scholarly journals The hepatopulmonary syndrome

2014 ◽  
Vol 27 (2) ◽  
pp. 145-147 ◽  
Author(s):  
Lucas Souto NACIF ◽  
Wellington ANDRAUS ◽  
Rafael Soares PINHEIRO ◽  
Liliana DUCATTI ◽  
Luciana BP HADDAD ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Clinical Manifestation ◽  
Therapeutic Option ◽  
Hepatopulmonary Syndrome ◽  
Supplemental Oxygen ◽  
Vascular Complication ◽  
Low Flow ◽  
Arterial Oxygen ◽  
Effective Therapeutic Option

INTRODUCTION: 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. AIM: To update clinical manifestation, diagnosis and treatment of this entity. METHOD: A literature review was performed on management of hepatopulmonary syndrome. The electronic search was held of the Medline-PubMed, in English crossing the headings "hepatopulmonary syndrome", "liver transplantation" and "surgery". The search was completed in September 2013. RESULTS: Hepatopulmonary syndrome is classically defined by a widened alveolar-arterial oxygen gradient (AaPO2) on room air (>15 mmHg, or >20 mmHg in patients >64 years of age) with or without hypoxemia resulting from intrapulmonary vasodilatation in the presence of hepatic dysfunction or portal hypertension. Clinical manifestation, diagnosis, classification, treatments and outcomes are varied. CONCLUSION: The severity of hepatopulmonary syndrome is an important survival predictor and determine the improvement, the time and risks for liver transplantation. The liver transplantation still remains the only effective therapeutic.

scholarly journals Hepatopulmonary Syndrome. Review

2021 ◽  
Vol 6 (3) ◽  
pp. 45-52
Author(s):  
V. V. Potii ◽  
◽  
V. T. Kiriienko ◽  
E. I. Glukhova ◽  
O. S. Kunickaya ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Gas Analysis ◽  
Arterial Oxygen ◽  
Common Symptom ◽  
Portopulmonary Hypertension ◽  
Oxygen Gradient ◽  
Arterial Blood ◽  
Intrapulmonary Shunting

Liver cirrhosis is often accompanied by complications from the pulmonary system. These include hydrothorax, portopulmonary hypertension and hepatopulmonary syndrome. Hepatic hydrothorax affects about 6-10% of patients with end-stage disease, which results in the passage of ascetic fluid into the pleural space through diaphragm defects. The common cause of the hepatopulmonary syndrome and portopulmonary hypertension is portal hypertension and portosystemic shunting, indicating that vasoactive and angiogenetic factors originating from the liver normally control the pulmonary circulation. Portopulmonary hypertension is like pulmonary arterial hypertension, which develops against the background of portal hypertension as a result of chronic liver disease or without other causes of increased pressure in the pulmonary vessels. The prevalence of portopulmonary hypertension ranges from 2% to 8.5% among patients with portal hypertension and is associated with a poor prognosis. Hepatopulmonary syndrome is characterized by intrapulmonary dilatation of microvessels, which causes intrapulmonary shunting and leads to impaired gas exchange in liver diseases, and is associated with a decrease in the quality and duration of life in patients with cirrhosis. Nitric oxide overproduction and angiogenesis seem to be the hallmarks of a complicated pathogenetic mechanism, leading to intrapulmonary shunting and ventilation-perfusion mismatch. A classification of hepatopulmonary syndrome according to the severity of hypoxemia has been suggested. Hepatopulmonary syndrome includes a triad: hepatic dysfunction and / or portal hypertension, dilatation of intrapulmonary vessels, and increased alveolar-arterial oxygen gradient. The prevalence of hepatopulmonary syndrome varies depending on the study groups from 5% to 30%. The most common symptom of the complication is shortness of breath, but in most cases, hepatopulmonary syndrome is asymptomatic. A decrease in oxygen saturation less than 96% corresponds to a decrease in PaO2<70 mm Hg and testifies to the possible development of hepatopulmonary syndrome. In the case of a positive screening, the patient should undergo arterial blood gas analysis, which helps to determine PaO2 and alveolar to arterial oxygen gradient. Conclusion. Contrast-enhanced echocardiography with agitated saline is the gold standard in the diagnosis of intrapulmonary dilatation. The only effective treatment for hepatopulmonary syndrome is liver transplantation. Complete recovery of hepatopulmonary syndrome after liver transplantation is observed within a year in most patients with cirrhosis and hepatopulmonary syndrome

Impact of Hepatopulmonary Syndrome in Liver Transplantation Candidates and the Role of Angiogenesis

