Table 1. Recommendations for efficacy of specific drug therapy for pulmonary arterial hypertension (group 1) according to New York Heart Association functional class (NYHA FC)
TreatmentNYHA FC
    Epoprostenol i.v.I-AI-A
    Iloprost inhaledI-AIIa-C
    Iloprost i.v.IIa-CIIa-C
    Treprostinil subcutaneousI-BIIa-C
    Treprostinil i.v.IIa-CIIa-C
    Treprostinil inhaledI-BIIa-C
Endothelin receptor antagonists
Phophodiesterase type 5 inhibitors
Calcium channel blockersI-C#I-C#
Initial drugs combination therapy
Sequential drugs combination therapyIIa-CIIa-B
  • Data are presented as conflicting evidence (levels of evidence A–C) and/or a divergence of opinion about the usefulness/efficacy of the given treatment or procedure (termed class I–III). Class I: evidence and/or general agreement that a given treatment or procedure is beneficial, useful, effective; class II and IIa: weight of evidence/opinion is in favour of usefulness/efficacy; class IIb: usefulness/efficacy is less well established by evidence/opinion; class III: evidence or general agreement that the given treatment or procedure is not useful/effective, and in some cases may be harmful; level A: data derived from multiple, randomised clinical trials or meta-analyses; level B: data derived from a single, randomised clinical trial or large nonrandomised studies; level C: consensus of opinion of the experts and/or small studies, retrospective studies, registries. #: only for responders to acute vasoreactivity tests.