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Digestive Diseases and Sciences

, Volume 60, Issue 7, pp 1914–1923 | Cite as

Hepatopulmonary Syndrome

  • Yong Lv
  • Daiming FanEmail author
Review

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.

Keywords

Liver disease Hepatopulmonary syndrome Intrapulmonary vascular dilatation Liver transplantation 

Notes

Conflict of interest

None.

References

  1. 1.
    Ratti L, Pozzi M. The pulmonary involvement in portal hypertension: portopulmonary hypertension and hepatopulmonary syndrome. Gastroenterol Hepatol. 2006;29:40–50.PubMedGoogle Scholar
  2. 2.
    Machicao VI, Balakrishnan M, Fallon MB. Pulmonary complications in chronic liver disease. Hepatology. 2014;59:1627–1637.PubMedGoogle Scholar
  3. 3.
    Fritz JS, Fallon MB, Kawut SM. Pulmonary vascular complications of liver disease. Am J Respir Crit Care Med. 2013;187:133–143.PubMedCentralPubMedGoogle Scholar
  4. 4.
    Ioachimescu OC, Mehta AC, Stoller JK. Hepatopulmonary syndrome following portopulmonary hypertension. Eur Respir J. 2007;29:1277–1280.PubMedGoogle Scholar
  5. 5.
    Martinez GP, Barbera JA, Visa J, Rimola A, et al. Hepatopulmonary syndrome in candidates for liver transplantation. J Hepatol. 2001;34:651–657.PubMedGoogle Scholar
  6. 6.
    Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004;363:1461–1468.PubMedGoogle Scholar
  7. 7.
    Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB. Pulmonary–hepatic vascular Disorders (PHD). Eur Respir J. 2004;24:861–880.PubMedGoogle Scholar
  8. 8.
    Rodriguez-Roisin R, Agusti AG, Roca J. The hepatopulmonary syndrome: new name, old complexities. Thorax. 1992;47:897–902.PubMedCentralPubMedGoogle Scholar
  9. 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.PubMedGoogle Scholar
  10. 10.
    Noli K, Solomon M, Golding F, Charron M, Ling SC. Prevalence of hepatopulmonary syndrome in children. Pediatrics. 2008;121:e522–e527.PubMedGoogle Scholar
  11. 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.PubMedGoogle Scholar
  12. 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.PubMedGoogle Scholar
  13. 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.PubMedGoogle Scholar
  14. 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.PubMedGoogle Scholar
  15. 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.PubMedGoogle Scholar
  16. 16.
    De BK, Sen S, Sanyal R. Hepatopulmonary syndrome in noncirrhotic portal hypertension. Ann Intern Med. 2000;132:924.PubMedGoogle Scholar
  17. 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.PubMedGoogle Scholar
  18. 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.PubMedGoogle Scholar
  19. 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.PubMedGoogle Scholar
  20. 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.PubMedCentralPubMedGoogle Scholar
  21. 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.PubMedGoogle Scholar
  22. 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.PubMedGoogle Scholar
  23. 23.
    Schraufnagel DE, Kay JM. Structural and pathologic changes in the lung vasculature in chronic liver disease. Clin Chest Med. 1996;17:1–15.PubMedGoogle Scholar
  24. 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.PubMedGoogle Scholar
  25. 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.PubMedGoogle Scholar
  26. 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.PubMedGoogle Scholar
  27. 27.
    Castaing Y, Manier G. Hemodynamic disturbances and VA/Q matching in hypoxemic cirrhotic patients. Chest. 1989;96:1064–1069.PubMedGoogle Scholar
  28. 28.
    Fallon MB. Mechanisms of pulmonary vascular complications of liver disease: hepatopulmonary syndrome. J Clin Gastroenterol. 2005;39:S138–S142.PubMedGoogle Scholar
  29. 29.
    Fallon MB, Zhang J. The lung in liver disease: old problem, new concepts. Trans Am Clin Climatol Assoc. 2013;124:250–262.PubMedCentralPubMedGoogle Scholar
  30. 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.PubMedGoogle Scholar
  31. 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.PubMedGoogle Scholar
  32. 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.PubMedGoogle Scholar
  33. 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.PubMedGoogle Scholar
  34. 34.
    Rolla G. Hepatopulmonary syndrome: role of nitric oxide and clinical aspects. Dig Liver Dis. 2004;36:303–308.PubMedGoogle Scholar
  35. 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.PubMedGoogle Scholar
  36. 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.PubMedGoogle Scholar
  37. 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.PubMedCentralPubMedGoogle Scholar
  38. 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.PubMedCentralPubMedGoogle Scholar
  39. 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.PubMedGoogle Scholar
  40. 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.PubMedGoogle Scholar
  41. 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.PubMedGoogle Scholar
  42. 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.PubMedGoogle Scholar
  43. 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.PubMedGoogle Scholar
  44. 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.PubMedGoogle Scholar
  45. 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.PubMedGoogle Scholar
  46. 46.
