Hepatopulmonary Syndrome

University of Alabama at Birmingham Liver Center, MCLM 290, 1918, University Boulevard, Birmingham, AL 35294, USA.
Journal of Hepatology (Impact Factor: 10.4). 11/2006; 45(4):617-25. DOI: 10.1016/j.jhep.2006.07.002
Source: PubMed

ABSTRACT The hepatopulmonary syndrome (HPS) is a pulmonary complication of cirrhosis and/or portal hypertension whereby patients develop hypoxemia as a result of alterations in pulmonary microvascular tone and architecture. HPS occurs in up to 30% of patients with cirrhosis. Although the degree of hypoxemia does not reliably correlate with the severity of liver disease, patients with HPS have a higher mortality than do patients with cirrhosis without the disorder. There has been progress into defining the mechanisms that lead to hypoxemia in HPS, but to date there are no therapeutic options for HPS aside from liver transplantation.

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    ABSTRACT: Intrapulmonary vasodilation is a hallmark of the hepatopulmonary syndrome (HPS). However, its effects on respiratory mechanical properties and lung morphology are unknown. To determine these effects, 28 rats were randomly divided to control and experimental HPS groups (eHPS). The spontaneous breathing pattern, gas exchange, respiratory system mechanical properties, and lung and liver morphology of the rats were evaluated. Tidal volume, minute ventilation and mean inspiratory flow were significantly reduced in the eHPS group. Chest wall pressure dissipation against the resistive and viscoelastic components and elastic elastance were increased in the eHPS group. The lung resistive pressure dissipation was lower but the viscoelastic pressure was higher in the eHPS group. The airway volume proportion of collagen and elastic fibers was increased in the eHPS animals (16% and 51.7%; P<0.05 and P<0.001, respectively). The proportion of collagen volume in the vasculature increased 29% in the eHPS animals (P<0.01). HPS presents with respiratory system mechanical disarray as well as airway and vascular remodeling.
    Respiratory Physiology & Neurobiology 12/2011; 179(2-3):326-33. DOI:10.1016/j.resp.2011.10.001 · 1.97 Impact Factor
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    ABSTRACT: Advanced portal hypertension accompanying end-stage liver disease results in an altered milieu due to inadequate detoxification of blood from splanchnic circulation by the failing liver. The portosystemic shunts with hepatic dysfunction result in an increased absorption and impaired neutralisation of the gastrointestinal bacteria and endotoxins leads to altered homeostasis with multiorgan dysfunction. The important cardiopulmonary complications are cirrhotic cardiomyopathy, hepatopulmonary syndrome, portopulmonary hypertension, and right-sided hydrothorax.
    01/2011; 2011:280569. DOI:10.4061/2011/280569
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    ABSTRACT: Background/AimHepatopulmonary syndrome in children has not been extensively studied particularly in different subsets of portal hypertension. We prospectively studied hepatopulmonary syndrome in children with cirrhosis and extrahepatic portal venous obstruction. Their comparison might shed light on the pathogenesis of hepatopulmonary syndrome.Methods Between 1 to 18 years of age, 135 children with portal hypertension (cirrhosis 35, extrahepatic portal venous obstruction 100) were studied. Hepatopulmonary syndrome was diagnosed by the presence intrapulmonary shunt using transthoracic contrast echocardiography and hypoxia /orthodeoxia (in arterial blood gas examination). Comparative analyses were done for both the groups, and also intra and inter groups with and without hepatopulmonary syndrome.ResultOf 135 children, hepatopulmonary syndrome occurred more frequently in cirrhosis (40%) than extrahepatic portal venous obstruction (13%) [p=0.01]. Cirrhotics with hepatopulmonary syndrome were symptomatic (85% vs. 14%, p <0.001) and had longer disease duration [16(1-120) months vs. 28 (1-168) months, p=0.01] as compared to cirrhotics without hepatopulmonary syndrome. Prevalence of hepatopulmonary syndrome among cirrhotics did not correlate with the severity of liver disease as assessed by both pediatric end-stage liver disease score and Child- Turcotte-Pugh score. Patients with extrahepatic portal venous obstruction and hepatopulmonary syndrome remain asymptomatic manifesting with clubbing alone (38%).Conclusions Hepatopulmonary syndrome occurs more frequently in cirrhosis, a majority being symptomatic with more severity in comparison to extrahepatic portal venous obstruction having no symptoms and less severity. There seems to be a role of liver dysfunction besides portal hypertension as an added factor in the pathogenesis of hepatopulmonary syndrome.This article is protected by copyright. All rights reserved.
    Liver international: official journal of the International Association for the Study of the Liver 10/2014; 35(6). DOI:10.1111/liv.12708 · 4.41 Impact Factor