Hepatorenal syndrome, hepatopulmonary syndrome, and now, hepatospinal syndrome?
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Hepatopulmonary Syndrome↗
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Transjugular intrahepatic portosystemic shunt improves oxygenation in hepatopulmonary syndrome.
Hepatopulmonary syndrome is a complication of chronic liver disease in which arterial hypoxemia results from abnormalities in pulmonary blood flow. Severe hypoxemia can lead to clinical deterioration and death. Although the etiology is unknown, portal hypertension seems to be an important factor in the development of hepatopulmonary syndrome. No effective pharmacological therapy has been identified, but liver transplantation may be curative. Arterial hypoxemia may complicate transplant surgery, however, and resolution of the syndrome after liver transplantation is performed may be delayed. In addition, it seems that complete reversal of oxygenation abnormalities after liver transplantation is performed is unpredictable. We described a patient with hepatopulmonary syndrome who noted improvement in symptoms of dyspnea after the placement of a transjugular intrahepatic portosystemic shunt. Arterial oxygenation and calculated shunt fraction improved significantly during the follow-up period, and liver transplantation was subsequently performed without difficulty. Portal decompression using transjugular intrahepatic portosystemic shunt may represent a palliative therapy for hepatopulmonary syndrome in patients awaiting liver transplantation.
Female↗
Effect on dyspnoea and hypoxaemia of inhaled N(G)-nitro-L-arginine methyl ester in hepatopulmonary syndrome.
Hepatopulmonary syndrome--a complication of chronic liver disease-is characterised by hypoxaemia, which results from widespread intrapulmonary vascular dilatations. Amplified production of pulmonary nitric oxide is thought to be important in development of this disorder in patients with liver cirrhosis. Here, we report a 64-year-old man with hepatopulmonary syndrome associated with hepatitis-C-virus-related cirrhosis. We gave the patient nebulised N(G)-nitro-L-arginine methyl ester (L-NAME), an inhibitor of NO synthesis, which enhanced oxygenation (arterial oxygen pressure increased from 6.98 to 9.45 kPa). After L-NAME, the distance the patient could walk in 6 min rose by 92 m. Administration of L-NAME by aerosol might offer a new approach to treatment of hepatopulmonary syndrome.
Administration, Inhalation↗
Transjugular intrahepatic portosystemic shunt: a successful treatment for hepatopulmonary syndrome.
Hepatopulmonary syndrome is a well described complication of chronic liver disease. Though uncommon, it carries a high morbidity and mortality. The pathogenesis of the syndrome has not been clearly defined. Portal hypertension seems to play a crucial role in the pathogenesis of the syndrome, probably by enhancing nitric oxide production. As yet, no pharmacological therapy has been proven effective. Many reports of successful reversal of the syndrome after liver transplantation have been published. We report a patient with hepatopulmonary syndrome who showed a significant and durable (4 months') improvement in his symptoms, arterial oxygenation, and intrapulmonary shunts, as calculated by radionuclide studies after transjugular intrahepatic portosystemic shunt placement. Transjugular intrahepatic portosystemic shunt may represent a durable treatment option for patients with hepatopulmonary syndrome.
Dilatation, Pathologic↗
Hypoxaemia--think of the liver! Every internist should be aware of the hepatopulmonary syndrome.
Hepatopulmonary syndrome is characterised by arterial hypoxaemia, liver disease, and intrapulmonary vascular dilatation. A case is reported in which severe hypoxaemia, detected by chance, led to the diagnosis of liver disease and hepatopulmonary syndrome.
Hepatopulmonary Syndrome↗
Liver transplantation of a child with child a cirrhosis and severe hepatopulmonary syndrome.
Hepatopulmonary syndrome (HPS) is a clinical state defined by a chronic hepatic disorder, intrapulmonary vascular dilatation, and altered gas exchange resulting in hypoxemia. Cirrhosis of the liver is the most common condition associated with HPS. A 3-year-old boy who presented with end-stage liver disease and severe hepatopulmonary syndrome underwent orthotopic liver transplantation (OLT). The findings of HPS resolved immediately after OLT. His status is within normal limits at 6 months after liver transplantation.
Angiography↗
Recent advances in hepatopulmonary syndrome.
