hepatopulmonary syndrome

Summary

Summary: A syndrome characterized by the clinical triad of advanced chronic liver disease, pulmonary vascular dilatations, and reduced arterial oxygenation (HYPOXEMIA) in the absence of intrinsic cardiopulmonary disease. This syndrome is common in the patients with LIVER CIRRHOSIS or portal hypertension (HYPERTENSION, PORTAL).

Top Publications

  1. Fleming G, Cornell T, Welling T, Magee J, Annich G. Hepatopulmonary syndrome: use of extracorporeal life support for life-threatening hypoxia following liver transplantation. Liver Transpl. 2008;14:966-70 pubmed publisher
    b>Hepatopulmonary syndrome is an uncommon complication of nonacute liver failure, and in rare cases, hypoxia may be the presenting sign of liver dysfunction...
  2. Alonso Martinez J, Zozaya Urmeneta J, Gutiérrez Dubois J, Abínzano Guillén M, Urbieta Echezarreta M, Annicchérico Sánchez F. [Prognostic significance of the hepatopulmonary syndrome in liver cirrhosis]. Med Clin (Barc). 2006;127:133-5 pubmed
    The prognosis of hepatopulmonary syndrome (HPS) has been only rarely investigated. We investigated the survival of 32 cirrhotic patients, 14 (44%) with HPS and 18 with a normal gaseous exchange (NGE), and the associated factors...
  3. El Shabrawi M, Omran S, Wageeh S, Isa M, Okasha S, Mohsen N, et al. (99m)Technetium-macroaggregated albumin perfusion lung scan versus contrast enhanced echocardiography in the diagnosis of the hepatopulmonary syndrome in children with chronic liver disease. Eur J Gastroenterol Hepatol. 2010;22:1006-12 pubmed publisher
    The hepatopulmonary syndrome (HPS) is a triad of advanced chronic liver disease (CLD), arterial hypoxemia and intrapulmonary arteriovenous shunting in the absence of a primary cardiopulmonary disease...
  4. Zhang J, Ling Y, Luo B, Tang L, Ryter S, Stockard C, et al. Analysis of pulmonary heme oxygenase-1 and nitric oxide synthase alterations in experimental hepatopulmonary syndrome. Gastroenterology. 2003;125:1441-51 pubmed
    Cirrhosis and portal hypertension due to chronic common bile duct ligation reproduce the features of human hepatopulmonary syndrome, whereas portal hypertension alone due to partial portal vein ligation does not...
  5. Arguedas M, Fallon M. Hepatopulmonary syndrome. Clin Liver Dis. 2005;9:733-46, viii pubmed
    The hepatopulmonary syndrome is an increasingly important vascular complication of cirrhosis where microvascular dilatation impairs arterial oxygenation in the setting of liver disease...
  6. Ryu J, Oh J. Hepatopulmonary syndrome: angiography and therapeutic embolization. Clin Imaging. 2003;27:97-100 pubmed
    ..embolization therapy was successfully performed for diffusely dilated pulmonary vessels in a patient with hepatopulmonary syndrome (HPS) as a palliative treatment before liver transplantation...
  7. Krug S, Seyfarth H, Hagendorff A, Wirtz H. Inhaled iloprost for hepatopulmonary syndrome: improvement of hypoxemia. Eur J Gastroenterol Hepatol. 2007;19:1140-3 pubmed
    b>Hepatopulmonary syndrome is characterized by advanced liver disease, hypoxemia, and intrapulmonary shunting. The only reported curative option is orthotopic liver transplantation...
  8. Saad N, Lee D, Waldman D, Saad W. Pulmonary arterial coil embolization for the management of persistent type I hepatopulmonary syndrome after liver transplantation. J Vasc Interv Radiol. 2007;18:1576-80 pubmed
    b>Hepatopulmonary syndrome (HPS) is a common complication of chronic liver disease. The definitive therapy is liver transplantation...
  9. Gupta S, Castel H, Rao R, Picard M, Lilly L, Faughnan M, et al. Improved survival after liver transplantation in patients with hepatopulmonary syndrome. Am J Transplant. 2010;10:354-63 pubmed publisher
    b>Hepatopulmonary syndrome (HPS) is present in 10-32% of chronic liver disease patients, carries a poor prognosis and is treatable by liver transplantation (LT)...