    Fallon MB. Methylene blue and cirrhosis: pathophysiologic insights, therapeutic dilemmas. Ann Intern Med. 2000;133:738–740.PubMedGoogle Scholar
  47. 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.PubMedGoogle Scholar
  48. 48.
    Maniscalco M, Sofia M, Higenbottam T. Effects of an NO-synthase inhibitor l-NMMA in the hepatopulmonary syndrome. Respiration. 2001;68:226.PubMedGoogle Scholar
  49. 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.Google Scholar
  50. 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.PubMedGoogle Scholar
  51. 51.
    Arguedas MR, Drake BB, Kapoor A, Fallon MB. Carboxyhemoglobin levels in cirrhotic patients with and without hepatopulmonary syndrome. Gastroenterology. 2005;128:328–333.PubMedGoogle Scholar
  52. 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.PubMedGoogle Scholar
  53. 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.PubMedCentralPubMedGoogle Scholar
  54. 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.PubMedCentralPubMedGoogle Scholar
  55. 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.PubMedGoogle Scholar
  56. 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.PubMedGoogle Scholar
  57. 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.Google Scholar
  58. 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.PubMedGoogle Scholar
  59. 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.PubMedCentralPubMedGoogle Scholar
  60. 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.PubMedGoogle Scholar
  61. 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.PubMedCentralPubMedGoogle Scholar
  62. 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.PubMedGoogle Scholar
  63. 63.
    Philit F, Wiesendanger T, Gille D, Boillot O, Cordier JF. Late resolution of hepatopulmonary syndrome after liver transplantation. Respiration. 1997;64:173–175.PubMedGoogle Scholar
  64. 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.PubMedCentralPubMedGoogle Scholar
  65. 65.
    Anand AC, Mukherjee D, Rao KS, Seth AK. Hepatopulmonary syndrome: prevalence and clinical profile. Indian J Gastroenterol. 2001;20:24–27.PubMedGoogle Scholar
  66. 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. 67.
    Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatology. 2005;41:1122–1129.PubMedGoogle Scholar
  68. 68.
    Palma DT, Philips GM, Arguedas MR, Harding SM, Fallon MB. Oxygen desaturation during sleep in hepatopulmonary syndrome. Hepatology. 2008;47:1257–1263.PubMedGoogle Scholar
  69. 69.
    Isaac J. Hypoxaemia, platypnoea, orthodeoxia and right-to-left shunts. Br J Anaesth. 2001;86:596–597.PubMedGoogle Scholar
  70. 70.
    Goossens N, Joshi D, O’Grady J. Image of the month. Digital clubbing in association with hepatopulmonary syndrome. Hepatology. 2011;53:365–366.PubMedGoogle Scholar
  71. 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.PubMedGoogle Scholar
  72. 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. 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.PubMedGoogle Scholar
  74. 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.PubMedGoogle Scholar
  75. 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.PubMedCentralPubMedGoogle Scholar
  76. 76.
    Arguedas MR, Singh H, Faulk DK, Fallon MB. Utility of pulse oximetry screening for hepatopulmonary syndrome. Clin Gastroenterol Hepatol. 2007;5:749–754.PubMedGoogle Scholar
  77. 77.
    Kochar R, Tanikella R, Fallon MB. Serial pulse oximetry in hepatopulmonary syndrome. Dig Dis Sci. 2011;56:1862–1868.PubMedGoogle Scholar
  78. 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.PubMedGoogle Scholar
  79. 79.
    Krowka MJ. Hepatopulmonary syndrome: monitoring at your fingertip. Dig Dis Sci. 2011;56:1599–1600.PubMedGoogle Scholar
  80. 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.PubMedGoogle Scholar
  81. 81.
    Palma DT, Fallon MB. The hepatopulmonary syndrome. J Hepatol. 2006;45:617–625.PubMedGoogle Scholar
  82. 82.
    Rodriguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome—a liver-induced lung vascular disorder. N Engl J Med. 2008;358:2378–2387.PubMedGoogle Scholar
  83. 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.PubMedCentralPubMedGoogle Scholar
  84. 84.
    Zhang J, Fallon MB. Hepatopulmonary syndrome: update on pathogenesis and clinical features. Nat Rev Gastroenterol Hepatol. 2012;9:539–549.PubMedGoogle Scholar
  85. 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.PubMedGoogle Scholar
  86. 86.
    Roberts DN, Arguedas MR, Fallon MB. Cost-effectiveness of screening for hepatopulmonary syndrome in liver transplant candidates. Liver Transpl. 2007;13:206–214.PubMedGoogle Scholar
  87. 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.Google Scholar
  88. 88.
    Ho V. Current concepts in the management of hepatopulmonary syndrome. Vasc Health Risk Manag. 2008;4:1035–1041.PubMedCentralPubMedGoogle Scholar
  89. 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.PubMedGoogle Scholar
  90. 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.PubMedGoogle Scholar
  91. 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.PubMedGoogle Scholar
  92. 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.PubMedGoogle Scholar
  93. 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.PubMedGoogle Scholar
  94. 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.PubMedGoogle Scholar
  95. 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.PubMedGoogle Scholar
