Correlation of Pulmonary Vascular Dilatation on HRCT to Expression of eNOS in a Rabbit Model of Hepatopulmonary Syndrome

Hepatopulmonary syndrome (HPS) is characterised by the development of intrapulmonary arteriovenous blood shunts and vascular dilatation with consequent hypoxaemia, usually in the context of end-stage liver disease (ESLD). The estimated incidence of HPS in ESLD has been reported to be 13%–47%. Chronic liver disease has been described in patients with hypothalamic–pituitary dysfunction, mainly in the form of non-alcoholic fatty liver disease due to metabolic syndrome, with occasional progression to cirrhosis. We report a challenging case of a 27-year-old man with a background of hypopituitarism with no known liver disease who presented with progressive dyspnoea and hypoxaemia and was eventually diagnosed with severe HPS.

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Hepatopulmonary syndrome: an update

Sao Paulo Medical Journal ◽

10.1590/s1516-31802009000400008 ◽

2009 ◽

Vol 127 (4) ◽

pp. 223-230 ◽

Cited By ~ 10

Author(s):

Liana Gonçalves de Macêdo ◽

Edmundo Pessoa de Almeida Lopes

Keyword(s):

Liver Transplantation ◽

Contrast Echocardiography ◽

Hepatopulmonary Syndrome ◽

Cochrane Library ◽

Microvascular Remodeling ◽

Vascular Dilatation ◽

Cirrhotic Patients ◽

Transplant Candidates ◽

Liver Transplant Waiting List ◽

Worse Prognosis

Hepatopulmonary syndrome (HPS) is a clinical threesome composed of liver disease, intrapulmonary vascular dilatation (IPVD) and arterial gas abnormalities. Its occurrence has been described in up to 32% of cirrhotic candidates for liver transplantation. It also affects non-cirrhotic patients with portal hypertension. Its pathogenesis is not well defined, but an association of factors such as imbalance in the endothelin receptor response, pulmonary microvascular remodeling and genetic predisposition is thought to lead to IPVD. Diagnosis is based on imaging methods that identify these dilatations, such as contrast echocardiography or perfusion scintigraphy with 99mTc, as well as analysis of arterial gases to identify elevated alveolar-arterial differences in O2 or hypoxemia. There is no effective pharmacological treatment and complete resolution only occurs through liver transplantation. The importance of diagnosing HPS lies in prioritizing transplant candidates, since presence of HPS is associated with worse prognosis. The aim of this paper was to review the pathogenetic theories and current diagnostic criteria regarding HPS, and to critically analyze the prioritization of patients with HPS on the liver transplant waiting list. Searches were carried out in the Medline (Medical Literature Analysis and Retrieval System Online) via PubMed, Cochrane Library and Lilacs (Literatura Latino-Americana e do Caribe em Ciências da Saúde) databases for articles published between January 2002 and December 2007 involving adults and written either in English or in Portuguese, using the term hepatopulmonary syndrome. The studies of greatest relevance were included in the review, along with text books and articles cited in references that were obtained through the review.

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Advances in Diagnostic Imaging of Hepatopulmonary Syndrome

Frontiers in Medicine ◽

10.3389/fmed.2021.817758 ◽

2022 ◽

Vol 8 ◽

Author(s):

Bi-Wei Luo ◽

Zhi-Yong Du

Keyword(s):

Liver Disease ◽

Pulmonary Complication ◽

Hepatopulmonary Syndrome ◽

Pulmonary Angiography ◽

Pulmonary Perfusion ◽

Clinical Prognosis ◽

Vascular Dilatation ◽

Progressive Liver Disease ◽

Poor Clinical Prognosis ◽

Timely Treatment

Hepatopulmonary syndrome (HPS) is a serious pulmonary complication of progressive liver disease that leads to a poor clinical prognosis. Patients with HPS may develop acute respiratory failure, which requires intensive care and therapy. At present, the only effective treatment is liver transplantation; therefore, early diagnosis and timely treatment are of considerable significance. The three main features of HPS are liver disease, oxygenation disorder, and intrapulmonary vascular dilatation (IPVD). Diagnosing HPS is challenging due to the difficulty in detecting the presence or absence of IPVD. As such, imaging examination is very important for detecting IPVD. This paper reviews the imaging methods for diagnosing HPS such as ultrasound, dynamic pulmonary perfusion imaging, pulmonary angiography, and computed tomography.

