TableĀ 21

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.