Hepatopulmonary syndrome is defined as the clinical triad of advanced liver disease, arterial deoxygenation and intrapulmonary vascular dilatation. Its pathogenesis is not completely understood. Excessive pulmonary nitric oxide production seems to be one of the factors that contribute to the intrapulmonary vascular dilatation. Other mediators such as endothelin-1 and the heme oxygenase-1/carbon monoxide system have recently been found to be important contributors. The major clinical manifestations are arterial hypoxemia, clubbed fingers and spider nevi. Orthodeoxia is the characteristic clinical feature. Contrast-enhanced echocardiography is the preferred screening test. 99mTechnetium macroaggregated albumin (Tc-99m MAA) lung perfusion scan can further specify the diagnosis of hepatopulmonary syndrome and quantify the magnitude of shunting. No clearly effective medical treatments have been found. Although liver transplantation seems feasible to reverse this situation, it is associated with increased postoperative morbidity and mortality. A preoperative arterial oxygen tension of 50 mmHg or less and Tc-99m MAA shunt fractions of 20% or more are strong predictors of postoperative mortality that can be used to stratify patients with better outcome.
Carbon Monoxide↗
Abernethy malformation: one of the etiologies of hepatopulmonary syndrome.
Hepatopulmonary syndrome (HPS) is the clinical relationship between hepatic disease and the existence of pulmonary vascular dilatations, which can result in a range of arterial oxygenation abnormalities. It is probably caused by an alteration in the synthesis or metabolism of vasoactive pulmonary substances at a hepatic level, leading to vasodilatation of pulmonary vessels and diffusion perfusion defects. The Abernethy malformation is characterized by the congenital diversion of portal blood away from the liver, by either end-to-side or side-to-side shunt. Here, we report on a 5-year-and-11-month-old-boy who had started cyanosis at age 4 years and 11 months, and did not have any other pulmonary or cardiac signs or symptoms. In the investigation, arterial blood gases revealed a PaO(2) of 41.4 mm Hg. The chest x-ray film and echo Doppler cardiography were normal. Nuclear scanning with Technetium 99m-labeled macroaggregated albumin showed the presence of arteriovenous shunt, at 47%. Abdominal echography revealed Abernethy malformation with an absence of portal vein. We concluded that the patient had HPS caused by Abernethy malformation. The possible mechanism is that in this malformation, there is a deviation in the blood that comes from the spleen to the vena cava without passing through the liver, so there is no metabolism of some substances which can be responsible for the imbalance between the vasodilatation and the vasoconstriction of the pulmonary circulation. Abernethy malformation must be included as one of the etiologies of hepatopulmonary syndrome. This is the first case described in the literature with this form of presentation.
Arteriovenous Fistula↗
[Hepatopulmonary syndrome].
Hepatopulmonary Syndrome is an uncommon clinical situation of unknown cause. It remains the focus of intense investigation and ongoing debate. The authors present a case of a 77 year old man with chronic liver disease known for 5 years, who developed central cyanoses, digital clubbing and hypoxemia. On searching for the cause of these clinical features, the diagnosis of Hepatopulmonary Syndrome was admitted and confirmed by contrast enhanced echocardiography using agitated saline, and also by technetium 99m-labelled macroaggregated albumin scanning.
Aged↗
Paroxetine for hepatopulmonary syndrome?
Hepatopulmonary syndrome (HPS) is a severe pulmonary sequela which can affect up to 15% of patients with cirrhotic liver disease. There is no treatment for HPS other than liver transplantation. The cause of HPS is incompletely understood, but the pulmonary vasodilation in HPS is thought to be related to increased levels of nitric oxide. We therefore suggest that, the extremely safe and commonly used antidepressant medication paroxetine (Paxil) which is a potent nitric oxide synthase (NOS) inhibitor be considered for use in HPS.
Hepatopulmonary Syndrome↗
Hepatopulmonary syndrome.
Hepatopulmonary syndrome is caused by intrapulmonary vasodilation that leads to abnormal arterial gas exchange in the setting of liver disease or portal hypertension. It is seen in up to 15% of cirrhotics and is an increasingly common indication for liver transplantation. Testing for the presence of oxygenation abnormalities and intrapulmonary vasodilation is needed to make the diagnosis. Excess production of nitric oxide in the lung contributes to pulmonary vasodilation and may be triggered by the release of mediators from the damaged liver. No medical therapies are established as effective, and liver transplantation is the only documented curative treatment.
