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Hepatopulmonary Syndrome

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Abstract

Hepatopulmonary syndrome (HPS) is a pulmonary complication observed in patients with chronic liver disease and/or portal hypertension, attributable to an intrapulmonary vascular dilatation that may induce severe hypoxemia. Microvascular dilation and angiogenesis in the lung have been identified as pathologic features that drive gas exchange abnormalities in experimental HPS. Pulse oximetry is a useful screening test for HPS, which can guide subsequent use of arterial blood gases. Contrast-enhanced echocardiography, perfusion lung scanning, and pulmonary arteriography are three currently used diagnostic imaging modalities that identify the presence of intrapulmonary vascular abnormalities. The presence of HPS increases mortality and impairs quality of life, but is reversible with liver transplantation. No medical therapy is established as effective for HPS. At the present time, liver transplantation is the only available treatment for HPS.

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References

  1. Ratti L, Pozzi M. The pulmonary involvement in portal hypertension: portopulmonary hypertension and hepatopulmonary syndrome. Gastroenterol Hepatol. 2006;29:40–50.

    CAS  PubMed  Google Scholar 

  2. Machicao VI, Balakrishnan M, Fallon MB. Pulmonary complications in chronic liver disease. Hepatology. 2014;59:1627–1637.

    PubMed  Google Scholar 

  3. Fritz JS, Fallon MB, Kawut SM. Pulmonary vascular complications of liver disease. Am J Respir Crit Care Med. 2013;187:133–143.

    PubMed Central  PubMed  Google Scholar 

  4. Ioachimescu OC, Mehta AC, Stoller JK. Hepatopulmonary syndrome following portopulmonary hypertension. Eur Respir J. 2007;29:1277–1280.

    CAS  PubMed  Google Scholar 

  5. Martinez GP, Barbera JA, Visa J, Rimola A, et al. Hepatopulmonary syndrome in candidates for liver transplantation. J Hepatol. 2001;34:651–657.

    CAS  PubMed  Google Scholar 

  6. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004;363:1461–1468.

    PubMed  Google Scholar 

  7. Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB. Pulmonary–hepatic vascular Disorders (PHD). Eur Respir J. 2004;24:861–880.

    CAS  PubMed  Google Scholar 

  8. Rodriguez-Roisin R, Agusti AG, Roca J. The hepatopulmonary syndrome: new name, old complexities. Thorax. 1992;47:897–902.

    CAS  PubMed Central  PubMed  Google Scholar 

  9. Lee JM, Choi MS, Lee SC, Park SW, et al. Prevalence and risk factors of significant intrapulmonary shunt in cirrhotic patients awaiting liver transplantation. Taehan Kan Hakhoe Chi. 2002;8:271–276.

    PubMed  Google Scholar 

  10. Noli K, Solomon M, Golding F, Charron M, Ling SC. Prevalence of hepatopulmonary syndrome in children. Pediatrics. 2008;121:e522–e527.

    PubMed  Google Scholar 

  11. Gupta D, Vijaya DR, Gupta R, Dhiman RK, et al. Prevalence of hepatopulmonary syndrome in cirrhosis and extrahepatic portal venous obstruction. Am J Gastroenterol. 2001;96:3395–3399.

    CAS  PubMed  Google Scholar 

  12. Espinosa MD, Nogueras F, Olmedo C, Macias R, et al. Hepatopulmonary syndrome among cirrhotic candidates for liver transplantation. Transplant Proc. 2012;44:1508–1509.

    CAS  PubMed  Google Scholar 

  13. Ferreira PP, Camara EJ, Paula RL, Zollinger CC, et al. Prevalence of hepatopulmonary syndrome in patients with decompensated chronic liver disease and its impact on short-term survival. Arq Gastroenterol. 2008;45:34–37.

    PubMed  Google Scholar 

  14. Macedo LG, Lopes EP, Albuquerque MF, Markman-Filho B, et al. Occurrence of hepatopulmonary syndrome in patients with cirrhosis who are candidates for liver transplantation. J Bras Pneumol. 2010;36:432–440.

    PubMed  Google Scholar 

  15. Boryczka G, Musialik J, Rudzki K, Nalewajka-Kolodziejczak J, et al. Assessment of prevalence of intrapulmonary arterio-venous shunt in cirrhotic patients qualified to liver transplantation. Wiad Lek. 2009;62:211–218.

    PubMed  Google Scholar 

  16. De BK, Sen S, Sanyal R. Hepatopulmonary syndrome in noncirrhotic portal hypertension. Ann Intern Med. 2000;132:924.

    CAS  PubMed  Google Scholar 

  17. Regev A, Yeshurun M, Rodriguez M, Sagie A, et al. Transient hepatopulmonary syndrome in a patient with acute hepatitis A. J Viral Hepat. 2001;8:83–86.