More Information

Publications62

  1. Muller C, Schenk P. Hepatopulmonary syndrome. Wien Klin Wochenschr. 1999;111:339-47 pubmed
    The hepatopulmonary syndrome (HPS) is a rare complication of liver cirrhosis and is characterised by the typical triad of liver cirrhosis, arterial hypoxemia, and intrapulmonary vascular dilatation...
  2. Zhang H, Han D, Su A, Zhang L, Zhao Z, Ji J, et al. Intestinal endotoxemia plays a central role in development of hepatopulmonary syndrome in a cirrhotic rat model induced by multiple pathogenic factors. World J Gastroenterol. 2007;13:6385-95 pubmed
    To characterize the correlation between severity of hepatopulmonary syndrome (HPS) and degree of hepatic dysfunction, and to explore how intestinal endotoxemia (IETM) affects the development of HPS in cirrhotic rats...
  3. Martinez Palli G, Drake B, García Pagán J, Barbera J, Arguedas M, Rodriguez Roisin R, et al. Effect of transjugular intrahepatic portosystemic shunt on pulmonary gas exchange in patients with portal hypertension and hepatopulmonary syndrome. World J Gastroenterol. 2005;11:6858-62 pubmed
    ..portosystemic shunt (TIPS) on pulmonary gas exchange and to evaluate the use of TIPS for the treatment of hepatopulmonary syndrome ( HPS )...
  4. Tanikella R, Philips G, Faulk D, Kawut S, Fallon M. Pilot study of pentoxifylline in hepatopulmonary syndrome. Liver Transpl. 2008;14:1199-203 pubmed publisher
    b>Hepatopulmonary syndrome (HPS) results when chronic liver disease or portal hypertension causes intrapulmonary microvascular dilatation with hypoxemia...
  5. Zhang H, Han D, Wang X, Zhao Y, Zhou X, Zhao H. Experimental study on the role of endotoxin in the development of hepatopulmonary syndrome. World J Gastroenterol. 2005;11:567-72 pubmed
    To evaluate the role of intestinal endotoxemia in the genesis of hepatopulmonary syndrome. A rat model of cirrhosis was prepared with the method of compound factors...
  6. Mandell M. The diagnosis and treatment of hepatopulmonary syndrome. Clin Liver Dis. 2006;10:387-405, x pubmed
    Few prognostic indicators and outcomes reported for patients who have hepatopulmonary syndrome can be consistently reproduced between institutions...
  7. Rodriguez Roisin R, Krowka M. Hepatopulmonary syndrome--a liver-induced lung vascular disorder. N Engl J Med. 2008;358:2378-87 pubmed publisher
  8. Krowka M. Hepatopulmonary syndrome and portopulmonary hypertension: implications for liver transplantation. Clin Chest Med. 2005;26:587-97, vi pubmed
    b>Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are uncommon pulmonary vascular consequences of advanced liver disease...
  9. Ferreira R, Domingues A, Markman Filho B, Véras F, Batista L, Albuquerque Filho E. Hepatopulmonary syndrome in patients with Schistosoma mansoni periportal fibrosis. Acta Trop. 2009;111:119-24 pubmed publisher
    b>Hepatopulmonary syndrome (HPS) is characterized by the presence of liver disease, arterial hypoxemia and intrapulmonary vascular dilatation (IPVD)...
  10. Añel R, Sheagren J. Novel presentation and approach to management of hepatopulmonary syndrome with use of antimicrobial agents. Clin Infect Dis. 2001;32:E131-6 pubmed
    ..b>Hepatopulmonary syndrome was diagnosed after demonstration of the presence of a massive intrapulmonary shunt...
  11. Koch D, BOGATKEVICH G, Ramshesh V, Lemasters J, Uflacker R, Reuben A. Elevated levels of endothelin-1 in hepatic venous blood are associated with intrapulmonary vasodilatation in humans. Dig Dis Sci. 2012;57:516-23 pubmed publisher
    b>Hepatopulmonary syndrome is a pulmonary vascular complication of cirrhosis in which intrapulmonary vasodilatation (IPV) results in hypoxemia...
