EMA: European Medicines Agency; ERA: endothelin receptor antagonist; PAH: pulmonary arterial hypertension; PDE-5i: phosphodiesterase type 5 inhibitor; RCT: randomized controlled trial; WHO-FC: World Health Organization functional class. ^aClass of recommendation. ^bLevel of evidence. ^cReference(s) supporting recommendations. ^dTime to clinical worsening as primary endpoint in RCTs or drugs with demonstrated reduction in all-cause mortality (prospectively defined). ^eThis drug was not approved by the EMA at the time of publication of these guidelines.