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

Authors Correale M, Ferraretti A, Monaco I, Grazioli D, Di Biase M, Brunetti ND

Received 26 October 2017

Accepted for publication 23 February 2018

Published 4 October 2018 Volume 2018:14 Pages 253—264

DOI https://doi.org/10.2147/VHRM.S133921

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 2

Editor who approved publication: Dr Takashi Kajiya


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

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