  12. Regev A, Yeshurun M, Rodriguez M, Sagie A, Neff G, Molina E, et al. Transient hepatopulmonary syndrome in a patient with acute hepatitis A. J Viral Hepat. 2001;8:83-6 pubmed
    The hepatopulmonary syndrome is defined as the triad of liver disease, hypoxaemia and intrapulmonary vascular dilatation...
  13. Gupta S, Faughnan M, Lilly L, Hutchison S, Fowler R, Bayoumi A. Norfloxacin therapy for hepatopulmonary syndrome: a pilot randomized controlled trial. Clin Gastroenterol Hepatol. 2010;8:1095-8 pubmed publisher
    The hepatopulmonary syndrome occurs in up to one-third of patients with cirrhosis...
  14. Roma J, Balbi E, Pacheco Moreira L, Gonzalez A, Leal C, Pousa F, et al. Methylene blue used as a bridge to liver transplantation postoperative recovery: a case report. Transplant Proc. 2010;42:601-4 pubmed publisher
    b>Hepatopulmonary syndrome is defined as a triad of liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation...
  15. Theysohn J, Schlaak J, Muller S, Ertle J, Schlosser T, Bockisch A, et al. Selective internal radiation therapy of hepatocellular carcinoma: potential hepatopulmonary shunt reduction after sorafenib administration. J Vasc Interv Radiol. 2012;23:949-52 pubmed publisher
    ..Repeated measurements revealed HPS reduction (from 26.5% to 7.5% on average), and subsequent SIRT became possible. In conclusion, sorafenib may reduce HPS in patients with advanced HCC in some cases. ..
  16. Ling Y, Zhang J, Luo B, Song D, Liu L, Tang L, et al. The role of endothelin-1 and the endothelin B receptor in the pathogenesis of hepatopulmonary syndrome in the rat. Hepatology. 2004;39:1593-602 pubmed
    ..of inducible nitric oxide synthase (iNOS) and heme oxygenase-1 (HO-1) are implicated in experimental hepatopulmonary syndrome (HPS) after common bile duct ligation (CBDL)...
  17. Zhang X, Katsuta Y, Akimoto T, Ohsuga M, Aramaki T, Takano T. Intrapulmonary vascular dilatation and nitric oxide in hypoxemic rats with chronic bile duct ligation. J Hepatol. 2003;39:724-30 pubmed
    Nitric oxide (NO) has been suggested as the major cause of pulmonary vascular dilatation and hypoxemia in hepatopulmonary syndrome (HPS)...
  18. Karabulut A, Iltumur K, Yalcin K, Toprak N. Hepatopulmonary syndrome and right ventricular diastolic functions: an echocardiographic examination. Echocardiography. 2006;23:271-8 pubmed
    ..74%) cirrhotic patients with hepatopulmonary syndrome (HPS) (group 1), 36 (78...
  19. Chang C, Chuang C, Lee F, Wang S, Lin H, Huang H, et al. Sorafenib treatment improves hepatopulmonary syndrome in rats with biliary cirrhosis. Clin Sci (Lond). 2013;124:457-66 pubmed publisher
    HPS (hepatopulmonary syndrome) is characterized by oxygen desaturation in patients with chronic liver disease. The initiation of HPS comes from abnormal pulmonary vasodilatation and/or angiogenesis...
  20. Kamath P. Portopulmonary hypertension and hepatopulmonary syndrome. J Gastroenterol Hepatol. 2002;17 Suppl 3:S253-5 pubmed
  21. Macêdo L, Lopes E, Albuquerque M, Markman Filho B, Véras F, Araújo A, et al. Occurrence of hepatopulmonary syndrome in patients with cirrhosis who are candidates for liver transplantation. J Bras Pneumol. 2010;36:432-40 pubmed
    To determine the occurrence of hepatopulmonary syndrome (HPS) in patients with cirrhosis who are candidates for liver transplantation; to compare demographic, clinical, laboratory, and spirometric characteristics, as well as ..
