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Pulmonary Complications of Cirrhosis

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Abstract

Pulmonary vascular complications of liver disease comprise two distinct clinical entities: hepatopulmonary syndrome (HPS—microvascular dilatation and angiogenesis) and portopulmonary hypertension (POPH—vasoconstriction and remodeling in resistance vessels). These complications occur in similar pathophysiologic environments and may share pathogenic mechanisms. HPS is found in 15% to 30% of patients with cirrhosis and its presence increases mortality and the risks of liver transplantation, particularly when hypoxemia is present. Contrast echocardiography and arterial blood gas analysis are required to establish the diagnosis. No medical therapies are available, although liver transplantation is effective in reversing the syndrome. POPH is found in 4% to 8% of patients undergoing liver transplantation evaluation, and the presence of moderate to severe disease significantly increases perioperative transplant mortality. Transthoracic echocardiography is recommended for screening and right-heart catheterization is required to establish the diagnosis. Medical therapies are increasingly effective in improving pulmonary vascular hemodynamics in POPH and may result in better perioperative outcomes.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance

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

    CAS  PubMed  Google Scholar 

  2. Martinez G, Barbera J, Navasa M, et al.: Hepatopulmonary syndrome associated with cardiorespiratory disease. J. Hepatol. 1999;30:882–9.

    Article  CAS  PubMed  Google Scholar 

  3. 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  CAS  PubMed  Google Scholar 

  4. Martinez G, Barbera J, Visa J, et al.: Hepatopulmonary Syndrome in candidates for liver transplantation. J. Hepatol. 2001;34:756–758.

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  6. Teuber G, Teupe C, Dietrich C, et al.: Pulmonary dysfunction in non-cirrhotic patients with chronic viral hepatitis. Eur J Intern Med 2002;13:311–318.

    Article  PubMed  Google Scholar 

  7. Fuhrmann V, Madl C, Mueller C, et al.: Hepatopulmonary Syndrome in Patients With Hypoxic Hepatitis. Gastroenterology 2006;131:69–75.

    Article  PubMed  Google Scholar 

  8. Dimand RJ, Heyman MB, Bass NM, et al.: Hepatopulmonary syndrome: response to hepatic transplantation. Hepatology 1991;141 (Pt. 2):55A [Abstract].

    Google Scholar 

  9. Abrams G, Fallon M: The Hepatopulmonary Syndrome. Clinics in Liver Disease 1997;1:185–200.

    Article  CAS  PubMed  Google Scholar 

  10. Binay K, Sen S, Biswas PK, et al.: Hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty. Gastroenterology. 2000;118:192–196.

    Article  Google Scholar 

  11. Gupta D, Vijaya DR, Gupta R, et al.: Prevalence of hepatopulmonary syndrome in cirrhosis and extrahepatic portal venous obstruction. American Journal of Gastroenterology 2001;96:3395–3399.

    Article  CAS  PubMed  Google Scholar 

  12. • Fallon M, Krowka M, Brown R, et al.; Pulmonary Vascular Complications of Liver Disease Study Group: Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterol 2008, Oct;135(4):1168–75. This article describes a recent multicenter prospective study of a large group of liver transplant candidates demonstrating reduced quality of life and survival in patients with HPS.

    Article  Google Scholar 

  13. Schraufnagel D, Kay J: Structural and pathologic changes in the lung vasculature in chronic liver disease. Clinics in Chest Medicine 1996;17:1–15.

    Article  CAS  PubMed  Google Scholar 

  14. Brussino L, Bucca C, Morello M, et al.: Effect on dyspnoea and hypoxaemia of inhaled NG-nitro-L-arginine methyl ester in hepatopulmonary syndrome. The Lancet 2003;362:43–44.

    Article  CAS  Google Scholar 

  15. Schenk P, Madl C, Rezale-Majd S, et al.: Methylene blue improves the hepatopulmonary syndrome. Ann. Int. Med. 2000;133:701–6.

    CAS  PubMed  Google Scholar 

  16. Degano B, Mittaine M, Hervé P, et al.: Nitric oxide production by the alveolar compartment of the lungs in cirrhotic patients. Eur Respir J. 2009 Jul;34(1):138–44.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  19. Zhang J, Ling Y, Luo B, et al.: Analysis of pulmonary heme oxygenase-1 and nitric oxide synthase alterations in experimental hepatopulmonary syndrome. Gastroenterology 2003;125:1441–51.