2021 ◽  
pp. 2102304
Author(s):  
Steven M. Kawut ◽  
Michael J. Krowka ◽  
Kimberly A. Forde ◽  
Nadine Al-Naamani ◽  
Karen L. Krok ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Vascular Complications ◽  
Hepatopulmonary Syndrome ◽  
Functional Class ◽  
Hazard Ratio ◽  
Arterial Oxygen ◽  
Risk Of Death ◽  
Increased Risk

Hepatopulmonary syndrome affects 10–30% of patients with cirrhosis and portal hypertension. We evaluated the serum angiogenic profile of hepatopulmonary syndrome and assessed the clinical impact of hepatopulmonary syndrome in patients evaluated for liver transplantation.The Pulmonary Vascular Complications of Liver Disease 2 study was a multicentre, prospective cohort study of adults undergoing their first liver transplantation evaluation. Hepatopulmonary syndrome was defined as an alveolar-arterial oxygen gradient ≥15 mmHg (≥20 mmHg if age >64 years), positive contrast-enhanced transthoracic echocardiography, and absence of lung disease.We included 85 patients with hepatopulmonary syndrome and 146 patients without hepatopulmonary syndrome. Patients with hepatopulmonary syndrome had more complications of portal hypertension and slightly higher Model for End-stage Liver Disease-Na score compared to those without hepatopulmonary syndrome (median [interquartile range] 15 [12, 19] versus 14 [10, 17], p=0.006). Hepatopulmonary syndrome patients had significantly lower six minute walk distance and worse functional class. Hepatopulmonary syndrome patients had higher circulating angiopoietin-2, Tie2, tenascin-C, c-kit, VCAM-1, and von Willebrand factor levels, and lower E-selectin levels. Patients with hepatopulmonary syndrome had an increased risk of death (hazard ratio 1.80 [1.03–3.16], p=0.04) which persisted despite adjustment for covariates (hazard ratio 1.79 [1.02–3.15], p=0.04). This association did not vary based on levels of oxygenation reflecting the severity of hepatopulmonary syndrome.Hepatopulmonary syndrome was associated with a profile of abnormal systemic angiogenesis, worse exercise and functional capacity, and an overall increased risk of death.

The Need for Consensus About Liver Transplantation For Patients With Neuropsychiatric Wilson’s Disease

2021 ◽  
pp. 152692482110028
Author(s):  
Alberto Ferrarese ◽  
Patrizia Burra
Keyword(s):  
Liver Transplantation ◽  
Patient Outcomes ◽  
Wilson’S Disease ◽  
Therapeutic Option ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Published Data ◽  
Neurological Improvement ◽  
Effective Therapeutic Option

Liver transplantation is considered an effective therapeutic option for Wilson’s disease (WD) patients with hepatic phenotype, since it removes the inherited defects of copper metabolism, and is associated with excellent graft and patient outcomes. The role of liver transplantation in WD patients with mixed hepatic and neuropsychiatric phenotype has remained controversial over time, mainly because of high post-operative complications, reduced survival and a variable, unpredictable rate of neurological improvement. This article critically discusses the recently published data in this field, focussing in more detail on isolated neuropsychiatric phenotype as a potential indication for liver transplantation in WD patients.

scholarly journals Liver cirrhosis and hepatic stellate cells

2006 ◽  
Vol 21 (suppl 1) ◽  
pp. 54-57 ◽  
Author(s):  
Daniel Ferracioli Brandão ◽  
Leandra Naira Zambelli Ramalho ◽  
Fernando Silva Ramalho ◽  
Sérgio Zucoloto ◽  
Ana de Lourdes Candolo Martinelli ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatic Stellate Cells ◽  
Therapeutic Option ◽  
Stellate Cells ◽  
Terminal Phase ◽  
Activation Process ◽  
Bioactive Substances ◽  
Hepatic Diseases ◽  
Cytoplasmic Processes

The cirrhosis represents the final stage of several chronic hepatic diseases and it is characterized by the presence of fibrosis and morphologic conversion from the normal hepatic architecture into structurally abnormal nodules. In the evolution of the disease there is loss of the normal vascular relationship and portal hypertension. There are also regenerative hepatocelular alterations that become more prominent with the progression of the disease. The liver transplantation continues to be the only therapeutic option in cases of disease in terminal phase. The hepatic stellate cells (HSC) are perisinusoidal cells that store vitamin A and produce growth factors, citocins, prostaglandins and other bioactive substances. They can suffer an activation process that convert them to cells with a phenotype similar to myofibroblasts. When activated, they present increased capacity of proliferation, mobility, contractility and synthesis of collagen and other components of extracelular matrix. They possess cytoplasmic processes adhered to sinusoids and can affect the sinusoidal blood flow. HSC are important in pathogenesis of fibrosis and portal hypertension.