  22. Kinane T, Westra S. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 31-2004. A four-year-old boy with hypoxemia. N Engl J Med. 2004;351:1667-75 pubmed
  23. Fallon M, Krowka M, Brown R, Trotter J, Zacks S, Roberts K, et al. Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterology. 2008;135:1168-75 pubmed publisher
    b>Hepatopulmonary syndrome (HPS) affects 10%-30% of patients with cirrhosis and portal hypertension, but the impact on functional status, quality of life, and survival is poorly defined...
  24. Liu L, Zhang M, Luo B, Abrams G, Fallon M. 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. Hepatology. 2001;33:722-7 pubmed
    The hepatopulmonary syndrome (HPS) results from pulmonary microvascular dilatation in cirrhosis and is associated with increased pulmonary endothelial nitric oxide synthase (eNOS) levels...
  25. Nunes H, Lebrec D, Mazmanian M, Capron F, Heller J, Tazi K, et al. Role of nitric oxide in hepatopulmonary syndrome in cirrhotic rats. Am J Respir Crit Care Med. 2001;164:879-85 pubmed
    The hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilatations and an increased alveolar-arterial oxygen difference (AaPO(2))...
  26. De B, Sen S, Biswas P, Sanyal R, Majumdar D, Biswas J. Hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty. Gastroenterology. 2000;118:192-6 pubmed
    Reports show that hepatopulmonary syndrome mostly occurs in the setting of advanced hepatic dysfunction, with the associated vasoactive substance imbalance believed to be responsible for its pathogenesis...
  27. Tekin A, Turkyilmaz S, Kucukkartallar T, Cakir M, Yilmaz H, Esen H, et al. Effects of caffeic acid phenethyl ester (CAPE) on hepatopulmonary syndrome. Inflammation. 2011;34:614-9 pubmed publisher
    ..ester (CAPE) on inflammatory and related histopathological changes in the lung and liver in experimental hepatopulmonary syndrome (HPS) model. Forty Sprague Dawley rats were used in this study...
  28. Tumgor G, Berdeli A, Arikan C, Levent E, Aydogdu S. Mcp-1, eNOS, tPA and PAI-1 gene polymorphism and correlation of genotypes and phenotypes in hepatopulmonary syndrome. Dig Dis Sci. 2008;53:1345-51 pubmed
    ..05). HPS is more common in patients with MCP-1 2518G gene carriage; conversely it is less frequent in patients with high frequency of eNOS 298Asp allele and eNOS 298Asp carriage. ..
  29. Kianifar H, Khalesi M, Mahmoodi E, Afzal Aghaei M. Pentoxifylline in hepatopulmonary syndrome. World J Gastroenterol. 2012;18:4912-6 pubmed
    ..the effects of pentoxifylline (PTX) on clinical manifestations and evaluate arterial blood gas data in hepatopulmonary syndrome (HPS) in children...
  30. Palma D, Fallon M. The hepatopulmonary syndrome. J Hepatol. 2006;45:617-25 pubmed
  31. Motomura T, Ikegami T, Mano Y, Nagata S, Sugimachi K, Gion T, et al. Living donor liver transplantation for end-stage liver disease with severe hepatopulmonary syndrome: report of a case. Surg Today. 2011;41:436-40 pubmed publisher
    b>Hepatopulmonary syndrome (HPS) is a serious complication of terminal liver disease, which manifests as severe hypoxia without any pulmonary anatomic or functional causes...
  32. Martínez G, Barbera J, Visa J, Rimola A, Pare J, Roca J, et al. Hepatopulmonary syndrome in candidates for liver transplantation. J Hepatol. 2001;34:651-7 pubmed
    b>Hepatopulmonary syndrome (HPS) has been defined as a clinical triad, including chronic liver disease, gas exchange defects (increased alveolar-arterial PO2 difference irrespective of the presence of arterial hypoxemia), and widespread ..
  33. Sindhu S, Ramesh P, Juneja R, Kabra S. Hepatopulmonary syndrome, an unusual cause of hypoxemia. Indian J Pediatr. 2007;74:1127-9 pubmed
    ..A diagnosis of hepatopulmonary syndrome was made based on the triad of hypoxemia, liver disease and intra pulmonary vascular communications.
