title: Novel Strategies in the Treatment of Pulmonary Arterial Hypertension.
abstract: Pulmonary arterial hypertension (PAH) is a pathophysiological condition characterized by increased pulmonary vascular resistance (PVR), initially due to abnormal pulmonary vasoconstriction in response to endothelial injury. Recent studies confirmed the key role of endothelin (ET)-1 in the vasoconstriction and remodeling of pulmonary microcirculation during PAH. In responders patients, classical treatments for PAH are prostanoids, phosphodiesterase (PDE)-5 inhibitors and endothelin receptor antagonists (ERAs), which target prostaglandin I2, nitric oxide and endothelin pathways, respectively. Randomised, placebo-controlled trials have shown that ERAs improves haemodynamic parameters of the pulmonary circulation, functional capacity and clinical outcome in patients affected by PAH. Here, we will review the definition, classification and pathophysiology of PH. Furthermore, we will provide an up-to-date overview of currently recommended diagnostic and therapeutic work-up in PAH.
authors.affiliation: Institute of Cardiology, Center of Excellence on Aging, "G. d'Annunzio" University, Chieti, Italy. rmadonna@unich.it.
authors.forename: Rosalinda
authors.initials: R
authors.lastname: Madonna
authors.forename: Nino
authors.initials: N
authors.lastname: Cocco
mesh_terms: D000077300:Bosentan; D065128:Endothelin Receptor Antagonists / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy*; D010666:Phenylpropionates / Q000494:pharmacology / Q000627:therapeutic use; D011379:Prognosis; D011652:Pulmonary Circulation / Q000187:drug effects; D011724:Pyridazines / Q000494:pharmacology / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D013449:Sulfonamides / Q000494:pharmacology / Q000627:therapeutic use; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000187:drug effects
chemical_list: D065128:Endothelin Receptor Antagonists; D010666:Phenylpropionates; D011724:Pyridazines; D013449:Sulfonamides; C467894:ambrisentan; D000077300:Bosentan