    CAS  PubMed  Google Scholar 

  18. Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD. Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol. 2002;34:391–394.

    PubMed  Google Scholar 

  19. Tercier S, Delarue A, Rouault F, Roman C, et al. Congenital portocaval fistula associated with hepatopulmonary syndrome: ligation vs liver transplantation. J Pediatr Surg. 2006;41:e1–e3.

    PubMed  Google Scholar 

  20. Schenk P, Fuhrmann V, Madl C, Funk G, et al. Hepatopulmonary syndrome: prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences. Gut. 2002;51:853–859.

    CAS  PubMed Central  PubMed  Google Scholar 

  21. Abrams GA, Jaffe CC, Hoffer PB, Binder HJ, Fallon MB. Diagnostic utility of contrast echocardiography and lung perfusion scan in patients with hepatopulmonary syndrome. Gastroenterology. 1995;109:1283–1288.

    CAS  PubMed  Google Scholar 

  22. Schraufnagel DE, Malik R, Goel V, Ohara N, Chang SW. Lung capillary changes in hepatic cirrhosis in rats. Am J Physiol. 1997;272:L139–L147.

    CAS  PubMed  Google Scholar 

  23. Schraufnagel DE, Kay JM. Structural and pathologic changes in the lung vasculature in chronic liver disease. Clin Chest Med. 1996;17:1–15.

    CAS  PubMed  Google Scholar 

  24. Robert V, Chabot F, Vial B, Guiot P, et al. Hepatopulmonary syndrome: physiopathology of impaired gas exchange. Rev Mal Respir. 1999;16:769–779.

    CAS  PubMed  Google Scholar 

  25. Gomez FP, Martinez-Palli G, Barbera JA, Roca J, et al. Gas exchange mechanism of orthodeoxia in hepatopulmonary syndrome. Hepatology. 2004;40:660–666.

    PubMed  Google Scholar 

  26. Katsuta Y, Honma H, Zhang XJ, Ohsuga M, et al. Pulmonary blood transit time and impaired arterial oxygenation in patients with chronic liver disease. J Gastroenterol. 2005;40:57–63.

    CAS  PubMed  Google Scholar 

  27. Castaing Y, Manier G. Hemodynamic disturbances and VA/Q matching in hypoxemic cirrhotic patients. Chest. 1989;96:1064–1069.

    CAS  PubMed  Google Scholar 

  28. Fallon MB. Mechanisms of pulmonary vascular complications of liver disease: hepatopulmonary syndrome. J Clin Gastroenterol. 2005;39:S138–S142.

    CAS  PubMed  Google Scholar 

  29. Fallon MB, Zhang J. The lung in liver disease: old problem, new concepts. Trans Am Clin Climatol Assoc. 2013;124:250–262.

    PubMed Central  PubMed  Google Scholar 

  30. Melo-Silva CA, Gaio E, Trevizoli JE, Souza CS, et al. Respiratory mechanics and lung tissue remodeling in a hepatopulmonary syndrome rat model. Respir Physiol Neurobiol. 2011;179:326–333.

    PubMed  Google Scholar 

  31. Chabot F, Mestiri H, Sabry S, Dall’Ava-Santucci J, et al. Role of NO in the pulmonary artery hyporeactivity to phenylephrine in experimental biliary cirrhosis. Eur Respir J. 1996;9:560–564.

    CAS  PubMed  Google Scholar 

  32. Fallon MB, Abrams GA, Luo B, Hou Z, et al. The role of endothelial nitric oxide synthase in the pathogenesis of a rat model of hepatopulmonary syndrome. Gastroenterology. 1997;113:606–614.

    CAS  PubMed  Google Scholar 

  33. Nunes H, Lebrec D, Mazmanian M, Capron F, et al. Role of nitric oxide in hepatopulmonary syndrome in cirrhotic rats. Am J Respir Crit Care Med. 2001;164:879–885.

    CAS  PubMed  Google Scholar 

  34. Rolla G. Hepatopulmonary syndrome: role of nitric oxide and clinical aspects. Dig Liver Dis. 2004;36:303–308.

    CAS  PubMed  Google Scholar 

  35. Fallon MB, Abrams GA, McGrath JW, Hou Z, Luo B. Common bile duct ligation in the rat: a model of intrapulmonary vasodilatation and hepatopulmonary syndrome. Am J Physiol. 1997;272:G779–G784.

    CAS  PubMed  Google Scholar 

  36. Ling Y, Zhang J, Luo B, Song D, et al. The role of endothelin-1 and the endothelin B receptor in the pathogenesis of hepatopulmonary syndrome in the rat. Hepatology. 2004;39:1593–1602.

    CAS  PubMed  Google Scholar 

  37. Zhang J, Ling Y, Tang L, Luo B, et al. Attenuation of experimental hepatopulmonary syndrome in endothelin B receptor-deficient rats. Am J Physiol Gastrointest Liver Physiol. 2009;296:G704–G708.

