Circulation. 2006;114:1417-1431
doi: 10.1161/CIRCULATIONAHA.104.503540
doi: 10.1161/CIRCULATIONAHA.104.503540
(Circulation. 2006;114:1417-1431.)
© 2006 American Heart Association, Inc.
Contemporary Reviews in Cardiovascular Medicine |
Pulmonary Arterial Hypertension
From The University of Michigan Health System (V.V.M.), Ann Arbor, Mich, and Mayo Clinic (M.D.M.), Rochester, Minn.
Correspondence to Vallerie V. McLaughlin, MD, Associate Professor of Medicine, Director, Pulmonary Hypertension Program, University of Michigan, 1500 E Medical Center Dr, Women’s Hospital–Room L3119, Ann Arbor, MI 48109-0273. E-mail vmclaugh@umich.edu
Key Words: endothelin • hemodynamics • hypertension, pulmonary • prostaglandins • pulmonary heart disease
An extract of the first 250 words of the full text is provided, because this article has no abstract. |
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Introduction |
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Most practicing cardiologists see patients with pulmonary hypertension (PH) on a regular basis. Although this is most commonly in the form of pulmonary venous hypertension related to elevated left heart pressures, the explosion in knowledge of and treatment for pulmonary arterial hypertension (PAH) over the past decade obligates cardiologists to be more cognizant of this disorder. In this review, we will discuss the pathobiology, classification, diagnosis, and treatment of PAH in adults. The term PAH in this context refers specifically to a pulmonary hypertensive state limited predominantly to the arterial component of the pulmonary vasculature and constitutes one of the main classifications of clinical PH diseases.
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Pathobiological Changes in PH |
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The normal pulmonary vasculature is a low-pressure system with less than one tenth the resistance to flow observed in the systemic vascular bed. The mechanistic and structural alterations underlying the development and progression of pulmonary vasculopathy have become increasingly well characterized. Some or all of these perturbations may play a role in all forms of PAH, but some molecular and cellular substrates, and the histopathologic consequences, have been associated with particular clinical types of PH.
PH refers to the hemodynamic state in which the pressure measured in the pulmonary artery is elevated. By expert consensus, PAH is regarded as a mean pulmonary artery pressure (mPAP) greater than 25 mm Hg in the setting of normal or reduced cardiac output and a normal pulmonary capillary wedge pressure.1 The evolution of pulmonary vascular disease frequently originates with the interaction of a predisposing state and 1 or
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