Skip to main content
SpringerLink
Log in
Book cover

Portal Hypertension pp 455–468Cite as

Pulmonary Complications Associated with Portal Hypertension

  • Chapter

Part of the book series: Clinical Gastroenterology ((CG))

Abstract

Hepatopulmonary syndrome (HPS) is a pulmonary vascular abnormality characterized by intrapulmonary vascular dilatations (IPVD) and arterial hypoxemia that occurs in the setting of either cirrhotic or noncirrhotic portal hypertension (1–4). The clinical diagnostic triad is shown in Fig. 1. Prevalence rates for HPS in patients undergoing liver transplant evaluation have ranged from 5% to 29% (5). HPS is not age-dependent and can be seen in children; it affects males and females equally. To date, no specific etiology of liver disease has been associated with HPS and the degree of HPS has not been correlated with the severity of liver disease using the Childs-Turcotte-Pugh classification or the model for end-stage liver disease scoring system (MELD) (6).

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   169.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   299.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Kaymakoglu S, Kahraman T, Kudat H, et al. Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients. Digest Dis Sci 2003;48:556–560.

    Article  PubMed  CAS  Google Scholar 

  2. Herve P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension. Eur Respir J 1998;11:1153–1166.

    Article  PubMed  CAS  Google Scholar 

  3. Krowka MJ. Hepatopulmonary syndromes. Gut 2000;40:1–4.

    Article  Google Scholar 

  4. Lange PA, Stoller JK. The hepatopulmonary syndrome. Ann Intern Med 1995;122:521–529.

    PubMed  CAS  Google Scholar 

  5. Rodriguez-Roisin R, Roca J. Hepatopulmonary syndrome: the paradigm of liver-induced hypoxaemia. Baillieres Clin Gastroenterol 1997;11:387–406.

    Article  PubMed  CAS  Google Scholar 

  6. Wiesner RH, Edwards E, Freeman R, et al. Model for end-stage liver disease (MELD) and allocation of donor livers. Gastroenterology 2003;124:91–96.

    Article  PubMed  Google Scholar 

  7. Brussino L, Bucca C, Morello M, Scappaticci E, Mauro M, Rolla G. Effect on dyspnoea and hypoxaemia of inhaled NG-nitro-L-arginine methyl ester in hepatopulmonary syndrome. Lancet 2003;362:43–44.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  10. Fallon MB, Abrams GA. Pulmonary dysfunction in chronic liver disease. Hepatology 2000;32:859–865.

    Article  PubMed  CAS  Google Scholar 

  11. Abrams GA, Jaffe CC, Hoffer PB, et al. Diagnostic utility of contrast echocardiography and lung perfusion scan in patients with hepatopulmonary syndrome. Gastroenterology 1995;109:1283–1288.

    Article  PubMed  CAS  Google Scholar 

  12. Krowka MJ, Tajik AJ, Dickson E, et al. Intrapulmonary vascular dilatation in liver transplant candidates: screening by two-dimensional contrast enhanced echocardiography. Chest 1990;97:1165–1170.

    Article  PubMed  CAS  Google Scholar 

  13. Moller S, Hillingso J, Christensen E, Henriksen JH, et al. Arterial hypoxemia in cirrhosis: fact or fiction? Gut 1998;42:868–874.

    Article  PubMed  CAS  Google Scholar 

  14. Vachiery F, Moreau R, Hadengue A, et al. Hypoxemia in patients with cirrhosis: relationship with liver failure and hemodynamic alterations. J Hepatol 1997;27:492–495.

    Article  PubMed  CAS  Google Scholar 

  15. 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.

    Article  PubMed  CAS  Google Scholar 

  16. Castro M, Krowka MJ. Hepatopulmonary syndrome. A pulmonary vascular complication of liver disease. Clin Chest Med 1996;17:35–48.

    Article  PubMed  CAS  Google Scholar 

  17. Krowka MJ, Dickson E, Cortese D. Hepatopulmonary syndrome: clinical observations and lack of therapeutic response to somatostatin analogue. Chest 1993;104:515–521.

    Article  PubMed  CAS  Google Scholar 

  18. Rydell R, Hoffbauer FW. Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis. Am J Med 1956;21:450–459.

    Article  PubMed  Google Scholar 

  19. Krowka MJ, Wiseman GA, Burnett OL, et al. Hepatopulmonary syndrome: a prospective study of relationships between severity of liver disease, PaO(2) response to 100% oxygen, and brain uptake after (99m)Tc MAA lung scanning. Chest 2000;118:615–624.