    CAS  PubMed Central  PubMed  Google Scholar 

  38. Luo B, Tang L, Wang Z, Zhang J, et al. Cholangiocyte endothelin 1 and transforming growth factor beta1 production in rat experimental hepatopulmonary syndrome. Gastroenterology. 2005;129:682–695.

    CAS  PubMed Central  PubMed  Google Scholar 

  39. Vettukattil JJ, Stumper O. Endothelin-1 in the rat bile duct ligation model of hepatopulmonary syndrome: correlation with pulmonary dysfunction. J Hepatol. 1999;31:192–193.

    CAS  PubMed  Google Scholar 

  40. Koch DG, Bogatkevich G, Ramshesh V, Lemasters JJ, et al. Elevated levels of endothelin-1 in hepatic venous blood are associated with intrapulmonary vasodilatation in humans. Dig Dis Sci. 2012;57:516–523.

    CAS  PubMed  Google Scholar 

  41. Luo B, Liu L, Tang L, Zhang J, et al. 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–G303.

    CAS  PubMed  Google Scholar 

  42. Schroeder RA, Ewing CA, Sitzmann JV, Kuo PC. Pulmonary expression of iNOS and HO-1 protein is upregulated in a rat model of prehepatic portal hypertension. Dig Dis Sci. 2000;45:2405–2410.

    CAS  PubMed  Google Scholar 

  43. Rolla G, Brussino L, Colagrande P, Dutto L, et al. Exhaled nitric oxide and oxygenation abnormalities in hepatic cirrhosis. Hepatology. 1997;26:842–847.

    CAS  PubMed  Google Scholar 

  44. Rolla G, Brussino L, Colagrande P, Scappaticci E, et al. Exhaled nitric oxide and impaired oxygenation in cirrhotic patients before and after liver transplantation. Ann Intern Med. 1998;129:375–378.

    CAS  PubMed  Google Scholar 

  45. Schenk P, Madl C, Rezaie-Majd S, Lehr S, Muller C. Methylene blue improves the hepatopulmonary syndrome. Ann Intern Med. 2000;133:701–706.

    CAS  PubMed  Google Scholar 

  46. Fallon MB. Methylene blue and cirrhosis: pathophysiologic insights, therapeutic dilemmas. Ann Intern Med. 2000;133:738–740.

    CAS  PubMed  Google Scholar 

  47. Miyamoto A, Katsuta Y, Zhang XJ, Li HL, et al. Effect of chronic methylene blue administration on hypoxemia in rats with common bile duct ligation. Hepatol Res. 2010;40:622–632.

    CAS  PubMed  Google Scholar 

  48. Maniscalco M, Sofia M, Higenbottam T. Effects of an NO-synthase inhibitor l-NMMA in the hepatopulmonary syndrome. Respiration. 2001;68:226.

    CAS  PubMed  Google Scholar 

  49. Guo SB, Duan ZJ, Li Q, Sun XY. Effects of heme oxygenase-1 on pulmonary function and structure in rats with liver cirrhosis. Chin Med J (Engl). 2011;124:918–922.

    CAS  Google Scholar 

  50. Carter EP, Hartsfield CL, Miyazono M, Jakkula M, et al. Regulation of heme oxygenase-1 by nitric oxide during hepatopulmonary syndrome. Am J Physiol Lung Cell Mol Physiol. 2002;283:L346–L353.

    CAS  PubMed  Google Scholar 

  51. Arguedas MR, Drake BB, Kapoor A, Fallon MB. Carboxyhemoglobin levels in cirrhotic patients with and without hepatopulmonary syndrome. Gastroenterology. 2005;128:328–333.

    CAS  PubMed  Google Scholar 

  52. Zhang J, Ling Y, Luo B, Tang L, et al. Analysis of pulmonary heme oxygenase-1 and nitric oxide synthase alterations in experimental hepatopulmonary syndrome. Gastroenterology. 2003;125:1441–1451.

    CAS  PubMed  Google Scholar 

  53. Zhang HY, Han DW, Su AR, Zhang LT, 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;13:6385–6395.

    CAS  PubMed Central  PubMed  Google Scholar 

  54. Zhang HY, Han DW, Wang XG, Zhao YC, et al. Experimental study on the role of endotoxin in the development of hepatopulmonary syndrome. World J Gastroenterol. 2005;11:567–572.

    CAS  PubMed Central  PubMed  Google Scholar 

  55. Sztrymf B, Libert JM, Mougeot C, Lebrec D, et al. Cirrhotic rats with bacterial translocation have higher incidence and severity of hepatopulmonary syndrome. J Gastroenterol Hepatol. 2005;20:1538–1544.

