Objectives: To assess whether switching patients with suboptimally controlled pulmonary arterial hypertension from bosentan or ambrisentan to macitentan would improve six-minute walk test (6MWT) distance and World Health Organization functional class.

Methods: This was a retrospective cohort analysis of 37 patients from a single center. Patients were separated into three heterogeneous treatment groups and followed for 18 months: switch group (n¼14): patients switched to macitentan from bosentan/ambrisentan; added group (n¼11): patients who began macitentan as de novo therapy (n¼5) or who added macitentan to an existing sildenafil regimen (n¼6); and control group (n¼12): patients for whom sildenafil and/or bosentan/ambrisentan therapy was unchanged.

Results: Mortality was observed in two patients (one each, switch and added groups). Patients in the control group had one hospital admission and 100% survival. There was significant improvement in functional class for the switch and added groups. Statistically significant improvement was observed in 6MWT distance in the added group alone. Overall, 92% of patients continued macitentan throughout the study.

Conclusion: Macitentan was well tolerated. For bosentan/ambrisentan-treated patients with suboptimally controlled pulmonary arterial hypertension, switching to macitentan may facilitate an improvement in functional class.


pulmonary arterial hypertension, World Health Organization functional class, echocardiography, six-minute walk test, macitentan, endothelin-I receptor antagonist

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