The 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: The hepatopulmonary syndrome has been acknowledged as an important vascular complication in lungs developing systemic hypoxemia in patients with cirrhosis and portal hypertension. Is formed by arterial oxygenation abnormalities induced from intrapulmonary vascular dilatations with liver disease. It is present in 4-32% of patients with cirrhosis. It increases mortality in the setting of cirrhosis and may influence the frequency and severity. Initially the hypoxemia responds to low-flow supplemental oxygen, but over time, the need for oxygen supplementation is necessary. The liver transplantation is the only effective therapeutic option for its resolution.
    09/2014; 27(2):145-147.
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    ABSTRACT: Introduction End-stage liver disease and its complications are a leading cause of death among adults. The liver plays a central role in health and homeostasis and thus the diseased liver leads to many deleterious effects on multiple organ systems, including the pulmonary system Julie et al. (2007) [1]. A variety of causes for pulmonary dysfunction in liver disease have been identified and include intrinsic cardiopulmonary disorders not specifically related to liver disease as well as unique problems associated with the presence of liver disease and/or portal hypertension Michael et al. (2000) [2]. Liver disease and portal hypertension can be associated with pulmonary vascular complications, including portopulmonary hypertension (POPH), and hepatopulmonary syndrome (HPS) Mateo et al. (2012) [3]. Aim This study aimed to evaluate the pulmonary dysfunctions complicating liver cirrhosis. Patients and methods Fifty patients with liver cirrhosis without intrinsic cardiopulmonary disease were enrolled in this study. All were subjected to complete clinical examination, laboratory investigations, radiological investigations including abdominal ultrasound, chest x ray and CT chest, ECG, contrast enhanced echocardiography with colored Doppler study, gastroscopy, ventilatory function tests, measurement of SaO2 using portable pulse oximetry and arterial blood gas analysis. Results The prevalence of arterial hypoxemia in cirrhotic patients was 14.6%. The presence of hypoxemia is increased in patients with advanced liver disease and the severity of hypoxemia was positively correlated with the severity of liver disease assessed by the Child Pugh score. HPS represents 64.1% of causes of hypoxemia. Pulse oximetry is a simple non-invasive method for detection of arterial hypoxemia as an initial screening test for HPS. Contrast enhanced echocardiography (CEE) is the gold standard method for the diagnosis of HPS by detection of intrapulmonary vasodilatation (IPV) characteristic of HPS, while CT chest assists in diagnosis by exclusion of intrinsic pulmonary disease. Conclusion Liver cirrhosis is associated with unique pulmonary complications. The early identification of pulmonary dysfunctions in cirrhotic patients is crucial as it affects the prognosis and guides the future management by speeding up orthotopic liver transplantation (OLT) recommendations.
    Egyptian Journal of Chest Diseases and Tuberculosis. 08/2014;
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    ABSTRACT: To evaluate surfactant protein A levels in an hepatopulmonary syndrome rat model. To date, there have been no studies aimed at evaluating surfactant levels in the setting of cirrhosis or hepatopulmonary syndrome.
    Acta cirurgica brasileira / Sociedade Brasileira para Desenvolvimento Pesquisa em Cirurgia 09/2014; 29(9):573-578. · 0.48 Impact Factor