    CAS  PubMed  Google Scholar 

  56. Rabiller A, Nunes H, Lebrec D, Tazi KA, et al. Prevention of gram-negative translocation reduces the severity of hepatopulmonary syndrome. Am J Respir Crit Care Med. 2002;166:514–517.

    PubMed  Google Scholar 

  57. Zhang J, Ling Y, Tang L, Luo B, et al. Pentoxifylline attenuation of experimental hepatopulmonary syndrome. J Appl Physiol (1985). 2007;102:949–955.

    CAS  Google Scholar 

  58. Gupta LB, Kumar A, Jaiswal AK, Yusuf J, et al. Pentoxifylline therapy for hepatopulmonary syndrome: a pilot study. Arch Intern Med. 2008;168:1820–1823.

    PubMed  Google Scholar 

  59. Zhang J, Yang W, Luo B, Hu B, et al. The role of CX(3)CL1/CX(3)CR1 in pulmonary angiogenesis and intravascular monocyte accumulation in rat experimental hepatopulmonary syndrome. J Hepatol. 2012;57:752–758.

    CAS  PubMed Central  PubMed  Google Scholar 

  60. Berthelot P, Walker JG, Sherlock S, Reid L. Arterial changes in the lungs in cirrhosis of the liver—lung spider nevi. N Engl J Med. 1966;274:291–298.

    CAS  PubMed  Google Scholar 

  61. Thenappan T, Goel A, Marsboom G, Fang YH, et al. A central role for CD68(+) macrophages in hepatopulmonary syndrome. Reversal by macrophage depletion. Am J Respir Crit Care Med. 2011;183:1080–1091.

    PubMed Central  PubMed  Google Scholar 

  62. Gomez FP, Barbera JA, Roca J, Burgos F, et al. Effects of nebulized N(G)-nitro-l-arginine methyl ester in patients with hepatopulmonary syndrome. Hepatology. 2006;43:1084–1091.

    CAS  PubMed  Google Scholar 

  63. Philit F, Wiesendanger T, Gille D, Boillot O, Cordier JF. Late resolution of hepatopulmonary syndrome after liver transplantation. Respiration. 1997;64:173–175.

    CAS  PubMed  Google Scholar 

  64. Zhang J, Luo B, Tang L, Wang Y, et al. Pulmonary angiogenesis in a rat model of hepatopulmonary syndrome. Gastroenterology. 2009;136:1070–1080.

    CAS  PubMed Central  PubMed  Google Scholar 

  65. Anand AC, Mukherjee D, Rao KS, Seth AK. Hepatopulmonary syndrome: prevalence and clinical profile. Indian J Gastroenterol. 2001;20:24–27.

    CAS  PubMed  Google Scholar 

  66. Mohammad AA, Fatemi SR, Mirzaee V, Khoshbaten M, et al. Clinical features of hepatopulmonary syndrome in cirrhotic patients. World J Gastroenterol. 2006;12:1954–1956.

    Google Scholar 

  67. Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatology. 2005;41:1122–1129.

    PubMed  Google Scholar 

  68. Palma DT, Philips GM, Arguedas MR, Harding SM, Fallon MB. Oxygen desaturation during sleep in hepatopulmonary syndrome. Hepatology. 2008;47:1257–1263.

    PubMed  Google Scholar 

  69. Isaac J. Hypoxaemia, platypnoea, orthodeoxia and right-to-left shunts. Br J Anaesth. 2001;86:596–597.

    CAS  PubMed  Google Scholar 

  70. Goossens N, Joshi D, O’Grady J. Image of the month. Digital clubbing in association with hepatopulmonary syndrome. Hepatology. 2011;53:365–366.

    PubMed  Google Scholar 

  71. Hira HS, Kumar J, Tyagi SK, Jain SK. A study of hepatopulmonary syndrome among patients of cirrhosis of liver and portal hypertension. Indian J Chest Dis Allied Sci. 2003;45:165–171.

    CAS  PubMed  Google Scholar 

  72. Silverio AO, Guimaraes DC, Elias LF, Milanez EO, Naves S. Are the spider angiomas skin markers of hepatopulmonary syndrome? Arq Gastroenterol. 2013;50:175–179.

    Google Scholar 

  73. Horvatits T, Drolz A, Roedl K, Herkner H, et al. Von Willebrand factor antigen for detection of hepatopulmonary syndrome in patients with cirrhosis. J Hepatol. 2014;61:544–549.

    CAS  PubMed  Google Scholar 

  74. Abrams GA, Sanders MK, Fallon MB. Utility of pulse oximetry in the detection of arterial hypoxemia in liver transplant candidates. Liver Transpl. 2002;8:391–396.

    PubMed  Google Scholar 

  75. Deibert P, Allgaier HP, Loesch S, Muller C, et al. Hepatopulmonary syndrome in patients with chronic liver disease: role of pulse oximetry. BMC Gastroenterol. 2006;6:15.

