Hepatopulmonary Syndrome and Portopulmonary Hypertension: Current Status and Implications for Liver Transplantation

被引：15
作者：
Kelley Weinfurtner
Kimberly Forde
机构：
[1] Division of Gastroenterology and Hepatology, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA
[2] Center for Clinical Epidemiology and Biostatistics, Department of Biostatistics, Epidemiology, and Informatics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA
来源：
Current Hepatology Reports | 2020年 / 19卷 / 3期
关键词：
Arterial hypoxemia with elevated A-a gradient; Hepatopulmonary syndrome; Intravascular pulmonary dilation; Liver transplant evaluation; Portopulmonary hypertension; Pulmonary hypertension medical therapy;
D O I：
10.1007/s11901-020-00532-y
中图分类号：
学科分类号：
摘要：
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. © Springer Science+Business Media, LLC, part of Springer Nature 2020.
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页码：174 / 185
页数：11
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