Endothelin Receptor Antagonists in Pulmonary Arterial Hypertension
Endothelin Receptor Antagonists in Pulmonary Arterial Hypertension
ERAs were the first oral therapy for PAH and remain a critical component of the therapeutic algorithm in the management of the disease. ERAs demonstrated improvements in pulmonary hemodynamics, exercise capacity, functional status, and clinical outcome in several randomized placebo-controlled trials, therefore representing a major therapy in PAH. Current clinical data suggest that both dual and specific ERAs have a similar efficacy in improving clinical outcomes in PAH patients, with differences in safety profiles. Recently, the randomized, doubleblind, multi-center, AMBITION study showed that firstline treatment with ambrisentan/tadalafil combination therapy in naïve PAH patients is superior in terms of the primary endpoint (time to first clinical failure event) compared with monotherapy (ambrisentan or tadalafil). This result promotes arguments to treat de novo PAH patients with a combination therapy in order to improve clinical outcomes in PAH patients where ERAs could take a primary role.
Conclusions and Future Directions
ERAs were the first oral therapy for PAH and remain a critical component of the therapeutic algorithm in the management of the disease. ERAs demonstrated improvements in pulmonary hemodynamics, exercise capacity, functional status, and clinical outcome in several randomized placebo-controlled trials, therefore representing a major therapy in PAH. Current clinical data suggest that both dual and specific ERAs have a similar efficacy in improving clinical outcomes in PAH patients, with differences in safety profiles. Recently, the randomized, doubleblind, multi-center, AMBITION study showed that firstline treatment with ambrisentan/tadalafil combination therapy in naïve PAH patients is superior in terms of the primary endpoint (time to first clinical failure event) compared with monotherapy (ambrisentan or tadalafil). This result promotes arguments to treat de novo PAH patients with a combination therapy in order to improve clinical outcomes in PAH patients where ERAs could take a primary role.