Hepatopulmonary syndrome

Fallon, Michael B.; Abrams, Gary A.

doi:10.1007/s11894-000-0050-8

Cited by 19 publications

(12 citation statements)

References 47 publications

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“…With the disparity of proposed mechanisms it is not surprising that no clearly effective therapy has been developed for HPS to date. 8 Pharmacologic agents that enhance vasoconstriction or inhibit vasodilatation have been used without success. Indomethacin, 9 sympathomimetics, 10 ␤-blockers, 11 and plasma exchange 12 had no beneficial effects.…”

Section: Discussionmentioning

confidence: 99%

Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome

Collisson

Nourmand

Fraiman

et al. 2002

Liver Transplantation

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The hepatopulmonary syndrome (HPS), consisting of elevated alveolar-arterial oxygen gradient and intrapulmonary vascular abnormalities in the presence of advanced liver disease, is associated with high mortality. Liver transplantation (LT) has been used for the treatment of HPS; however, the success of LT for the treatment of HPS is not uniformly documented. We reviewed our experience over a 5-year period and identified eight adult patients with incapacitating respiratory symptoms compatible with HPS. Inclusion criteria included hypoxemia, normal lung volumes, reduced oxygen diffusing capacity (D L CO), and the presence of intrapulmonary shunting. Underlying liver disease was caused by hepatitis C (2 patients), primary biliary cirrhosis (1 patient), cryptogenic cirrhosis (1 patient), alcohol (2 patients), and hepatitis C with alcohol P atients with chronic advanced liver disease frequently show abnormalities in numerous organs. For example, renal insufficiency (hepatorenal syndrome) and peripheral arteriovenous shunting are widely recognized sequelae of cirrhosis. Less often recognized are the defects in pulmonary gas exchange observed with liver disease. A hepatopulmonary syndrome (HPS) has been described and encompasses a specific group of patients with severe liver disease and without cardiorespiratory disease who develop impaired gas exchange because of intrapulmonary vasodilatation (IPVD).In the past, hypoxemia was considered an absolute contraindication to liver transplantation (LT) 1 because of the operative and perioperative complications that arise in patients with gas exchange abnormalities. As the recognition of and experience with HPS has increased, concepts regarding indications LT for transplantation have come full circle, and it has been suggested that, in the face of otherwise stable liver disease, symptomatic HPS in and of itself may constitute an indication for LT. The role for transplantation in HPS is supported by the observation that the metabolic defects responsible for the pulmonary manifestations of HPS clearly are hepatic in origin, progressive, and, at present, refractory to medical management. However, the success of LT for the treatment of HPS is not uniformly documented.In the current study, we reviewed our single-center experience of eight patients with diagnosed HPS evaluated for LT, and report their long-term outcome and complications. Experimental Procedures Preoperative EvaluationAll patients were referred to UCLA for liver transplantation (LT) routinely undergo pulmonary function

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Section: Discussionmentioning

confidence: 99%

Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome

Collisson

Nourmand

Fraiman

et al. 2002

Liver Transplantation

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“…Hepatopulmonary syndrome is the clinical situation which includes liver function abnormalities, intrapulmonary vascular dilatations and hypoxemia [2]. The prevalence of hepatopulmonary syndrome in adult cirrhotic patients ranges between 15-20% [1].…”

Section: Discussionmentioning

confidence: 99%

“…Due to liver dysfunction, the amounts of vasodilator factors such as nitric oxide increase in circulatory system while their clearance is decreased. This is accepted as the main cause of intrapulmonary shunts and vasodilatation [2]. In this case report, we presented the imaging findings of hepatopulmonary syndrome in a cirrhotic patient.…”

Section: Introductionmentioning

confidence: 92%

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Thick section reformatted computed tomography images are useful for the diagnosis of hepatopulmonary syndrome: A case report

Çilengir¹,

Karasu²,

Çetinoğlu³

et al. 2017

Marmara Medical Journal

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Thick section reformatted computed tomography images are useful for the diagnosis of hepatopulmonary syndrome: A case report Hepatopulmoner ABSTRACTRespiratory complications, which range from mild hypoxemia to hepatopulmonary syndrome, can occur due to some mediators within the circulation in end-stage liver disease. Intrapulmonary vascular dilatations and shunts are the reasons of these complications. Thoracic imaging modalities such as radiography and computed tomography (CT) can be performed to evaluate these vascular pathologies. We reported a 37-year-old woman with previously known liver cirrhosis, presented with new onset exertional dyspnea. There were intrapulmonary shunts and dilated vascular structures on her thorax CT. She was diagnosed with hepatopulmonary syndrome by clinical symptoms, laboratory and imaging findings. Thick section reformatted images of CT are superior in demonstration of intrapulmonary shunts and dilated vascular structures compared with thin section axial source images; so we recommend that thorax CT of cirrhotic patients must be evaluated with thick section reformatted images for the determination of intrapulmonary shunts and dilated vascular structures.

