caa a22 4500 606158626 CHVBK 20210128100620.0 cr unu---uuuuu 210128e20151001xx s 000 0 eng 10.1007/s10557-015-6605-6 doi (NATIONALLICENCE)springer-10.1007/s10557-015-6605-6 Pathways and Drugs in Pulmonary Arterial Hypertension - Focus on the Role of Endothelin Receptor Antagonists [Elektronische Daten] [Rosalinda Madonna, Nino Cocco, Raffaele De Caterina] Pulmonary arterial hypertension (PAH) is a group of diseases characterized by a progressive increase of pulmonary vascular resistance (PVR), initially due to abnormal pulmonary vasoconstriction in response to endothelial injury. Recent studies have here confirmed the prominent role of endothelin (ET)-1 in vasoconstriction and remodelling of the pulmonary microcirculation. In patients who are acute-vasoreactive, classical treatments for PAH are calcium channels blockers (CCBs), while drugs targeting the prostacyclin, nitric oxide and endothelin pathways, i.e., prostanoids, phosphodiesterase (PDE)-5 inhibitors and endothelin receptor antagonists (ERAs), respectively, are indicated in non-vasoreactive patients or in vasoreactive patients not responding to initial CCB therapy. Randomised, placebo-controlled trials have shown that ERAs improve pulmonary haemodynamics, exercise capacity, functional status and clinical outcome in patients with PAH. Here we provide an overview of the currently recommended diagnostic and therapeutic work-up in PAH, with special emphasis on ERAs. Springer Science+Business Media New York, 2015 Pulmonary arterial hypertension nationallicence Endothelin receptor antagonists nationallicence PDE-5 inhibitors nationallicence Prostanoids nationallicence Calcium channels blockers nationallicence Madonna Rosalinda Institute of Cardiology, Center of Excellence on Aging, "G. d'Annunzio” University, Chieti, Italy aut Cocco Nino Department "Cuore e Grossi Vasi Attilio Reale”, "Sapienza” University, Policlinico Umberto I, Rome, Italy aut De Caterina Raffaele Institute of Cardiology, Center of Excellence on Aging, "G. d'Annunzio” University, Chieti, Italy aut Cardiovascular Drugs and Therapy Springer US; http://www.springer-ny.com 29/5(2015-10-01), 469-479 0920-3206 29:5<469 2015 29 10557 https://doi.org/10.1007/s10557-015-6605-6 text/html Onlinezugriff via DOI BK010053 XK010053 XK010000 Metadata rights reserved Springer special CC-BY-NC licence nationallicence 1 research-article jats NATIONALLICENCE NATIONALLICENCE NL-springer NATIONALLICENCE 856 40 https://doi.org/10.1007/s10557-015-6605-6 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Madonna Rosalinda Institute of Cardiology, Center of Excellence on Aging, "G. d'Annunzio” University, Chieti, Italy aut NATIONALLICENCE 700 1- Cocco Nino Department "Cuore e Grossi Vasi Attilio Reale”, "Sapienza” University, Policlinico Umberto I, Rome, Italy aut NATIONALLICENCE 700 1- De Caterina Raffaele Institute of Cardiology, Center of Excellence on Aging, "G. d'Annunzio” University, Chieti, Italy aut NATIONALLICENCE 773 0- Cardiovascular Drugs and Therapy Springer US; http://www.springer-ny.com 29/5(2015-10-01), 469-479 0920-3206 29:5<469 2015 29 10557