    Article  PubMed  CAS  Google Scholar 

  20. Gupta P, Mordin C, Curtis J, Hughes JMB, Shovlin CL, Jackson JE. Pulmonary arteriovenous malformations: effect of embolization on right-to-left shunt, hypoxemia, and exercise tolerance in 66 patients. Am J Radiol 2002;179:347–355.

    CAS  Google Scholar 

  21. Ryu JK, Oh JIT. Hepatopulmonary syndrome: angiography and therapeutic embolization. J Clin Imag 2003;27:97–100.

    Article  Google Scholar 

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

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  24. Maniscalco M, Sofia M, Higenbottam T. Effects of an NO-synthase inhibitor L-NMMA in the hepato-pulmonary syndrome. Respiration 2001;68:226.

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  26. Paramesh AS, Husain SZ, Shneider B, et al. Improvement of hepatopulmonary syndrome after transjugular intrahepatic portasystemic shunting: case report and review of the literature. Pediat Transpl 2003;7:157–162.

    Article  Google Scholar 

  27. Lasch HM, Fried MW, Zacks SL, et al. Use of transjugular intrahepatic portosystemic shunt as a bridge to liver transplantation in a patient with severe hepatopulmonary syndrome. Liver Transpl 2001;17:147–149.

    Article  Google Scholar 

  28. Egawa H, Kasahara M, Inomata Y, et al. Long-term outcome of living related liver transplantation for patients with intrapulmonary shunting and strategy for complications. Transplantation 1999;67:712–717.

    Article  PubMed  CAS  Google Scholar 

  29. Krowka M, Porayko M, Plevak D, et al. Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: case reports and review of the literature. Mayo Clinic Proc 1997;72:44–53.

    CAS  Google Scholar 

  30. Taille C, Cadranel J, Bellocq A, et al. Liver transplantation for hepatopulmonary syndrome: a ten year experience in Paris, France. Transplantation 2003;75:1482–1489.

    Article  PubMed  Google Scholar 

  31. Swanson KL, Wiesner RH, Rosen CB, Krowka MJ. Pulmonary vascular complications of liver disease: survival in 140 Mayo Clinic patients. Hepatology 2003;A250.

    Google Scholar 

  32. Schenk P, Schoniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Muller C. Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology 2003;125:1042–1052.

    Article  PubMed  Google Scholar 

  33. (UNOS) UNfOS.http://www.unos.org.

  34. Edwards B, Weir K, Edwards WD, Ludwig J, Dykoski RK, Edwards JE. Coexistent pulmonary and portal hypertension: morphologic and clinical features. J Am Coll Cardiol 1987;10:1233–1238.

    Article  PubMed  CAS  Google Scholar 

  35. Krowka MJ. Hepatopulmonary syndromes. Gut 2000;46:1–4.

    Article  PubMed  CAS  Google Scholar 

  36. Third World Symp Pulmonary Arterial Hypertension, Venice, Italy; June 23–25, 2003.

    Google Scholar 

  37. Ramsay MAE, Simpson BR, Nguyen AT, Ramsay KJ, East C, Klintmalm GB. Severe pulmonary hypertension in liver transplant candidates. Liver Transpl Surg 1997;3:494–500.

    Article  PubMed  CAS  Google Scholar 

  38. Tamara P, Garcia-Valdecasas JC, Beltran J, et al. Moderate primary pulmonary hypertension in patients undergoing liver transplantation. Anaesth Analg 1996;83:675–680.

    Article  Google Scholar 

  39. Castro M, Krowka MJ, Schroeder DR, et al. Frequency and clinical implications of increased pulmonary artery pressures in liver transplantation. Mayo Clinic Proc 1996;71:543–551.

    CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  41. Kim WR, Krowka MJ, Plevak DJ, et al. Accuracy of doppler echocardiography in the assessment of pulmonary hypertension in liver transplant candidates. Liver Transpl 2000;6:453–458.

    Article  PubMed  Google Scholar 

  42. Torregrosa M, Genesca J, Gonzalez A, et al. Role of doppler echocardiography in the assessment of portopulmonary hypertension in liver transplantation candidates. Transplantation 2001;71:572–574.