  34. Luo B, Liu L, Tang L, Zhang J, Stockard C, Grizzle W, et al. Increased pulmonary vascular endothelin B receptor expression and responsiveness to endothelin-1 in cirrhotic and portal hypertensive rats: a potential mechanism in experimental hepatopulmonary syndrome. J Hepatol. 2003;38:556-63 pubmed
    In experimental hepatopulmonary syndrome (HPS), hepatic endothelin-1 (ET-1) release during common bile duct ligation (CBDL) and ET-1 infusion in pre-hepatic portal hypertension after portal vein ligation (PVL) initiate vasodilatation ..
  35. Lima B, França A, Pazin Filho A, Araújo W, Martinez J, Maciel B, et al. Frequency, clinical characteristics, and respiratory parameters of hepatopulmonary syndrome. Mayo Clin Proc. 2004;79:42-8 pubmed
    To determine the frequency and the clinical characteristics of hepatopulmonary syndrome (HPS) in cirrhotic candidates for orthotopic liver transplantation and to identify the major respiratory parameters predictive of the presence of ..
  36. Zhang Z, Yang C. Progress in investigating the pathogenesis of hepatopulmonary syndrome. Hepatobiliary Pancreat Dis Int. 2010;9:355-60 pubmed
    The pathogenesis of hepatopulmonary syndrome is complicated and remains unknown. This review aims to provide an updated knowledge about the pathogenesis of the syndrome...
  37. Dehghani S, Aleyasin S, Honar N, Eshraghian A, Kashef S, Haghighat M, et al. Pulmonary evaluation in pediatric liver transplant candidates. Indian J Pediatr. 2011;78:171-5 pubmed publisher
    ..Patients with and without hepatopulmonary syndrome (HPS) as well as patients with and without severe hypoxemia (partial pressure of arterial oxygen, PaO(2)&..
  38. Zeng J, Yi B, Wang Z, Ning J, Wang X, Lu K. Effect of annexin A2 on hepatopulmonary syndrome rat serum-induced proliferation of pulmonary arterial smooth muscle cells. Respir Physiol Neurobiol. 2013;185:332-8 pubmed publisher
    b>Hepatopulmonary syndrome (HPS) is characterizes by an arterial oxygenation defect induced by intrapulmonary vasodilation that increase morbidity and mortality...
  39. Zhang J, Luo B, Tang L, Wang Y, Stockard C, Kadish I, et al. Pulmonary angiogenesis in a rat model of hepatopulmonary syndrome. Gastroenterology. 2009;136:1070-80 pubmed publisher
    b>Hepatopulmonary syndrome (HPS), defined as intrapulmonary vasodilation, occurs in 10%-30% of cirrhotics and increases mortality...
  40. Zamirian M, Aslani A, Shahrzad S. Left atrial volume: a novel predictor of hepatopulmonary syndrome. Am J Gastroenterol. 2007;102:1392-6 pubmed
    ..903 (Cut point >/= 50 mL, sensitivity 86.3%, specificity 81.2%). In the context of liver cirrhosis, LAV >/= 50 mL is a simple and feasible parameter to detect HPS. ..
  41. Fuhrmann V, Madl C, Mueller C, Holzinger U, Kitzberger R, Funk G, et al. Hepatopulmonary syndrome in patients with hypoxic hepatitis. Gastroenterology. 2006;131:69-75 pubmed
    The hepatopulmonary syndrome (HPS) is defined as the triad of liver disease, arterial deoxygenation, and widespread pulmonary vasodilatation...
  42. Luo B, Liu L, Tang L, Zhang J, Ling Y, Fallon M. ET-1 and TNF-alpha in HPS: analysis in prehepatic portal hypertension and biliary and nonbiliary cirrhosis in rats. Am J Physiol Gastrointest Liver Physiol. 2004;286:G294-303 pubmed
    Common bile duct ligation (CBDL) triggers a molecular cascade resulting in the hepatopulmonary syndrome (HPS)...
  43. Tang L, Luo B, Patel R, Ling Y, Zhang J, Fallon M. Modulation of pulmonary endothelial endothelin B receptor expression and signaling: implications for experimental hepatopulmonary syndrome. Am J Physiol Lung Cell Mol Physiol. 2007;292:L1467-72 pubmed
    The hepatopulmonary syndrome (HPS) results from intrapulmonary vasodilation in the setting of cirrhosis and portal hypertension...
