EMA: European Medicines Agency; PAH: pulmonary arterial hypertension; RCT: randomized controlled trial; WHO-FC: World Health Organization functional class. ^aClass of recommendation. ^bLevel of evidence. ^cReference(s) supporting recommendations. ^dOnly in responders to acute vasoreactivity tests = class I, for idiopathic PAH, heritable PAH and PAH due to drugs; class IIa, for conditions associated with PAH. ^eTime to clinical worsening as primary endpoint in RCTs or drugs with demonstrated reduction in all-cause mortality. ^fIn patients not tolerating the subcutaneous form. ^gThis drug is not approved by the EMA at the time of publication of these guidelines.