title: Pathways and Drugs in Pulmonary Arterial Hypertension - Focus on the Role of Endothelin Receptor Antagonists.
abstract: 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.
authors.affiliation: Institute of Cardiology, Center of Excellence on Aging, "G. d'Annunzio" University, Chieti, Italy.
authors.forename: Rosalinda
authors.initials: R
authors.lastname: Madonna
authors.affiliation: Department "Cuore e Grossi Vasi Attilio Reale", "Sapienza" University, Policlinico Umberto I, Rome, Italy.
authors.forename: Nino
authors.initials: N
authors.lastname: Cocco
authors.affiliation: Institute of Cardiology, Center of Excellence on Aging, "G. d'Annunzio" University, Chieti, Italy. rdecater@unich.it.|Institute of Cardiology, "G. d'Annunzio" University - Chieti, C/o Centro Scienze dell'Invecchiamento CESI, Via dei Vestini, 66013, Chieti, Italy. rdecater@unich.it.
authors.forename: Raffaele
authors.initials: R
authors.lastname: De Caterina
mesh_terms: D004347:Drug Interactions; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists / Q000494:pharmacology / Q000627:therapeutic use*; D016232:Endothelins / Q000378:metabolism; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000378:metabolism; D017466:Receptors, Endothelin / Q000378:metabolism*
chemical_list: D065128:Endothelin Receptor Antagonists; D016232:Endothelins; D017466:Receptors, Endothelin
keywords: Calcium channels blockers; Endothelin receptor antagonists; PDE-5 inhibitors; Prostanoids; Pulmonary arterial hypertension