    PubMed Central  PubMed  Google Scholar 

  76. Arguedas MR, Singh H, Faulk DK, Fallon MB. Utility of pulse oximetry screening for hepatopulmonary syndrome. Clin Gastroenterol Hepatol. 2007;5:749–754.

    PubMed  Google Scholar 

  77. Kochar R, Tanikella R, Fallon MB. Serial pulse oximetry in hepatopulmonary syndrome. Dig Dis Sci. 2011;56:1862–1868.

    PubMed  Google Scholar 

  78. Voiosu A, Voiosu T, Stanescu CM, Chirila L, et al. Novel predictors of intrapulmonary vascular dilatations in cirrhosis: extending the role of pulse oximetry and echocardiography. Acta Gastroenterol Belg. 2013;76:241–245.

    CAS  PubMed  Google Scholar 

  79. Krowka MJ. Hepatopulmonary syndrome: monitoring at your fingertip. Dig Dis Sci. 2011;56:1599–1600.

    PubMed  Google Scholar 

  80. Hoerning A, Raub S, Neudorf U, Muntjes C, et al. Pulse oximetry is insufficient for timely diagnosis of hepatopulmonary syndrome in children with liver cirrhosis. J Pediatr. 2014;164:546–552.

    PubMed  Google Scholar 

  81. Palma DT, Fallon MB. The hepatopulmonary syndrome. J Hepatol. 2006;45:617–625.

    PubMed  Google Scholar 

  82. Rodriguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome—a liver-induced lung vascular disorder. N Engl J Med. 2008;358:2378–2387.

    CAS  PubMed  Google Scholar 

  83. Khan AN, Al-Jahdali H, Abdullah K, Irion KL, et al. Pulmonary vascular complications of chronic liver disease: pathophysiology, imaging, and treatment. Ann Thorac Med. 2011;6:57–65.

    PubMed Central  PubMed  Google Scholar 

  84. Zhang J, Fallon MB. Hepatopulmonary syndrome: update on pathogenesis and clinical features. Nat Rev Gastroenterol Hepatol. 2012;9:539–549.

    CAS  PubMed  Google Scholar 

  85. Porres-Aguilar M, Altamirano JT, Torre-Delgadillo A, Charlton MR, Duarte-Rojo A. Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview. Eur Respir Rev. 2012;21:223–233.

    PubMed  Google Scholar 

  86. Roberts DN, Arguedas MR, Fallon MB. Cost-effectiveness of screening for hepatopulmonary syndrome in liver transplant candidates. Liver Transpl. 2007;13:206–214.

    PubMed  Google Scholar 

  87. Suceveanu AI, Mazilu L, Tomescu D, Ciufu N, et al. Screening of hepatopulmonary syndrome (HPS) with CEUS and pulse-oximetry in liver cirrhosis patients eligible for liver transplant. Chirurgia (Bucur). 2013;108:684–688.

    CAS  Google Scholar 

  88. Ho V. Current concepts in the management of hepatopulmonary syndrome. Vasc Health Risk Manag. 2008;4:1035–1041.

    CAS  PubMed Central  PubMed  Google Scholar 

  89. Rollan MJ, Munoz AC, Perez T, Bratos JL. Value of contrast echocardiography for the diagnosis of hepatopulmonary syndrome. Eur J Echocardiogr. 2007;8:408–410.

    PubMed  Google Scholar 

  90. Mimidis KP, Vassilakos PI, Mastorakou AN, Spiropoulos KV, et al. Evaluation of contrast echocardiography and lung perfusion scan in detecting intrapulmonary vascular dilatation in normoxemic patients with early liver cirrhosis. Hepatogastroenterology. 1998;45:2303–2307.

    CAS  PubMed  Google Scholar 

  91. Aller R, Moya JL, Moreira V, Garcia-Lledo A, et al. Diagnosis and grading of intrapulmonary vascular dilatation in cirrhotic patients with contrast transesophageal echocardiography. J Hepatol. 1999;31:1044–1052.

    CAS  PubMed  Google Scholar 

  92. Aller R, Moya JL, Moreira V, Boixeda D, et al. Diagnosis of hepatopulmonary syndrome with contrast transesophageal echocardiography: advantages over contrast transthoracic echocardiography. Dig Dis Sci. 1999;44:1243–1248.

    CAS  PubMed  Google Scholar 

  93. Pacca R, Maddukuri P, Pandian NG, Kuvin JT. Echocardiographic detection of intrapulmonary shunting in a patient with hepatopulmonary syndrome: case report and review of the literature. Echocardiography. 2006;23:56–59.