Liver Transplantation for Hepatopulmonary Syndrome Due to Noncirrhotic Portal Hypertension

2011 ◽  
Vol 43 (7) ◽  
pp. 2814-2816 ◽  
Author(s):  
K. Maganty ◽  
R. Ghanta ◽  
P. Bejarano ◽  
D. Weppler ◽  
A. Tekin ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Noncirrhotic Portal Hypertension

Portopulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2534-2537
Author(s):  
Olivier Sitbon ◽  
Laurent Savale
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Channel Blockers ◽  
Portopulmonary Hypertension ◽  
Right Heart ◽  
Pulmonary Haemodynamics

Portal hypertension, with or without liver disease, may have major consequences on pulmonary circulation due to complex pathophysiological interactions between the liver and the lungs. There are two distinct pulmonary vascular disorders associated with portal hypertension: hepatopulmonary syndrome, characterized by gas exchange impairment due to intrapulmonary shunts, and portopulmonary hypertension (PoPH), which is a particular form of pulmonary arterial hypertension (PAH). PoPH is a severe complication of portal hypertension, affecting functional status, exercise capacity, and survival of affected patients. Detection of PoPH by transthoracic echocardiography must be performed in symptomatic patients and in all candidates for liver transplantation. Right heart catheterization is mandatory to confirm the diagnosis of PoPH and exclude other causes of pulmonary artery pressure elevation, including fluid overload and high cardiac output, that are common features in portal hypertension. The management of PoPH is similar to that of other forms of PAH although very few trials have investigated the effects of PAH-targeted therapies in this particular indication. Anticoagulants and calcium channel blockers are usually not indicated in this setting. PAH-targeted medications can be used to improve pulmonary haemodynamics and bridge patients with severe PoPH to liver transplantation. However, the impact of liver transplantation on outcomes of patients with PoPH remains unpredictable. While uncontrolled PoPH is associated with a higher risk of perioperative right heart failure and death, stabilization, improvement, or normalization of pulmonary haemodynamics after liver transplantation seem to be achievable goals in selected patients with PoPH.

Komplikationen der Leberzirrhose – medikamentöse versus interventionelle Therapie

2019 ◽  
Vol 144 (18) ◽  
pp. 1259-1266
Author(s):  
Lukas Sturm ◽  
Martin Rössle ◽  
Michael Schultheiß
Keyword(s):  
Liver Transplantation ◽  
Liver Cirrhosis ◽  
Variceal Bleeding ◽  
Hepatorenal Syndrome ◽  
Beta Blockers ◽  
Therapeutic Option ◽  
Treatment Options ◽  
Refractory Ascites ◽  
Effective Therapeutic Option ◽  
Intrahepatic Portosystemic Shunt

AbstractThe prognosis of patients with liver cirrhosis is impaired by complications such as variceal bleeding, ascites, hepatorenal syndrome, hepatic encephalopathy and hepatocellular carcinoma. A steadily increasing array of treatment options for these complications is available, including pharmaceutical treatment (e. g. beta blockers for varices or diuretics for ascites), endoscopic treatment (e. g. band ligation of varices), radiological interventions (e. g. transjugular shunt, transarterial chemoembolization) and liver transplantation. Most of the complications occur due to portal hypertension. Therefore, decompressive treatment by implantation of a transjugular intrahepatic portosystemic shunt (TIPS) an effective therapeutic option for many complications of liver cirrhosis. Its main indications are acute and recurrent variceal bleeding in patients with advanced disease as well as refractory ascites. The TIPS does not affect options of abdominal surgery and may therefore be used as a bridge to liver transplantation.

Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil

2020 ◽  
Vol 23 (6) ◽  
pp. 467-471
Author(s):  
Voytek Slowik ◽  
Amber Hildreth ◽  
M Cristina Pacheco ◽  
Laura S Finn ◽  
Jeremy King ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Hepatopulmonary Syndrome ◽  
Normal Activity ◽  
Portosystemic Shunting ◽  
Allograft Function ◽  
Persistent Fatigue

We report a patient without known preexisting liver disease who presented with hepatopulmonary syndrome (HPS) due to aberrant intrahepatic portal venous development leading to portosystemic shunting. Liver transplantation resulted in resolution of portal hypertension and HPS and sildenafil was safely tolerated in the treatment of persistent fatigue and hypoxemia. Twelve months later, patient has normal allograft function and has returned to normal activity.

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