  44. Fallon M. Mechanisms of pulmonary vascular complications of liver disease: hepatopulmonary syndrome. J Clin Gastroenterol. 2005;39:S138-42 pubmed
    ..The most common such abnormality, the hepatopulmonary syndrome, is found in 15% to 20% of patients with cirrhosis. These disorders have no effective medical therapies...
  45. Sztrymf B, Rabiller A, Nunes H, Savale L, Lebrec D, Le Pape A, et al. Prevention of hepatopulmonary syndrome and hyperdynamic state by pentoxifylline in cirrhotic rats. Eur Respir J. 2004;23:752-8 pubmed
    ..b>Hepatopulmonary syndrome, which is characterised by intrapulmonary vascular dilatation and increased alveolar to arterial oxygen ..
  46. Brussino L, Bucca C, Morello M, Scappaticci E, Mauro M, Rolla G. Effect on dyspnoea and hypoxaemia of inhaled N(G)-nitro-L-arginine methyl ester in hepatopulmonary syndrome. Lancet. 2003;362:43-4 pubmed
    b>Hepatopulmonary syndrome--a complication of chronic liver disease-is characterised by hypoxaemia, which results from widespread intrapulmonary vascular dilatations...
  47. Arguedas M, Abrams G, Krowka M, Fallon M. Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation. Hepatology. 2003;37:192-7 pubmed
    The hepatopulmonary syndrome (HPS) occurs in a subgroup of patients with cirrhosis and results from intrapulmonary vasodilatation, which may cause significant hypoxemia...
  48. Carter E, Hartsfield C, Miyazono M, Jakkula M, Morris K, McMurtry I. Regulation of heme oxygenase-1 by nitric oxide during hepatopulmonary syndrome. Am J Physiol Lung Cell Mol Physiol. 2002;283:L346-53 pubmed
    During hepatopulmonary syndrome caused by liver cirrhosis, pulmonary endothelial nitric oxide (NO) synthase (NOS) expression and NO production are increased...
  49. De B, Sen S, Biswas P, Mandal S, Das D, Das U, et al. Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression. Gastroenterology. 2002;122:897-903 pubmed
    b>Hepatopulmonary syndrome (HPS) has been predominantly detected in cirrhotic patients and rarely in patients with noncirrhotic portal hypertension...
  50. Vercelino R, Tieppo J, Dias A, Marroni C, Garcia E, Meurer L, et al. N-acetylcysteine effects on genotoxic and oxidative stress parameters in cirrhotic rats with hepatopulmonary syndrome. Basic Clin Pharmacol Toxicol. 2008;102:370-6 pubmed publisher
    The aim of this study was to evaluate the potential antioxidant effects of N-acetylcysteine in hepatopulmonary syndrome, a complication of cirrhosis, using an experimental model of common bile duct ligation in rats...
  51. Pacca R, Maddukuri P, Pandian N, Kuvin J. Echocardiographic detection of intrapulmonary shunting in a patient with hepatopulmonary syndrome: case report and review of the literature. Echocardiography. 2006;23:56-9 pubmed
    ..study helped to establish the diagnosis of intrapulmonary arteriovenous shunting consistent with the hepatopulmonary syndrome, thereby confirming the etiology of her symptoms and laboratory findings...
  52. Rabiller A, Nunes H, Lebrec D, Tazi K, Wartski M, Dulmet E, et al. Prevention of gram-negative translocation reduces the severity of hepatopulmonary syndrome. Am J Respir Crit Care Med. 2002;166:514-7 pubmed
    b>Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilatations and an increased alveoloarterial oxygen difference (AaPO(2))...
  53. Schenk P, Madl C, Rezaie Majd S, Lehr S, Muller C. Methylene blue improves the hepatopulmonary syndrome. Ann Intern Med. 2000;133:701-6 pubmed
    The hypoxemia of the hepatopulmonary syndrome, seen in patients with severe chronic liver dysfunction, results from widespread pulmonary vasodilation. No established drug therapy is available for this condition...