    PubMed  Google Scholar 

  94. Fischer CH, Campos O, Fernandes WB, Kondo M, et al. Role of contrast-enhanced transesophageal echocardiography for detection of and scoring intrapulmonary vascular dilatation. Echocardiography. 2010;27:1233–1237.

    PubMed  Google Scholar 

  95. Vedrinne JM, Duperret S, Bizollon T, Magnin C, et al. Comparison of transesophageal and transthoracic contrast echocardiography for detection of an intrapulmonary shunt in liver disease. Chest. 1997;111:1236–1240.

    CAS  PubMed  Google Scholar 

  96. Lenci I, Alvior A, Manzia TM, Toti L, et al. Saline contrast echocardiography in patients with hepatopulmonary syndrome awaiting liver transplantation. J Am Soc Echocardiogr. 2009;22:89–94.

    PubMed  Google Scholar 

  97. Gaber R, Ziada DH, Kotb NA, Abo EG, Hamisa M. Detection of hepatopulmonary syndrome in patients with liver cirrhosis using 3D contrast echocardiography. Arab J Gastroenterol. 2012;13:14–19.

    PubMed  Google Scholar 

  98. Abrams GA, Nanda NC, Dubovsky EV, Krowka MJ, Fallon MB. Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome: a new approach. Gastroenterology. 1998;114:305–310.

    CAS  PubMed  Google Scholar 

  99. Nishiyama Y, Yamamoto Y, Satoh K, Ohkawa M, et al. Tc-99 m MAA perfusion lung scanning in hepatopulmonary syndrome. Clin Nucl Med. 1999;24:616–617.

    CAS  PubMed  Google Scholar 

  100. Hosono M, Machida K, Honda N, Takahashi T, et al. Quantitative lung perfusion scintigraphy and detection of intrapulmonary shunt in liver cirrhosis. Ann Nucl Med. 2002;16:577–581.

    PubMed  Google Scholar 

  101. Izquierdo V, Gomez D, Macias E, Siurana R, et al. Hepatopulmonary syndrome and radionuclide lung perfusion imaging. Clin Nucl Med. 1998;23:550–551.

    CAS  PubMed  Google Scholar 

  102. El-Shabrawi MH, Omran S, Wageeh S, Isa M, et al. (99 M)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;22:1006–1012.

    CAS  PubMed  Google Scholar 

  103. Krowka MJ, Dickson ER, Cortese DA. Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue. Chest. 1993;104:515–521.

    CAS  PubMed  Google Scholar 

  104. Ryu JK, Oh JH. Hepatopulmonary syndrome: angiography and therapeutic embolization. Clin Imaging. 2003;27:97–100.

    PubMed  Google Scholar 

  105. Saad NE, Lee DE, Waldman DL, Saad WE. Pulmonary arterial coil embolization for the management of persistent type I hepatopulmonary syndrome after liver transplantation. J Vasc Interv Radiol. 2007;18:1576–1580.

    PubMed  Google Scholar 

  106. Alonso MJ, Zozaya UJ, Gutierrez DJ, Abinzano GM, et al. Prognostic significance of the hepatopulmonary syndrome in liver cirrhosis. Med Clin (Barc). 2006;127:133–135.

    Google Scholar 

  107. Arguedas MR, Abrams GA, Krowka MJ, Fallon MB. Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation. Hepatology. 2003;37:192–197.

    PubMed  Google Scholar 

  108. Fallon MB, Krowka MJ, Brown RS, Trotter JF, et al. Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterology. 2008;135:1168–1175.

    PubMed Central  PubMed  Google Scholar 

  109. Yi HM, Wang GS, Yi SH, Yang Y, et al. Prospective evaluation of postoperative outcome after liver transplantation in hepatopulmonary syndrome patients. Chin Med J (Engl). 2009;122:2598–2602.

    Google Scholar 

  110. Kim HY, Choi MS, Lee SC, Park SW, et al. Outcomes in patients with hepatopulmonary syndrome undergoing liver transplantation. Transplant Proc. 2004;36:2762–2763.

    CAS  PubMed  Google Scholar 

  111. Schiffer E, Majno P, Mentha G, Giostra E, et al. Hepatopulmonary syndrome increases the postoperative mortality rate following liver transplantation: a prospective study in 90 patients. Am J Transplant. 2006;6:1430–1437.

    CAS  PubMed  Google Scholar 

  112. Deberaldini M, Arcanjo AB, Melo E, Da SR, et al. Hepatopulmonary syndrome: morbidity and survival after liver transplantation. Transplant Proc. 2008;40:3512–3516.

    CAS  PubMed  Google Scholar 

  113. Gupta S, Castel H, Rao RV, Picard M, et al. Improved survival after liver transplantation in patients with hepatopulmonary syndrome. Am J Transplant. 2010;10:354–363.