Animals↗
Use of transjugular intrahepatic portosystemic shunt as a bridge to liver transplantation in a patient with severe hepatopulmonary syndrome.
Hepatopulmonary syndrome (HPS) is defined by the presence of the triad of liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation. The clinical implication of this disorder is impairment of gas exchange. Numerous reports in the literature show that this condition is reversible with orthotopic liver transplantation (OLT). However, patients with HPS often present with PaO(2) levels that are quite low. OLT with a preoperative PaO(2) less than 50 mm Hg is associated with unacceptably high mortality and morbidity. We report a case of severe HPS in which a transjugular intrahepatic portosystemic shunt was successfully used to improve oxygenation, thus allowing a successful elective OLT.
Female↗
Successful liver transplantation in a child with biliary atresia and hepatopulmonary syndrome.
Hepatopulmonary syndrome (HPS) is characterized by hypoxemia in patients with severe chronic liver disease and pulmonary vasodilatation in the absence of primary cardiac or pulmonary disease. Severe hypoxemia resulting from HPS is generally considered a contraindication to liver transplantation. We describe the case of a 6-year-old girl with biliary atresia complicated with HPS who was successfully treated with liver transplantation. Cyanosis and dyspnea had initially developed at the age of 5 years. Arterial blood gas showed a PaO2 of 46.6 mm Hg on room air. The diagnosis of HPS was confirmed by contrast echocardiography, lung perfusion scan with 99mTc macroaggregate albumin, and pulmonary angiography. The lung scan revealed an intrapulmonary shunt of 24%. She underwent living donor liver transplantation and received a left lateral segment graft from her mother. One year after successful liver transplantation, she had normal arterial oxygen saturation and a normal lung scan without intrapulmonary shunting. This case demonstrates that HPS associated with end-stage liver disease is potentially curable by liver transplantation.
Biliary Atresia↗
Pentoxifylline attenuation of experimental hepatopulmonary syndrome.
Hepatopulmonary syndrome (HPS) following rat common bile duct ligation results from pulmonary molecular changes that may be influenced by circulating TNF-alpha and increased vascular shear stress, through activation of NF-kappaB or Akt. Increased pulmonary microvascular endothelin B (ET(B)) receptor and endothelial nitric oxide synthase (eNOS) levels contribute to nitric oxide production and the development of experimental HPS. Pentoxifylline (PTX), a phosphodiesterase and nonspecific TNF-alpha inhibitor, ameliorates experimental HPS when begun before hepatic injury. However, how PTX influences the molecular events associated with initiation of experimental HPS after liver injury is established is unknown. We assessed the effects of PTX on the molecular and physiological features of HPS in vivo and on shear stress or TNF-alpha-mediated events in rat pulmonary microvascular endothelial cells in vitro. PTX significantly improved HPS without altering portal or systemic hemodynamics and downregulated pulmonary ET(B) receptor levels and eNOS expression and activation. These changes were associated with a reduction in circulating TNF levels and NF-kappaB activation and complete inhibition of Akt activation. In rat pulmonary microvascular endothelial cells, PTX inhibited shear stress-induced ET(B) receptor and eNOS expression and eNOS activation. These effects were also associated with inhibition of Akt activation and were reproduced by wortmanin. In contrast, TNF-alpha had no effects on endothelial ET(B) and eNOS alterations in vitro. PTX has direct effects in the pulmonary microvasculature, likely mediated through Akt inhibition, that ameliorate experimental HPS.
Animals↗
Prevention of gram-negative translocation reduces the severity of hepatopulmonary syndrome.
Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilatations and an increased alveoloarterial oxygen difference (AaPO(2)). These abnormalities are related to augmented pulmonary nitric oxide (NO) production, dependent primarily on increases in the expression and activity of inducible NO-synthase (iNOS) within pulmonary intravascular macrophages and, to a lesser extent, of endothelial NOS (eNOS). Production of iNOS by pulmonary intravascular macrophages might be related to translocated gut bacteria present in the pulmonary circulation. To test this hypothesis, we determined whether macrophage sequestration, lung iNOS expression and activity, and HPS severity were decreased after norfloxacin was given for 5 weeks to prevent Gram-negative bacterial translocation in rats with common bile duct ligation-induced cirrhosis. Norfloxacin decreased the incidence of Gram-negative translocation from 70 to 0% and the percentage of pulmonary microvessels containing more than 10 macrophages from 52 +/- 7 to 21 +/- 8% (p < 0.01). AaPO(2) and cerebral uptake of intravenous (99m)Tc-labeled albumin macroaggregates (reflecting intrapulmonary vascular dilatations) were intermediate to those of untreated cirrhotic and sham-operated rats. The activity and expression of lung iNOS, but not eNOS, were reduced to normal. Norfloxacin may reduce HPS severity by inhibiting Gram-negative bacterial translocation, thereby decreasing NO production by pulmonary intravascular macrophages. Bacterial translocation may be the key to the pathogenesis of HPS.
Animals↗
Common bile duct ligation in the rat: a model of intrapulmonary vasodilatation and hepatopulmonary syndrome.
Hepatopulmonary syndrome (HPS) causes impaired oxygenation due to intrapulmonary vasodilatation in patients with cirrhosis. Chronic common bile duct ligation (CBDL) in the rat results in gas-exchange abnormalities similar to HPS, but intrapulmonary vasodilatation has not been evaluated. We assess intrapulmonary vasodilatation, measured in vivo, after CBDL. Sham, 2- and 5-wk CBDL, and 3-wk partial portal vein ligated (PVL) rats had hepatic and lung injury, portal pressure, and arterial blood gases assessed. The pulmonary microcirculation was evaluated by injecting microspheres (size range 5.5-10 microm) intravenously and measuring the size and number of microspheres bypassing the lungs in arterial blood. CBDL animals developed progressive hepatic injury and portal hypertension accompanied by gas-exchange abnormalities and intrapulmonary vasodilatation. PVL animals, with a similar degree of portal hypertension, did not develop intrapulmonary vasodilatation or abnormal gas exchange. No lung injury was observed. CBDL, but not PVL, causes progressive intrapulmonary vasodilatation, which accompanies worsening arterial gas exchange. These findings validate CBDL as a model to study HPS.
Animals↗
Pregnancy exacerbating hepatopulmonary syndrome.
BACKGROUND: Hepatopulmonary syndrome is an uncommon complication of liver cirrhosis. The natural history of this condition and its optimal management during pregnancy are not yet known. CASE: We present the case of a 35-year-old woman with liver cirrhosis who developed severe dyspnea in the 25th week of gestation and was diagnosed as suffering from hepatopulmonary syndrome. She was managed conservatively until 35 weeks of gestation, when she was delivered by cesarean. CONCLUSION: The natural history, in this case, indicates that pregnancy may induce hepatopulmonary syndrome in an otherwise asymptomatic cirrhotic patient. Oxygen supplementation was the cornerstone of treatment and resulted in a favorable outcome.
Adult↗
Regulation of heme oxygenase-1 by nitric oxide during hepatopulmonary syndrome.
During hepatopulmonary syndrome caused by liver cirrhosis, pulmonary endothelial nitric oxide (NO) synthase (NOS) expression and NO production are increased. Increased NO contributes to the blunted hypoxic pressor response (HPR) during cirrhosis and may induce heme oxygenase-1 (HO-1) expression and carbon monoxide (CO) production, exacerbating the blunted HPR. We hypothesized that NO regulates the expression of HO-1 during cirrhosis, contributing to hepatopulmonary syndrome. Cirrhosis was induced in rats by common bile duct ligation (CBDL). Rats were studied 2 and 5 wk after CBDL or sham surgery. Lung HO-1 expression was elevated 5 wk after CBDL. Liver HO-1 was increased at 2 wk and remained elevated at 5 wk. In catheterized rats, the blunted HPR was partially restored by HO inhibition. Rats treated with the NOS inhibitor N(G)-nitro-L-arginine methyl ester for the entire 2- or 5-wk duration had normalized HO-1 expression and HPR. These data provide in vivo evidence for the NO-mediated upregulation of HO-1 expression and support the concept that hepatopulmonary syndrome is multifactorial, involving not only NO, but also HO-1 and CO.
Animals↗