title: Pulmonary arterial hypertension: the clinical syndrome.
abstract: Pulmonary arterial hypertension is a progressive disorder in which endothelial dysfunction and vascular remodeling obstruct small pulmonary arteries, resulting in increased pulmonary vascular resistance and pulmonary pressures. This leads to reduced cardiac output, right heart failure, and ultimately death. In this review, we attempt to answer some important questions commonly asked by patients diagnosed with pulmonary arterial hypertension pertaining to the disease, and aim to provide an explanation in terms of classification, diagnosis, pathophysiology, genetic causes, demographics, and prognostic factors. Furthermore, important molecular pathways that are central to the pathogenesis of pulmonary arterial hypertension are reviewed, including nitric oxide, prostacyclin, endothelin-1, reactive oxygen species, and endothelial and smooth muscle proliferation.
authors.affiliation: From Vascular Medicine Institute (Y.-C.L., K.C.P., A.L.M., M.T.G.) and Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine (A.L.M., M.T.G.), University of Pittsburgh, PA.
authors.forename: Yen-Chun
authors.initials: YC
authors.lastname: Lai
authors.affiliation: From Vascular Medicine Institute (Y.-C.L., K.C.P., A.L.M., M.T.G.) and Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine (A.L.M., M.T.G.), University of Pittsburgh, PA.
authors.forename: Karin C
authors.initials: KC
authors.lastname: Potoka
authors.affiliation: From Vascular Medicine Institute (Y.-C.L., K.C.P., A.L.M., M.T.G.) and Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine (A.L.M., M.T.G.), University of Pittsburgh, PA.
authors.forename: Hunter C
authors.initials: HC
authors.lastname: Champion
authors.affiliation: From Vascular Medicine Institute (Y.-C.L., K.C.P., A.L.M., M.T.G.) and Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine (A.L.M., M.T.G.), University of Pittsburgh, PA.
authors.forename: Ana L
authors.initials: AL
authors.lastname: Mora
authors.affiliation: From Vascular Medicine Institute (Y.-C.L., K.C.P., A.L.M., M.T.G.) and Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine (A.L.M., M.T.G.), University of Pittsburgh, PA. gladwinmt@upmc.edu.
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
mesh_terms: D052006:Bone Morphogenetic Protein Receptors, Type II / Q000235:genetics / Q000502:physiology; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000235:genetics / Q000401:mortality / Q000503:physiopathology; D008297:Male; D009154:Mutation; D049268:Positron-Emission Tomography; D011379:Prognosis; D012737:Sex Factors; D015398:Signal Transduction
chemical_list: D052006:Bone Morphogenetic Protein Receptors, Type II
keywords: hemodynamics; hypertension, pulmonary; nitric oxide