    Article  PubMed  CAS  Google Scholar 

  43. Bhatia V, Nayyar P, Dhindsa S. Brain natriuretic peptide in diagnosis and treatment of heart failure. J Postgrad Med 2003;49:182–185.

    PubMed  CAS  Google Scholar 

  44. Nagaya N, Nishikimi T, Uematsu M, et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 2000;102:865–970.

    PubMed  CAS  Google Scholar 

  45. Krowka MJ, Frantz RP, McGoon MD, Severson C, Plevak DJ, Wiesner RH. Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): a study of 15 patients with moderate to severe portopulmonary hypertension. Hepatology 1999;30:641–648.

    Article  PubMed  CAS  Google Scholar 

  46. Kuo PC, Johnson LB, Plotkin JS, Howell CD, Bartlett ST, Rubin LJ. Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension. Transplantation 1997;63:604–606.

    Article  PubMed  CAS  Google Scholar 

  47. Krowka MJ, Plevak DJ, Findlay JY, Rosen CB, Wiesner RH, Krom RA. Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation. Liver Transpl 2000;6:443–450.

    Article  PubMed  CAS  Google Scholar 

  48. Morrow CS, Kantor M, Armen RN. Hepatic hydrothorax. Ann Int Med 1958;49:193–203.

    PubMed  CAS  Google Scholar 

  49. Strauss RM, Boyer TD. Hepatic hydrothorax. Sem Liver Dis 1997;17:227–232.

    CAS  Google Scholar 

  50. Alberts WM, Salem AJ, Solomon DA, Boyce G. Hepatic hydrothorax: cause and management. Arch Int Med 1991;151:2383–2388.

    Article  CAS  Google Scholar 

  51. Sahn SA. State of the art: the pleura. Am Rev Respirat Dis 1988;138:184–234.

    CAS  Google Scholar 

  52. Kinasewitz GT, Keddissi JI. Hepatic hydrothorax. Cun Opin Pulmon Med 2003;9:261–265.

    Article  Google Scholar 

  53. Benet A, Vidal F, Toda R, et al. Diagnosis of hepatic hydrothorax in the absence of ascites by intra-peritoneal injection of 99mTc-sulfur colloid. Postgrad Med J 1992;68:153.

    Article  PubMed  CAS  Google Scholar 

  54. Milenas de Campos JR, Filho LO, Campos Werebe E, et al. Thoracoscopy and talc poudrage in the management of hepatic hydrothorax. Chest 2000;118:13–17.

    Article  Google Scholar 

  55. Mouroux J, Perrin C, Venissac N, Blaive B, Richelme H. Management of pleural effusion of cirrhotic origin. Chest 1996;109:1093–1096.

    PubMed  CAS  Google Scholar 

  56. Artemiou O, Marta G, Klepetka W, Wolner E, Muller M. Pleurovenous shunting in the treatment of nonmalignant pleural effusion. Ann Thoracic Surg 2003;76:231–233.

    Article  Google Scholar 

  57. Andrade RJ, Martin-Palance A, Faile JM, et al. Transjugular intrahepatic portosystemic shunt for the management of hepatic hydrothorax in the absence of ascites. J Clin Gastroenterol 1996;22:305–307.

    Article  PubMed  CAS  Google Scholar 

  58. Strauss RM, Martin LG, Kaufman SL, Boyer TD. Transjugular intrahepatic portal systemic shunt for the management of symptomatic cirrhotic hydrothorax. Am J Gastroenterol 1994;89:1520–1522.

    PubMed  CAS  Google Scholar 

  59. Spencer EB, Cohen DT, Darcy ML). Safety and efficacy of transjugular intrahepatic portosystemic shunt creation for the treatment of hepatic hydrothorax. J Vase Intl Radiol 2002;13:385–390.

    Article  Google Scholar 

  60. Kaplan LM, Epstein SK, Schwartz SL, Cao QL, Pandian NG. Clinical, echocardiographic, and hemodynamic evidence of cardiac tamponade caused by large pleural effusions. Am J Respirat Crit Care Med 1995;151:904–908.

    CAS  Google Scholar 

  61. Van Thiel DH, Nadir A, Hassanein T, Wright HI. Simultaneous right-sided volume overload and left-sided hypovolemia in a man with massive ascites and a hydrothorax. Am J Gastroenterol 1995;90:478–481.

