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Pulmonary Arterial Hypertension
Author(s) -
YenChun Lai,
Karin C. Potoka,
Hunter C. Champion,
Ana L. Mora,
Mark T. Gladwin
Publication year - 2014
Publication title -
circulation research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.899
H-Index - 336
eISSN - 1524-4571
pISSN - 0009-7330
DOI - 10.1161/circresaha.115.301146
Subject(s) - pulmonary hypertension , medicine , prostacyclin , cardiology , pathophysiology of hypertension , vascular resistance , pathophysiology , endothelial dysfunction , nitric oxide , pathogenesis , heart failure , pulmonary artery , pulmonary vasculature , hemodynamics , blood pressure
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.

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