Hepatopulmonary Syndrome and Portopulmonary Hypertension: Current Status and Implications for Liver Transplantation
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MANAGEMENT OF THE CIRRHOTIC PATIENT (A CARDENAS AND P TANDON, SECTION EDITORS)
Hepatopulmonary Syndrome and Portopulmonary Hypertension: Current Status and Implications for Liver Transplantation Kelley Weinfurtner 1
&
Kimberly Forde 1,2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are both pulmonary vascular complications of advanced liver disease; however, these syndromes have distinct pathophysiology, clinical implications, and management. Recent Findings While both conditions are associated with portal hypertension, HPS results from diffuse pulmonary capillary vasodilation and PoPH results from vasoconstriction and vascular remodeling of pulmonary arteries. In HPS, no medical therapies clearly improve outcomes; however, patients have excellent post-LT outcomes with near uniform reversal of hypoxemia. In PoPH, several medical therapies used in idiopathic pulmonary hypertension have been shown improve pulmonary hemodynamics, symptoms, and potentially LT outcomes; however, further study is needed to determine best treatment regimens, long-term outcomes on medical therapy, and role of LT. Summary While HPS results in severe hypoxemia that is usually reversible by LT, PoPH patients develop progressive pulmonary hypertension that may improve with medical therapy. Keywords Hepatopulmonary syndrome . Portopulmonary hypertension . Liver transplant evaluation . Intravascular pulmonary dilation . Arterial hypoxemia with elevated A-a gradient . Pulmonary hypertension medical therapy
Introduction Over the last three decades, there has been increased recognition of two important pulmonary vascular complications of liver disease, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) (1). While there is a growing literature regarding the impact of these complications on survival and post-liver transplant (LT) outcomes, the
pathophysiology and optimal therapeutic strategies are still not well characterized. It is, however, clear that HPS and PoPH are distinct syndromes with differing pathophysiology and clinical implications. This review summarizes our current understanding of these conditions and highlights areas in need of further study.
Hepatopulmonary Syndrome Definition and Pathophysiology This article is part of the Topical Collection on Management of the Cirrhotic Patient * Kelley Weinfurtner [email protected] 1
Division of Gastroenterology and Hepatology, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
2
Center for Clinical Epidemiology and Biostatistics, Department of Biostatistics, Epidemiology, and Informatics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
HPS is defined as an arterial oxygenation defect secondary to intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease. It occurs most commonly in patients with cirrhosis and portal
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