; §: approved for s.c. administration for PAH WHO FC II–IV by the FDA and Health Canada in 2002; ƒ: approved for oral administration for idiopathic PAH in Japan in 1995 [36]. Information from [15, 16].