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Portopulmonary Hypertension*: A Tale of Two Circulations

Rohit Budhiraja, MD; Paul M. Hassoun, MD
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*From the Pulmonary and Critical Care Division, Department of Medicine, Tufts-New England Medical Center, Tufts University School of Medicine, Boston, MA.

Correspondence to: Paul M. Hassoun, MD, Associate Professor of Medicine, Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care, 5501 Hopkins Bayview Circle, Baltimore, MD 21224



Chest. 2003;123(2):562-576. doi:10.1378/chest.123.2.562
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Pulmonary involvement is common in patients with portal hypertension and can manifest in diverse manners. Changes in pulmonary arterial resistance, manifesting either as the hepatopulmonary syndrome or portopulmonary hypertension (PPHTN), have been increasingly recognized in these patients in recent years. This review summarizes the clinicopathologic features, diagnostic criteria, as well as the latest concepts in the pathogenesis and management of PPHTN, which is defined as an elevated pulmonary artery pressure in the setting of an increased pulmonary vascular resistance and a normal wedge pressure in a patient with portal hypertension.

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