Figures
- Figure 1.
Schematic representation of the endothelin system in vascular tissue. ET-1: endothelin type 1; ETA: endothelin receptor isoform A; ETB: endothelin receptor isoform B.
- Figure 2.
Schematic representation of the guanosine monophosphate (GMP) and nitric oxide pathways. sGC: soluble guanylate cyclase; cGMP: cyclic GMP; PDE-5: phosphodiesterase type 5.
Tables
- 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)
Treatment NYHA FC II III IV Prostanoids Epoprostenol i.v. I-A I-A Iloprost inhaled I-A IIa-C Iloprost i.v. IIa-C IIa-C Treprostinil subcutaneous I-B IIa-C Treprostinil i.v. IIa-C IIa-C Treprostinil inhaled I-B IIa-C Endothelin receptor antagonists Bosentan I-A I-A IIa-C Ambrisentan I-A I-A IIa-C Phophodiesterase type 5 inhibitors Sildenafil I-A I-A Tadalafil I-B I-B Calcium channel blockers I-C# I-C# Initial drugs combination therapy Sequential drugs combination therapy IIa-C IIa-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.
