Michele Correale, Valentina Mercurio, Ester Maria Lucia Bevere, Beatrice Pezzuto, Lucia Tricarico, Umberto Attanasio, Angela Raucci, Anne Lise Ferrara, Stefania Loffredo, Claudio Puteo, Massimo Iacoviello, Maurizio Margaglione, Natale Daniele Brunetti, Carlo G. Tocchetti, Piergiuseppe Agostoni, Claudio Mussolino, Maria Cristina Vinci
Pulmonary arterial hypertension (PAH) is a rare condition characterized by high pulmonary artery pressure leading to right ventricular dysfunction and potential life-threatening consequences. It primarily affects the pre-capillary pulmonary vascular system. The exact pathophysiological mechanisms underlying PAH are not entirely known. Environmental factors; genetic predisposition; mitochondrial and microRNA dysfunction; and inflammatory, metabolic, and hormonal mechanisms may be involved. A central role is played by the dysfunction of the pulmonary vascular endothelium. This alteration is characterized by a reduction in vasodilatory and antiproliferative factors such as prostacyclin and nitric oxide and an increase in vasoconstrictive and mitogenic substances such as endothelin and thromboxane A2. Such imbalance leads to a progressive increase in pulmonary vascular resistance. The aim of the present review is to focus on the vascular endothelium and its role as a potential therapeutic target in PAH.