Ambrisentan response in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) - A subgroup analysis of the ARIES-E clinical trial.

•Long-term ambrisentan therapy resulted in survival and clinical worsening benefits.•6MWD, BDI, and WHO FC changes support the survival and clinical worsening benefits.•Baseline WHO FC and 6MWD hold prognostic relevance for this population.•An absolute 6MWD of 222 m is associated with survival.•Ambrisentan demonstrates long-term safety and efficacy in treating CTD-PAH patients.