    Article  CAS  PubMed  Google Scholar 

  20. Carter EP, Hartsfield CL, Miyazono M, et al.: Regulation of heme oxygenase-1 by nitric oxide during hepatopulmonary syndrome. American Journal of Physiology—Lung Cellular & Molecular Physiology. 2002;283:L346–L353.

    CAS  Google Scholar 

  21. Zhang J, Luo B, Tang L, et al.: Pulmonary Angiogenesis in a Rat Model of Hepatopulmonary Syndrome. Gastroenterology 2009;136:1070–1080.

    Article  CAS  PubMed  Google Scholar 

  22. Sood G, Fallon MB, Niwas S, et al.: Utility of a dyspnea-fatigue index for screening liver transplant candidates for hepatopulmonary syndrome. Hepatology. 1998;28:2319 [abstract].

    Google Scholar 

  23. Robin ED, Laman D, Horn BR, et al.: Platypnea related to orthodeoxia caused by true vascular lung shunts. N Engl J Med 1976;294:941–943.

    Article  CAS  PubMed  Google Scholar 

  24. Martinez GP, Barbera JA, Visa J, et al.: Hepatopulmonary syndrome in candidates for liver transplantation. Journal of Hepatology. 2001;34(5):651–7.

    Article  CAS  PubMed  Google Scholar 

  25. McAdams HP, Erasmus J, Crockett R, et al.: The hepatopulmonary syndrome: radiologic findings in 10 patients. Am J Roentgenol 1996;166 (6):1379–85.

    CAS  Google Scholar 

  26. Lima B, Franca A, Pazin-Filho A, et al.: Frequency, clinical characteristics, and respiratory parameters of hepatopulmonary syndrome. Mayo Clin Proc 2004;79:42–8.

    Article  PubMed  Google Scholar 

  27. Abrams GA, Sanders MK, Fallon MB: Utility of pulse oximetry in the detection of arterial hypoxemia in liver transplant candidates. Liver Transpl.. 2002;8(4):391–6.

    Article  PubMed  Google Scholar 

  28. Abrams G, Nanda N, Dubovsky E, et al.: Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome:a new approach. Gastroenterology. 1998;114(305–10).

    Google Scholar 

  29. Lee KN, Lee HJ, Shin WW, et al.: Hypoxemia and liver cirrhosis (hepatopulmonary syndrome) in eight patients: comparison of the central and peripheral pulmonary vasculature. Radiology 1999;211:549–553.

    CAS  PubMed  Google Scholar 

  30. Swanson K, Wiesner R, Krowka M: Natural history of hepatopulmonary syndrome: Impact of liver transplantation. Hepatology 2005;41:1122–1129.

    Article  PubMed  Google Scholar 

  31. Abrams GA, Fallon MB: Treatment of hepatopulmonary syndrome with allium sativum L. (garlic): A pilot trial. J. Clin. Gastro. 1998;27:232–235.

    Article  CAS  Google Scholar 

  32. Song JY, Choi JY, Ko JT, et al.: Long-term aspirin therapy for hepatopulmonary syndrome. Pediatrics 1996;97 (6 Pt 1):917–920.

    CAS  PubMed  Google Scholar 

  33. Anel RM, Sheagren JN: Novel presentation and approach to management of hepatopulmonary syndrome with use of antimicrobial agents. Clinical Infectious Diseases. 2001;32:E131–136.

    Article  CAS  PubMed  Google Scholar 

  34. Brussino L, Bucca C, Morello M, et al.: Effect on dyspnoea and hypoxaemia of inhaled NG-nitro-L-arginine methyl ester in hepatopulmonary syndrome. The Lancet 2003;362:43–44.

    Article  CAS  Google Scholar 

  35. Tanikella R, Philips G, Faulk D, et al.: Pilot study of pentoxifylline in hepatopulmonary syndrome. Liver Transplantation 2008;14:1199–1203.

