Pulmonary arterial hypertension: the clinical syndrome

YC Lai, KC Potoka, HC Champion, AL Mora… - Circulation …, 2014 - Am Heart Assoc
YC Lai, KC Potoka, HC Champion, AL Mora, MT Gladwin
Circulation research, 2014Am Heart Assoc
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 …
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.
Am Heart Assoc