    CAS  PubMed  Google Scholar 

  114. Saigal S, Choudhary N, Saraf N, Kotecha H, et al. Excellent outcome of living donor liver transplantation in patients with hepatopulmonary syndrome: a single centre experience. Clin Transplant. 2013;27:530–534.

    PubMed  Google Scholar 

  115. Iyer VN, Swanson KL, Cartin-Ceba R, Dierkhising RA, et al. Hepatopulmonary syndrome: favorable outcomes in the MELD exception era. Hepatology. 2013;57:2427–2435.

    PubMed  Google Scholar 

  116. Eriksson LS, Soderman C, Ericzon BG, Eleborg L, et al. Normalization of ventilation/perfusion relationships after liver transplantation in patients with decompensated cirrhosis: evidence for a hepatopulmonary syndrome. Hepatology. 1990;12:1350–1357.

    CAS  PubMed  Google Scholar 

  117. Houlihan DD, Holt A, Elliot C, Ferguson JW. Review article: liver transplantation for the pulmonary disorders of portal hypertension. Aliment Pharmacol Ther. 2013;37:183–194.

    CAS  PubMed  Google Scholar 

  118. Carey EJ, Douglas DD, Balan V, Vargas HE, et al. Hepatopulmonary syndrome after living donor liver transplantation and deceased donor liver transplantation: a single-center experience. Liver Transpl. 2004;10:529–533.

    PubMed  Google Scholar 

  119. Chen K, Li B. Reversal of severe hepatopulmonary syndrome in chronic hepatic cirrhosis by living donor liver transplantation: report of two cases. Surg Today. 2011;41:441–443.

    PubMed  Google Scholar 

  120. Urahashi T, Mizuta K, Sanada Y, Umehara M, et al. Pediatric living donor liver transplantation for biliary atresia with hepatopulmonary syndrome: the gift of a second wind. Pediatr Surg Int. 2011;27:817–821.

    PubMed  Google Scholar 

  121. Motomura T, Ikegami T, Mano Y, Nagata S, et al. Living donor liver transplantation for end-stage liver disease with severe hepatopulmonary syndrome: report of a case. Surg Today. 2011;41:436–440.

    PubMed  Google Scholar 

  122. Jounieaux V, Leleu O, Mayeux I. Cardiopulmonary effects of nitric oxide inhalation and methylene blue injection in hepatopulmonary syndrome. Intensive Care Med. 2001;27:1103–1104.

    CAS  PubMed  Google Scholar 

  123. Soderman C, Juhlin-Dannfelt A, Lagerstrand L, Eriksson LS. Ventilation-perfusion relationships and central haemodynamics in patients with cirrhosis. Effects of a somatostatin analogue. J Hepatol. 1994;21:52–57.

    CAS  PubMed  Google Scholar 

  124. Song JY, Choi JY, Ko JT, Bae EJ, et al. Long-term aspirin therapy for hepatopulmonary syndrome. Pediatrics. 1996;97:917–920.

    CAS  PubMed  Google Scholar 

  125. Anel RM, Sheagren JN. Novel presentation and approach to management of hepatopulmonary syndrome with use of antimicrobial agents. Clin Infect Dis. 2001;32:E131–E136.

    CAS  PubMed  Google Scholar 

  126. Gupta S, Faughnan ME, Lilly L, Hutchison S, et al. Norfloxacin therapy for hepatopulmonary syndrome: a pilot randomized controlled trial. Clin Gastroenterol Hepatol. 2010;8:1095–1098.

    CAS  PubMed  Google Scholar 

  127. Tieppo J, Vercelino R, Dias AS, Silva VM, et al. Evaluation of the protective effects of quercetin in the hepatopulmonary syndrome. Food Chem Toxicol. 2007;45:1140–1146.

    CAS  PubMed  Google Scholar 

  128. Tieppo J, Cuevas MJ, Vercelino R, Tunon MJ, et al. Quercetin administration ameliorates pulmonary complications of cirrhosis in rats. J Nutr. 2009;139:1339–1346.

    CAS  PubMed  Google Scholar 

  129. Moreira SH, Reis G, Guedes M, Cleto E, et al. A case of hepatopulmonary syndrome solved by mycophenolate mofetil (an inhibitor of angiogenesis and nitric oxide production). J Hepatol. 2013;58:630–633.

    Google Scholar 

  130. Altschuler EL, Kast RE. Paroxetine for hepatopulmonary syndrome? Med Hypotheses. 2004;62:446–447.

    CAS  PubMed  Google Scholar 

  131. Yilmaz S, Dursum M, Canoruc F, Bayan K, et al. A severe (type II) hepatopulmonary syndrome in a patient with idiopathic portal hypertension and treatment with paroxetine. Neth J Med. 2005;63:448–452.

