Recommendations for efficacy of sequential drug combination therapy for pulmonary arterial hypertension (group 1) according to World Health Organization functional class. Sequence is by rating and by alphabetical order

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  • 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.