Table 1.
Treatment recommendations from the 5th World Symposium on Pulmonary Hypertension17
| Initial therapy with PAH approved drugs | ||||
|---|---|---|---|---|
| Recommendation | Evidence* | WHO FC II | WHO FC III | WHO FC IV |
| I | A or B | Ambrisentan Bosentan Macitentan Riociguat Sildenafil Tadalafil |
Ambrisentan Bosentan Epoprostenol intravenous Iloprost inhaled Macitentan Riociguat Sildenafil Tadalafil Treprostinil subcutaneous, inhaled† |
Epoprostenol intravenous |
| IIa | C | Iloprost intravenous† Treprostinil intravenous |
Ambrisentan Iloprost inhaled, intravenous† Macitentan Riociguat Sildenafil, tadalafil Treprostinil subcutaneous, intravenous, inhaled† |
|
| IIb | B | Beraprost† | ||
| C | Initial combination therapy | Initial combination therapy | ||
Reprinted with permission from Elsevier.
Level definition:
A: Data derived from multiple randomised clinical trials or meta-analyses.
B: Data derived from a single randomised clinical trial or large non-randomised studies.
C: Consensus of opinion of the experts and/or small studies, retrospective studies, registries.
Results on the basis of post-hoc and subgroup analyses of clinical trials most often do not meet the criteria of a level of evidence A.
Classes of recommendations.
Class I: Evidence and/or general agreement that a given treatment or procedure is beneficial, useful, effective. Is recommended, is indicated.
Class II: Conflicting evidence and/or a divergence of opinion about the usefulness/efficacy of the given treatment or procedure.
Class IIa: Weight of evidence/opinion is in favour of usefulness/efficacy. Should be considered.
Class IIb: Usefulness/efficacy is less well established by evidence/opinion. May be considered.
Class III: Evidence or general agreement that the given treatment or procedure is not useful/effective, and in some cases may be harmful. Is not recommended.
*Level of evidence is based on the WHO FC of the majority of patients in the studies.
†Approved only: by the FDA (treprostinil inhaled); in New Zealand (iloprost intravenous); in Japan and South Korea (beraprost).
FDA, US Food and Drug Administration; PAH, pulmonary arterial hypertension; WHO FC, WHO functional class.