Recommendations for efficacy of drug monotherapy for pulmonary arterial hypertension (group 1) according to World Health Organization functional class. The sequence is by pharmacological group, by rating and by alphabetical order

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  • 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.