    Article  PubMed  Google Scholar 

  36. Arguedas M, Abrams GA, Krowka MJ, et al.: Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation. Hepatology 2003;37:192–197.

    Article  PubMed  Google Scholar 

  37. • Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB, on behalf of the ERS Task Force Pulmonary-Hepatic Vascular Disorders Scientific Committee ERS Task Force PHD Scientific Committee. Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J 2004;24:861–880. Recent comprehensive review of HPS and POPH outlining epidemiology, pathogenesis, diagnosis and current therapies.

    Article  CAS  PubMed  Google Scholar 

  38. • Colle I, Moreau R, Godinho E, et al.: Portopulmonary Hypertension in Candidates For Liver Transplantation: Diagnosis at Evaluation Comparing Doppler Echocardiography With Cardiac Catheterization and Incidence on The Waiting List. Hepatology 2003;37:401–409. In this study the authors emphasize the importance of Doppler echocardiography as a excellent screening modality for portopulmonary hypertension and the need for right side cardiac catheterization as a confirmatory test.

    Article  PubMed  Google Scholar 

  39. 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  CAS  PubMed  Google Scholar 

  40. • Kawut S, Krowka M, Trotter J, et al.: Clinical risk factors for portopulmonary hypertension. Hepatology 2008;48:196–203. This article describes a multicenter case-control study that assessed the different risk factors associated with portopulmonary hypertension.

    Article  PubMed  Google Scholar 

  41. Budhiraja R, Hassoun PM: Portopulmonary Hypertension: A Tale of Two Circulations. Chest 2003;123(2):562–576.

    Article  PubMed  Google Scholar 

  42. Swanson KL, Krowka MJ: Screen for portopulmonary hypertension, especially in liver transplant candidates. Cleve Clin J Med 2008; 75:121–36.

    Article  PubMed  Google Scholar 

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

    Article  Google Scholar 

  44. Krowka MJ: Portopulmonary hypertension: Diagnostic advances and caveats. Liver Transpl 2003; 9:1336–7.

    PubMed  Google Scholar 

  45. Murray KF, Carrithers RL, Jr.: AASLD Practice Guidelines: Evaluation of the patient for liver transplantation. Hepatology 2005; 1–26.

  46. Swanson K, Wiesner R, Nyberg S, et al.: Survival in Portopulmonary Hypertension: Mayo Clinic Experience Categorized by Treatment Subgroups. American Journal of Transplantation 2008;8:2445–2453.

    Article  CAS  PubMed  Google Scholar 

  47. Le Pavec J, Souza R, Herve P, et al.: Portopulmonary Hypertension: Survival and Prognostic Factors. Am. J. Respir. Crit. Care Med. 2008;178:637–643.

    Article  PubMed  Google Scholar 

  48. Kawut S, Taichman D, Ahya V, et al.: Hemodynamics and survival of patients with portopulmonary hypertension. Liver Transpl 2005;11:1107–1111.

    Article  PubMed  Google Scholar 

  49. Ashfaq M, Chinnakotla S, Rogers L, et al.: The Impact of Treatment of Portopulmonary Hypertension on Survival Following Liver Transplantation. American Journal of Transplantation 2007;7:1258–1264.

    Article  CAS  PubMed  Google Scholar 

  50. Fix O, Bass N, De Marco T, Merriman R: Long-term follow-up of portopulmonary hypertension: Effect of treatment with epoprostenol. Liver Transplantation 2007;13:875–885.

    Article  PubMed  Google Scholar 

  51. Austin MJ, McDougall NI, Wendon JA, et al.: Safety and efficacy of combined use of sildenafil, bosentan, and iloprost before and after liver transplantation in severe portopulmonary hypertension. Liver Transplantation 2008;14:287–291.

    Article  PubMed  Google Scholar 

  52. Hoeper MM, Seyfarth HJ, Hoeffken G, et al.: Experience with inhaled iloprost and bosentan in portopulmonary hypertension. Eur Respir J 2007;30:1096–1102.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Michael B. Fallon.

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Kochar, R., Nevah Rubin, M.I. & Fallon, M.B. Pulmonary Complications of Cirrhosis. Curr Gastroenterol Rep 13, 34–39 (2011). https://doi.org/10.1007/s11894-010-0164-6

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