    PubMed  Google Scholar 

  62. Wong F, Liu P, Lilly L, et al. The role of cardiac structural and functional abnormalities in the pathogenesis of hyperdynamic circulation and renal sodium retention in cirrhosis. Clin Sci 1999;97:259–267.

    Article  PubMed  CAS  Google Scholar 

  63. Pozzi M, Carugo S, Boari G, et al. Functional and structural cardiac abnormalities in cirrhotic patients with and without ascites. Hepatology 1997;26:1131–1137.

    PubMed  CAS  Google Scholar 

  64. Naschitz JE, Slobodin G, Lewis RJ, Zuckerman E, Yeshurun D. Heart diseases affecting the liver and liver diseases affecting the heart. Am Heart J 2000;140:111–120.

    Article  PubMed  CAS  Google Scholar 

  65. Ahah A, Variyam E. Pericardia! effusion and left ventricular dysfunction associated with ascites secondary to hepatic cirrhosis. Arch Int Med 1988;148:585–588.

    Article  Google Scholar 

  66. Wong F, Girgrah N, Graba J, Allidina Y, Liu P, Blendis L. The cardiac response to exercise in cirrhosis. Gut 2001;49:268–275.

    Article  PubMed  CAS  Google Scholar 

  67. Finucci G, Desideri A, Sacerdoti D, et al. Left ventricular diastolic dysfunction in liver cirrhosis. Scand J Gastroenterol 1996;31:279–284.

    PubMed  CAS  Google Scholar 

  68. Bernardi M, Rubboli A, Trevisani F, et al. Reduced cardiobascular responsiveness to exercise-induced sympathoadrenergic stimulation in patients with cirrhosis. J Hepatol 1991;12:207–216.

    Article  PubMed  CAS  Google Scholar 

  69. Grose RD, Nolan J, Dillon JF, et al. Exercise-induced left ventricular dysfunction in alcoholic and nonalcoholic cirrhosis. J Hepatol 1995;22:326–332.

    Article  PubMed  CAS  Google Scholar 

  70. Wong F, Siu S, Liu P, Blendis L. Brain natriuretic peptide: is it a predictor of cardiomyopathy in cirrhosis. Clin Sci 2001;101:621–628.

    Article  PubMed  CAS  Google Scholar 

  71. Henriksen JH, Gotze JP, Fuglsang S, Christensen E, Bendtsen F, Moller S. Increased circulating pro-brain natriuretic peptide (proBNP) and brain natriuretic peptide (BNP) in patients with cirrhosis: relation to cardiovascular dysfunction and severity of disease. Gut 2003;52:1511–1517.

    Article  PubMed  CAS  Google Scholar 

  72. Agraou B, Le Tourneau T, Agraou H, Leroy F, Asseman P, Dujardin JJ. Primary biliary cirrhosis associated with pericardial effusion. Ann Cardiol Angeiol (Paris) 1998;47:576–578.

    CAS  Google Scholar 

  73. Beyer N. Improved physical performance after orthotopic liver transplantation. Liver Transpl Surg 1999;5:301–309.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Mayo Clinic, Division of Pulmonary and Critical Care, Rochester, MN

    Karen L. Swanson DO

  2. Mayo Clinic, Divisions of Pulmonary and Critical Care and of Gastroenterology and Hepatology, Rochester, MN

    Michael J. Krowka MD

Authors
  1. Karen L. Swanson DO
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Michael J. Krowka MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Division of Gastroenterology, Virginia Commonwealth Medical Center, Richmond, VA

    Arun J. Sanyal MBBS, MD

  2. GI Research Unit, Mayo Clinic, Rochester, MN

    Vijay H. Shah MD

Rights and permissions

Reprints and permissions

Copyright information

© 2005 Humana Press Inc., Totowa, NJ

About this chapter

Cite this chapter

Swanson, K.L., Krowka, M.J. (2005). Pulmonary Complications Associated with Portal Hypertension. In: Sanyal, A.J., Shah, V.H. (eds) Portal Hypertension. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-59259-885-4_27

Download citation

  • DOI: https://doi.org/10.1007/978-1-59259-885-4_27

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-386-2

  • Online ISBN: 978-1-59259-885-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation