Pulmonary Arterial Hypertension: The Clinical Syndrome

• Published in: Circulation research Vol. 115; no. 1; pp. 115 - 130
• Main Authors: Lai, Yen-Chun, Potoka, Karin C, Champion, Hunter C, Mora, Ana L, Gladwin, Mark T
• Format: Journal Article
• Language: English
• Published: United States American Heart Association, Inc 20.06.2014
• Subjects: Bone Morphogenetic Protein Receptors, Type II - genetics; Bone Morphogenetic Protein Receptors, Type II - physiology; Familial Primary Pulmonary Hypertension; Female; Hemodynamics; Humans; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - genetics; Hypertension, Pulmonary - mortality; Hypertension, Pulmonary - physiopathology; Male; Mutation; Positron-Emission Tomography; Prognosis; Sex Factors; Signal Transduction; hypertension, pulmonary; nitric oxide; hemodynamics
• ISSN: 0009-7330; 1524-4571
• DOI: 10.1161/CIRCRESAHA.115.301146

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.