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“…HPS does occur in children with biliary atresia 22 and in adults with cholestatic diseases, but is also observed in other forms of cirrhosis and less commonly in pre-and posthepatic portal hypertension. 23 It remains to be determined whether hepatic ET-1 release contributes to pulmonary vasodilatation in these situations. However, a thorough understanding of the pathogenesis of experimental HPS will provide a mechanistic framework to investigate the development of human disease.…”

Section: Discussionmentioning

confidence: 99%

Biliary cyst fluid from common bile duct–ligated rats stimulates endothelial nitric oxide synthase in pulmonary artery endothelial cells: A potential role in hepatopulmonary syndrome

et al. 2001

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The hepatopulmonary syndrome (HPS) results from pulmonary microvascular dilatation in cirrhosis and is associated with increased pulmonary endothelial nitric oxide synthase (eNOS) levels. In the common bile duct ligation (CBDL) model, endothelin-1 (ET-1) released from the liver contributes to the rise in pulmonary eNOS and intrapulmonary vasodilatation. Whether substances, including ET-1, are found in the biliary tree and selectively enter the circulation after CBDL to influence the pulmonary vasculature is unknown. We assessed if control bile and fluid obtained from the obstructed biliary tree in CBDL animals contains ET-1 and alters eNOS expression and activity in bovine pulmonary artery endothelial cells (BPAECs). Control bile and biliary cyst fluid contained concentrations of ET-1 25-to 42-fold normal plasma levels, and hepatic venous concentrations of ET-1 were selectively increased after CBDL. Biliary cyst fluid caused a dose-dependent induction of eNOS messenger RNA (mRNA) (1.9-fold control), protein (2.5-fold control), and enzyme activity (2.2-fold control) maximal at a 1:10 dilution. The increases were associated with enhanced nitric oxide (NO) production ( The hepatopulmonary syndrome (HPS) is one well recognized complication of chronic liver disease characterized by intrapulmonary vasodilatation, which results in impaired arterial oxygenation. 1 It is found in 10% to 20% of patients with cirrhosis and studies in humans suggest that increased pulmonary nitric oxide (NO) production contributes to vasodilatation. 2 In previous studies, we have established chronic common bile duct ligation (CBDL) in the rat as a model of HPS. 3,4 These animals reliably develop increased pulmonary vascular endothelial nitric oxide synthase (eNOS) levels, intrapulmonary vasodilatation, and gas exchange abnormalities similar to human disease. In contrast, prehepatic portal hypertension induced by partial portal vein ligation (PVL) does not trigger pulmonary alterations suggesting that hepatic injury in addition to portal hypertension is required for the development of experimental HPS. Subsequently, we have found a selective and progressive increase in hepatic and plasma endothelin-1 (ET-1) levels after CBDL, which correlate directly with the onset and increase in pulmonary eNOS alterations and gas exchange abnormalities. 5 The rise in hepatic ET-1 levels is accompanied by immunohistochemical localization of ET-1 to biliary epithelium. Most recently, we have observed that delivery of a chronic low level ET-1 infusion to PVL animals triggers an increase in pulmonary eNOS levels, intrapulmonary vasodilatation, and gas exchange abnormalities indicative of HPS. 6 Together, these findings suggest that circulating ET-1 derived from the liver contributes to the development of experimental HPS.However, whether biliary production and release of ET-1 is involved in the unique susceptibility of CBDL animals to HPS remains undefined. We hypothesized that biliary obstruction may result in the release of mediators, including ET-1, from th...

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Hepatopulmonary syndrome

Cited by 19 publications

References 47 publications

Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome

Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome

Thick section reformatted computed tomography images are useful for the diagnosis of hepatopulmonary syndrome: A case report

Biliary cyst fluid from common bile duct–ligated rats stimulates endothelial nitric oxide synthase in pulmonary artery endothelial cells: A potential role in hepatopulmonary syndrome

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