Endothelin-receptor antagonists in the management of pulmonary arterial hypertension: where do we stand?

Abstract

Michele Correale,1 Armando Ferraretti,2 Ilenia Monaco,3 Davide Grazioli,1 Matteo Di Biase,4 Natale Daniele Brunetti3 1Department of Cardiology, Ospedali Riuniti University Hospital, Foggia, 2Cardiology Unit, “Caduti in guerra” Hospital, Canosa di Puglia, BT, 3Department of Cardiology, University of Foggia, 4Santa Maria Hospital, GVM Care and Research, Bari, Italy Abstract: Pulmonary arterial hypertension, a disease largely neglected until a few decades ago, is presently the object of intense studies by several research teams. Despite considerable progress, pulmonary arterial hypertension remains a major clinical problem, because it is not always easy to diagnose, treat, and prevent. The disease was considered incurable until the late 1990s, when Epoprostenol was introduced as the first tool against this illness. More recently, therapy for pulmonary arterial hypertension gained momentum after publication of the SERAPHIN and AMBITION trials, which also highlighted the importance of upfront therapy. This review also focuses on recent substudies from these trials and progress in drugs targeting the endothelin pathway. Future perspectives with regard to endothelin-receptor antagonists are also discussed. Keywords: endothelin-receptor antagonists, pulmonary arterial hypertension, Bosentan, ambrisentan, sitaxentan, macitentan

Keywords

• Endothelin Receptor Antagonists
• Pulmonary Arterial Hypertension
• Bosentan
• Ambrisentan
• Sitaxetan
• Macitentan.