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First presentation of portal hypertension complicated by hepatopulmonary syndrome

BMJ Case Reports ◽

10.1136/bcr-2021-244712 ◽

2021 ◽

Vol 14 (9) ◽

pp. e244712

Author(s):

Nahima Miah ◽

Aidan Ryan ◽

Ceyhun Aksel Oztumer ◽

Mohamed Saleh

Keyword(s):

Portal Hypertension ◽

Unusual Case ◽

Oxygen Demand ◽

Hepatopulmonary Syndrome ◽

Antitrypsin Deficiency ◽

Vascular Dilatation ◽

Alpha 1 Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin ◽

Made In

Hepatopulmonary syndrome (HPS) is a serious complication of chronic liver disease, characterised by portal hypertension and arterial hypoxaemia due to intrapulmonary vascular dilatation. We report an unusual case in which a 27-year-old man had a first presentation of portal hypertension and cirrhosis complicated by HPS. This patient presented with progressive dyspnoea on exertion and deterioration in mobility, with a type 1 respiratory failure and increased oxygen demand. A bubble echocardiogram showed a possible right-to-left shunt, CT aortogram displayed evidence of portal hypertension and cirrhosis, and liver biopsy findings were consistent with alpha-1 antitrypsin deficiency. The patient’s increased oxygen demand was subsequently treated with continuous positive airway pressure before he was discharged with 8 L home oxygen. With no current established medical therapy for HPS, the patient was assessed for liver transplantation and a decision was made in favour of this.

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A RARE CASE OF CYANOSIS AND CLUBBING –LIVER INDUCED VASCULAR DISORDER OF LUNG

Journal of Health and Allied Sciences NU ◽

10.1055/s-0040-1703788 ◽

2014 ◽

Vol 04 (02) ◽

pp. 149-151

Author(s):

Pothukuchi Venkata Krishna ◽

Manasa Manne ◽

Venkata Ravikumar Chepuri

Keyword(s):

Liver Disease ◽

Poor Prognosis ◽

Rare Case ◽

Rare Complication ◽

Hepatopulmonary Syndrome ◽

Liver Disorder ◽

Cyanotic Congenital Heart Disease ◽

Chronic Liver ◽

Vascular Disorder ◽

Vascular Dilatation

Abstract:The hepatopulmonary syndrome (HPS) is under recognized complication of chronic liver disease. Hepatopulmonary syndrome has three components: liver disease, pulmonary vascular dilatation, and a defect in oxygenation. If hypoxemia and dyspnea develop in these patients in the absence of known intrinsic cardiopulmonary disorder, the hepatopulmonary syndrome must be considered. Clinical features include digital clubbing, cyanosis, spider neavi. It is a rare complication of liver disease of varied etiology and indicates a poor prognosis. We are reporting a case of severe clubbing of fingers associated with severe cyanosis and chronic liver disorder in a very young girl to highlight that other causes also should be thought of in addition to cyanotic congenital heart disease in such a young age.

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M2 macrophage accumulation contributes to pulmonary fibrosis, vascular dilatation, and hypoxemia in rat hepatopulmonary syndrome

Journal of Cellular Physiology ◽

10.1002/jcp.30420 ◽

2021 ◽

Author(s):

Bing Chen ◽

Yong Yang ◽

Congwen Yang ◽

Jiaxiang Duan ◽

Lin Chen ◽

...

Keyword(s):

Pulmonary Fibrosis ◽

Hepatopulmonary Syndrome ◽

M2 Macrophage ◽

Vascular Dilatation ◽

Macrophage Accumulation

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Lung capillary changes in hepatic cirrhosis in rats

AJP Lung Cellular and Molecular Physiology ◽

10.1152/ajplung.1997.272.1.l139 ◽

1997 ◽

Vol 272 (1) ◽

pp. L139-L147 ◽

Cited By ~ 6

Author(s):

D. E. Schraufnagel ◽

R. Malik ◽

V. Goel ◽

N. Ohara ◽

S. W. Chang

Keyword(s):

Pulmonary Veins ◽

Pulmonary Arteries ◽

Hepatopulmonary Syndrome ◽

Capillary Density ◽

Systemic Circulation ◽

Vasa Vasorum ◽

Pulmonary Vasculature ◽

Biliary Cirrhosis ◽

Vascular Dilatation ◽

Lung Capillaries

The hypoxemia of the hepatopulmonary syndrome may result from dilated intrapulmonary vascular segments. Knowledge of the size, density, and branching frequency of the lung capillaries might confirm this hypothesis and suggest that the pathogenesis may involve vascular dilatation or angiogenesis. To investigate these changes, the common bile duct of rats was tied off to cause biliary cirrhosis. Later (4 wk), the pulmonary vasculature of these animals was cast, and the casts were studied with scanning electron microscopy. In the ligated animals, evidence for enhancement of the bronchial to pulmonary circulation was found: cast vasa vasorum of the pulmonary arteries and cast bronchial veins emptying into pulmonary veins. Ligated animals had more adherent intracapillary cells per alveolus than the sham-operated animals. The diameters of all capillary beds were larger in the ligated animals. The alveolar capillary density was increased, but the branching frequency was not. A few areas suggesting angiogenesis were found. Induction of biliary cirrhosis enhances the pulmonary-systemic circulation, increases intracapillary adherent cells, capillary diameter, and density, and may be associated with angiogenesis.

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