  96. 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.PubMedGoogle Scholar
  97. 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.PubMedGoogle Scholar
  98. 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.PubMedGoogle Scholar
  99. 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.PubMedGoogle Scholar
  100. 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.PubMedGoogle Scholar
  101. 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.PubMedGoogle Scholar
  102. 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.PubMedGoogle Scholar
  103. 103.
    Krowka MJ, Dickson ER, Cortese DA. Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue. Chest. 1993;104:515–521.PubMedGoogle Scholar
  104. 104.
    Ryu JK, Oh JH. Hepatopulmonary syndrome: angiography and therapeutic embolization. Clin Imaging. 2003;27:97–100.PubMedGoogle Scholar
  105. 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.PubMedGoogle Scholar
  106. 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. 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.PubMedGoogle Scholar
  108. 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.PubMedCentralPubMedGoogle Scholar
  109. 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. 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.PubMedGoogle Scholar
  111. 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.PubMedGoogle Scholar
  112. 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.PubMedGoogle Scholar
  113. 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.PubMedGoogle Scholar
  114. 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.PubMedGoogle Scholar
  115. 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.PubMedGoogle Scholar
  116. 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.PubMedGoogle Scholar
  117. 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.PubMedGoogle Scholar
  118. 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.PubMedGoogle Scholar
  119. 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.PubMedGoogle Scholar
  120. 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.PubMedGoogle Scholar
  121. 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.PubMedGoogle Scholar
  122. 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.PubMedGoogle Scholar
  123. 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.PubMedGoogle Scholar
  124. 124.
    Song JY, Choi JY, Ko JT, Bae EJ, et al. Long-term aspirin therapy for hepatopulmonary syndrome. Pediatrics. 1996;97:917–920.PubMedGoogle Scholar
  125. 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.PubMedGoogle Scholar
  126. 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.PubMedGoogle Scholar
  127. 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.PubMedGoogle Scholar
  128. 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.PubMedGoogle Scholar
  129. 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. 130.
    Altschuler EL, Kast RE. Paroxetine for hepatopulmonary syndrome? Med Hypotheses. 2004;62:446–447.PubMedGoogle Scholar
  131. 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.PubMedGoogle Scholar
  132. 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.PubMedGoogle Scholar
  133. 133.
    Tanikella R, Philips GM, Faulk DK, Kawut SM, Fallon MB. Pilot study of pentoxifylline in hepatopulmonary syndrome. Liver Transpl. 2008;14:1199–1203.PubMedCentralPubMedGoogle Scholar
  134. 134.
    Kianifar HR, Khalesi M, Mahmoodi E, Afzal AM. Pentoxifylline in hepatopulmonary syndrome. World J Gastroenterol. 2012;18:4912–4916.PubMedCentralPubMedGoogle Scholar
  135. 135.
    Rolla G, Bucca C, Brussino L. Methylene blue in the hepatopulmonary syndrome. N Engl J Med. 1994;331:1098.PubMedGoogle Scholar
  136. 136.
    Groneberg DA, Fischer A. Methylene blue improves the hepatopulmonary syndrome. Ann Intern Med. 2001;135:380–381.PubMedGoogle Scholar
  137. 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.PubMedGoogle Scholar
  138. 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.PubMedGoogle Scholar
  139. 139.
    Abrams GA, Fallon MB. Treatment of hepatopulmonary syndrome with Allium sativum L. (Garlic): a pilot trial. J Clin Gastroenterol. 1998;27:232–235.PubMedGoogle Scholar
  140. 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.PubMedCentralPubMedGoogle Scholar
  141. 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.PubMedGoogle Scholar
  142. 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.PubMedGoogle Scholar
  143. 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.PubMedGoogle Scholar
  144. 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.PubMedGoogle Scholar
  145. 145.
    Krowka MJ. Management of pulmonary complications in pretransplant patients. Clin Liver Dis. 2011;15:765–777.PubMedGoogle Scholar
  146. 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.PubMedGoogle Scholar
  147. 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.PubMedGoogle Scholar
  148. 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.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Department of Liver Disease, Xijing Hospital of Digestive DiseasesFourth Military Medical UniversityXi’anChina
  2. 2.State Key Laboratory of Cancer Biology, Xijing Hospital of Digestive DiseasesFourth Military Medical UniversityXi’anChina

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