    CAS  PubMed  Google Scholar 

  132. Sztrymf B, Rabiller A, Nunes H, Savale L, et al. Prevention of hepatopulmonary syndrome and hyperdynamic state by pentoxifylline in cirrhotic rats. Eur Respir J. 2004;23:752–758.

    CAS  PubMed  Google Scholar 

  133. Tanikella R, Philips GM, Faulk DK, Kawut SM, Fallon MB. Pilot study of pentoxifylline in hepatopulmonary syndrome. Liver Transpl. 2008;14:1199–1203.

    PubMed Central  PubMed  Google Scholar 

  134. Kianifar HR, Khalesi M, Mahmoodi E, Afzal AM. Pentoxifylline in hepatopulmonary syndrome. World J Gastroenterol. 2012;18:4912–4916.

    CAS  PubMed Central  PubMed  Google Scholar 

  135. Rolla G, Bucca C, Brussino L. Methylene blue in the hepatopulmonary syndrome. N Engl J Med. 1994;331:1098.

    CAS  PubMed  Google Scholar 

  136. Groneberg DA, Fischer A. Methylene blue improves the hepatopulmonary syndrome. Ann Intern Med. 2001;135:380–381.

    CAS  PubMed  Google Scholar 

  137. Roma J, Balbi E, Pacheco-Moreira L, Gonzalez AC, et al. Methylene blue used as a bridge to liver transplantation postoperative recovery: a case report. Transplant Proc. 2010;42:601–604.

    CAS  PubMed  Google Scholar 

  138. Caldwell SH, Jeffers LJ, Narula OS, Lang EA, et al. Ancient remedies revisited: does Allium sativum (garlic) palliate the hepatopulmonary syndrome? J Clin Gastroenterol. 1992;15:248–250.

    CAS  PubMed  Google Scholar 

  139. Abrams GA, Fallon MB. Treatment of hepatopulmonary syndrome with Allium sativum L. (Garlic): a pilot trial. J Clin Gastroenterol. 1998;27:232–235.

    CAS  PubMed  Google Scholar 

  140. De BK, Dutta D, Pal SK, Gangopadhyay S, et al. The role of garlic in hepatopulmonary syndrome: a randomized controlled trial. Can J Gastroenterol. 2010;24:183–188.

    PubMed Central  PubMed  Google Scholar 

  141. Selim KM, Akriviadis EA, Zuckerman E, Chen D, Reynolds TB. Transjugular intrahepatic portosystemic shunt: a successful treatment for hepatopulmonary syndrome. Am J Gastroenterol. 1998;93:455–458.

    CAS  PubMed  Google Scholar 

  142. Lasch HM, Fried MW, Zacks SL, Odell P, et al. Use of transjugular intrahepatic portosystemic shunt as a bridge to liver transplantation in a patient with severe hepatopulmonary syndrome. Liver Transpl. 2001;7:147–149.

    CAS  PubMed  Google Scholar 

  143. Paramesh AS, Husain SZ, Shneider B, Guller J, et al. Improvement of hepatopulmonary syndrome after transjugular intrahepatic portasystemic shunting: case report and review of literature. Pediatr Transplant. 2003;7:157–162.

    PubMed  Google Scholar 

  144. Chevallier P, Novelli L, Motamedi JP, Hastier P, et al. Hepatopulmonary syndrome successfully treated with transjugular intrahepatic portosystemic shunt: a three-year follow-up. J Vasc Interv Radiol. 2004;15:647–648.

    PubMed  Google Scholar 

  145. Krowka MJ. Management of pulmonary complications in pretransplant patients. Clin Liver Dis. 2011;15:765–777.

    PubMed  Google Scholar 

  146. Martinez-Palli G, Drake BB, Garcia-Pagan JC, Barbera JA, et al. Effect of transjugular intrahepatic portosystemic shunt on pulmonary gas exchange in patients with portal hypertension and hepatopulmonary syndrome. World J Gastroenterol. 2005;11:6858–6862.

    PubMed  Google Scholar 

  147. Boyer TD, Haskal ZJ. The Role of Transjugular Intrahepatic Portosystemic Shunt (TIPS) in the Management of Portal Hypertension: update 2009. Hepatology. 2010;51:306.

    PubMed  Google Scholar 

  148. Yoshimi M, Takayama K, Aizawa H, Inoue H, et al. Pulmonary arterio-venous fistula treated by embolization with steel coils. Nihon Kyobu Shikkan Gakkai Zasshi. 1996;34:569–574.

    CAS  PubMed  Google Scholar 

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  1. Department of Liver Disease, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi’an, China

    Yong Lv

  2. State Key Laboratory of Cancer Biology, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, 15 West Changle Road, Xi’an, 710032, China

    Daiming Fan

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Lv, Y., Fan, D. Hepatopulmonary Syndrome. Dig Dis Sci 60, 1914–1923 (2015). https://doi.org/10.1007/s10620-015-3593-0

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