Skip to main content

Hepatopulmonary syndrome

  • Chapter
Hepatology Textbook and Atlas
  • 3261 Accesses

Abstract

Recognition of a hepatopulmonary syndrome goes back over one hundred years (1884) to the detection of the triad: cyanosis, clubbed fingers and liver cirrhosis. (18). The same constellation of findings was described by A. Gilbert et al. in 1895 in juvenile patients suffering from hypertrophic biliary cirrhosis. • Reports on hypertrophic osteoarthropathy were published by A. A. Hijmans van den Bergh in 1901. • A. M. Snell detected hypoxaemia in chronic liver patients for the first time in 1935. (52) A right shift of the dissociation curve of oxyhaemoglobin was ascertained by A. Keys et al. in 1938. In the course of acute progressive liver failure, R. Rydell et al. (1956) also observed hypoxaemia; at autopsy, this patient showed intrapulmonary arteriovenous shunts. (45) Since then, there have been many reports on the detection of arteriovenous anastomoses in the lungs (further details in references 6, 7, 23). These arteriovenous shunts had already been attributed to vasoactive substances. The development of clubbed fingers was deemed to be the result of arteriovenous anastomoses in the tips of the fingers and the impact of reduced ferritin.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Abrams, G.A., Fallon, M.B.: Treatment of hepatopulmonary syndrome with Allium Sativum L. A. pilot trial. J. Clin. Gastroenterol. 1998; 27: 232–235

    Article  PubMed  CAS  Google Scholar 

  2. Abrams, G.A., Nanda, N.C., Dubovsky, E.V., Krowka, M.J., Fallon, M.B.: Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome: a new approach. Gastroenterology 1998; 114: 305–310

    Article  PubMed  CAS  Google Scholar 

  3. Aller, R., Moreira, V., Boixeda, D., Moya, J.L., de Luis, D.A., Enriquez, J.L., Fogué, L.: Diagnosis of hepatopulmonary syndrome with contrast transthoracic echocardiography and histological confirmation. Liver 1998; 18: 285–287

    PubMed  CAS  Google Scholar 

  4. Anel, R.M., Sheagren, J.N.: Novel presentation and approach to management of hepatopulmonary syndrome with the use of antimicrobial agents. Clin. Infect. Dis. 2001; 10: E 131–136

    Article  Google Scholar 

  5. Arguedas, M.R., Fallon, M.B.: Hepatopulmonary syndrome. Curr. Treat. Opt. Gastroenterol. 2005; 8: 451–456

    Article  Google Scholar 

  6. Baltzer, G., Arndt, H., Martini, G.A.: Sauerstoffun tersättigung und veränderte Hämodynamik bei Lebercirrhose. Klin. Wschr. 1973; 51: 1033–1042

    Article  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  8. Bindl, L., Wagner, N., Knöpfli, g., Gosseve, St. Lentze, M.J.: Peliosis hepatis with hepato-pulmonary syndrome. Klin. Paediatr. 1998; 210: 47–49

    Article  CAS  Google Scholar 

  9. Cadranel, J.L., Milleron, B.J., Cadranel, J.F., Fermand, J.P., Andrivet, P., Brouet, J.C., Adnot, S., Akoun, G.M.: Severe hypoxemia-associated intrapulmonary shunt in a patient with chronic liver disease: improvement after medical treatment. Amer. Rev. Resp. Dis. 1992; 146: 526–527

    PubMed  CAS  Google Scholar 

  10. Caruso, G., Catalano, D.: Esophageal varices and hepatopulmonary syndrome in liver cirrhosis. J. Hepatol. 1991; 12: 262–263

    Article  PubMed  CAS  Google Scholar 

  11. Cremona, G., Higenbottam, T.W., Mayoral, V., Alexander, G., Demoncheaux, E., Borland, C., Roe, J., Jones, G.J.: Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. Eur. Respir. J. 1995; 8: 1883–1885

    Article  PubMed  CAS  Google Scholar 

  12. De, B.K., Sen, S., Biswas, P.K., Sanyal, R., Majumdar, D., Biswas, J.: Hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty. Gastroenterology 2000; 118: 192–196

    Article  PubMed  CAS  Google Scholar 

  13. De, B.K., Sen, S., Biswas, P.K., Mandral, S.K., Das, D., Das, U., Guru, S., Bandyopadhyay, K.: Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression. Gastroenterology 2002; 122: 897–903

    Article  PubMed  Google Scholar 

  14. Dickinson, C.J.: The aetiology of clubbing and hypertrophic osteoarthropathy. Eur. J. Clin. Invest. 1993; 23: 330–338

    Article  PubMed  CAS  Google Scholar 

  15. Eriksson, L.S., Söderman, C., Ericzon, B.-G., Eleborg, L., Wahren, J., Hedenstierna, G.: Normalization of ventilation perfusion relationship after liver transplantation in patients with decompensated cirrhosis: evidence for an hepatopulmonary syndrome. Hepatology 1990; 12: 1350–1357

    Article  PubMed  CAS  Google Scholar 

  16. Fallon, M.B., Abrams, G.A., Luo, B., Hou, Z., Dai, J.: The role of endothelial nitric oxide synthase in the pathogenesis of a rat model of hepatopulmonary syndrome. Gastroenterology 1997; 113: 606–614

    Article  PubMed  CAS  Google Scholar 

  17. Fiel, M.I., Schiaro, T.D., Suriawinata, A., Emre, S.: Portal hypertension and hepatopulmonary syndrome in a middle-aged man with hepatitis B infection. Semin. Liver Dis. 2000; 20: 391–395

    Article  PubMed  CAS  Google Scholar 

  18. Flückiger, M.: Vorkommen von trommelschlägelförmigen Fingerendphalangen ohne chronische Veränderungen an den Lungen oder am Herzen. Wien. Med. Wschr. 1884; 34: 1457–1458

    Google Scholar 

  19. Ginies, J.L., Couetil, J.P., Houssin, D., Guillemain, R., Champion, G., Bernard, O.: Hepatopulmonary syndrome in a child with cystic fibrosis. J. Pediatr. Gastroenterol. Nutr. 1996; 23: 497–500

    Article  PubMed  CAS  Google Scholar 

  20. Gupta, D., Vijaya, D.R., Gupta, R., Dhiman, R.K., Bhargava, M., Verma, D.M.J., Chawla, Y.K.: Prevalence of hepatopulmonary syndrome in cirrhosis and extrahepatic portal venous obstruction. Amer. J. Gastroenterol. 2001; 96: 3395–3399

    Article  CAS  Google Scholar 

  21. Hannam, P.D., Sandokji, A.K.M., Machan, L.S., Erb, S.R., Champion, P., Buczkowski, A.K., Scudamore, C.H., Steinbrecher, U.P., Chung, S.W., Weiss, A.A., Yoshida, E.M.: Post-surgical shunt hepatopulmonary syndrome in a case of non-cirrhotic portal hypertension: lack of efficacy of shunt reversal. Eur. J. Gastroenterol. Hepatol. 1999; 11: 1425–1427

    Article  PubMed  CAS  Google Scholar 

  22. Hourani, J.M., Bellamy, P.E.B., Tashkin, D.P., Batra, P., Simmons, M.S.: Pulmonary dysfunction in advanced liver disease: frequent occurrence of an abnormal diffusing capacity. Amer. J. Med. 1991; 90: 693–700

    PubMed  CAS  Google Scholar 

  23. Irle, U., Ohlms, F.B.: Leberzirrhose mit intrapulmonalen arterioven ösen Mikroanastomosen. Schweiz. Rundsch. Med. 1968; 57: 1720–1722

    CAS  Google Scholar 

  24. Jeffrey, G.P., Prince, R.L., van der Schaaf, A.: Fatal intrapulmonary arteriovenous shunting in cirrhosis: diagnosis by radionuclide lung perfusion scan. Med. J. Aust. 1990; 152: 549–553

    PubMed  CAS  Google Scholar 

  25. Kaymakoglu, S., Kahraman, T., Kudat, H., Demir, K., Cakaloglu, Y., Adalet, I., Dincer, D., Besisik, F. Boztas, G., Sözen, A.B., Mungan, Z., Okten, A.: Hepatopulmonary syndrom in noncirrhotic portal hypertensive patients. Dig. Dis. Sci. 2003; 48: 556–560

    Article  PubMed  CAS  Google Scholar 

  26. Kennedy, T.C., Knudson, R.J.: Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis. Chest 1977; 72: 305–309.

    Article  PubMed  CAS  Google Scholar 

  27. Krowka, M.J., Cortese, D.A.: Hepatopulmonary syndrome — current concepts in diagnostic and therapeutic considerations. Chest 1994; 105: 1528–1537

    Article  PubMed  CAS  Google Scholar 

  28. Krowka, M.J., Porayko, M.K., Plevak, D.J., Pappas, S.C., Steers, J.L., Krom, R.A.F., Wiesner, R.H.: Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: case reports and literature review. Mayo Clin. Proc. 1997; 72: 44–53

    Article  PubMed  CAS  Google Scholar 

  29. Lange, P.A., Stoller, J.K.: The hepatopulmonary syndrome. Effect of liver transplantation. Clin. Chest Med. 1996; 17: 115–123

    Article  PubMed  CAS  Google Scholar 

  30. Lasch, H.M., Fried, M.W., Zacks, S.L., Odell, P., Johnson, M.W., Gerber, D.A., Sandhu, F.S., Fair, J.H., Shrestha, R.: Use of transjugular intrahepatic portosystemic shunt as a bridge to liver transplantation in a patient with severe hepatopulmonary syndrome. Liver Transplant. 2001; 7: 147–149

    Article  CAS  Google Scholar 

  31. Lee, K.-N., Lee, H.-J., Shin, W.W., Webb, W.R.: Hypoxemia and liver cirrhosis (hepatopulmonary syndrome) in eight patients: comparison of the central and peripheral pulmonary vasculature. Radiology 1999; 211: 549–553

    PubMed  CAS  Google Scholar 

  32. Lima, B.L.G., Franca, A.V.C., Pazin-Filho, A., Araujo, W.M., Martinez, J.A.B., Maciel, B.C., Simoes, M.V., Terra-Filho, J., Martinelli, A.L.C.: Frequency, clinical characteristics and respiratory parameters of hepatopulmonary syndrome. Mayo Clin. Proc. 2004; 79: 42–48

    Article  PubMed  Google Scholar 

  33. Mandel, M.S.: The diagnosis and treatment of hepatopulmonary syndrome. Clin. Liver Dis. 2006; 10: 387–405

    Article  Google Scholar 

  34. Martinez, G.P., Barbera, J.A., Visa, J., Rimola, A., Pare, J.C., Roca, J., Navasa, M. Rodes, J., Rodriguez-Roisin, R.: Hepatopulmonary syndrome in candidates for liver transplantation. J. Hepatol. 2001; 34: 651–657

    Article  PubMed  CAS  Google Scholar 

  35. Martini, G.A., Hagemann, J.E.: Über Fingernagelveränderungen bei Lebercirrhose als Folge veränderter peripherer. Durchblutung. Klin. Wschr. 1956; 34: 25–31

    Article  CAS  Google Scholar 

  36. McAdams, H.P., Erasmus, J., Crockett, R., Mitchell, J., Godwin, J.D., McDermott, V.G.: The hepatopulmonary syndrome: radiologic findings in 10 patients. Amer. J. Roentgenol. 1996; 166: 1379–1385

    CAS  Google Scholar 

  37. Numes, H., Lebrec, D., Mazmanian, M., Eapron, F., Heller, J., Tazi, K.A., Zerbib, E., Dulmet, E., Moreau, R., Dinh-Kuan, A., Simonneau, G.: Role of nitric oxide in hepatopulmonary syndrome in cirrhotic rats. Amer. J. Respir. Crit. Care Med. 2001; 164: 879–885

    Google Scholar 

  38. Palma, D.T., Fallon, M.B.: The hepatopulmonary syndrome. J. Hepatol. 2006; 45: 617–625

    Article  PubMed  Google Scholar 

  39. Poterucha, J.J., Krowka, M.J., Dickson, E.R., Cortese, D.A., Stanson, A.W., Krom, R.A.: Failure of hepatopulmonary syndrome to resolve after liver transplantation and successful treatment with embolotherapy. Hepatology 1995; 21: 96–100

    Article  PubMed  CAS  Google Scholar 

  40. Regev, A., Yeshurun, M., Rodriguez, M., Sagie, A., Neff, G.W., Molina, E.G., Schiff, E.R.: Transient hepatopulmonary syndrome in a patient with acute hepatitis A. J. Viral Hepat. 2001; 8: 83–86

    Article  PubMed  CAS  Google Scholar 

  41. Riegler, J.L., Lang, K.A., Johnson, S.P., Westerman, H.H.: Transjugular intrahepatic portosystemic shunt improves oxygenation in hepatopulmonary syndrome. Gastroenterology 1995; 109: 978–983

    Article  PubMed  CAS  Google Scholar 

  42. Robin, E.D., Laman, D., Horn, B.R., Theodore, J.: Platypnea related to orthodeoxia caused by true vascular lung shunts. New Engl. J. Med. 1976; 294: 941–943

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  44. Rutishauser, M., Egli, F., Wyler, F.: Juvenile Leberzirrhose mit multiplen arterio-venösen Aneurysmen der Lunge. Schweiz. Med. Wschr. 1972; 102: 514–517

    PubMed  CAS  Google Scholar 

  45. Rydell, R., Hoffbauer, F.W.: Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis. Amer. J. Med. 1956; 21: 450–460

    Article  PubMed  Google Scholar 

  46. Schenk, P., Madl, C., Rezzaie-Majd, S., Lehr, St. Müller, C.: Methylene blue improves the hepatopulmonary syndrome. Ann. Intern. Med. 2000; 133: 701–706

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  48. Scott, V., Miro, A., Kang, Y., DeWolf, A., Bellary, S., Martin, M., Kramer, A., Selby, R., Doyle, H., Paradis, I., Starzl, T.E.: Reversibility of the hepatopulmonary syndrome by orthotopic liver transplantation. Transplant. Proc. 25 (1993), 1787–1788

    PubMed  CAS  Google Scholar 

  49. Scott, V.L., Dodson, S.F., Kang, Y.: The hepatopulmonary syndrome. Surg. Clin. North Amer. 1999; 79: 23–31

    Article  CAS  Google Scholar 

  50. Selim, K.M., Akriviadis, E.A., Zuckerman, E., Chen, D., Reynolds, T.B.: Transjugular intrahepatic portosystemic shunt: a successful treatment for hepatopulmonary syndrome. Amer. J. Gastroenterol. 1998; 93: 455–458

    Article  CAS  Google Scholar 

  51. Shijo, H., Sasaki, H., Miyajima, Y., Okumura, M.: Prostaglandin F and indomethacin in hepatogenic pulmonary angiodysplasia: effects on pulmonary hemodynamics and gas exchange. Chest 1991; 100; 873–875

    Article  PubMed  CAS  Google Scholar 

  52. Snell, A.M.: The effects of chronic disease of the liver on the composition and physicochemical properties of blood: changes in the serum proteins; reduction in the oxygen saturation of the arterial blood. Ann. Intern. Med. 1935; 9: 690–711

    Google Scholar 

  53. Stein, H., Stein, S.: Digital clubbing in cirrhosis of the liver. Lancet 1961/I: 999–1000

    Article  Google Scholar 

  54. Teuber, G., Teube, C., Dietrich, C.F., Caspary, W.F., Buhl, R., Zeuzem, St.: Pulmonary dysfunction in non-cirrhotic patients with chronic viral hepatitis. Eur. J. Intern. Med. 2002; 13: 311–318

    Article  PubMed  Google Scholar 

  55. Vachiéry, F., Mora, J.R., Hadengue, A., Gadano, A., Soupison, T., Valla, D., Lebrec, D.: Hypoxemia in patients with cirrhosis: relationship with liver failure and hemodynamic alterations. J. Hepatol. 1997; 27: 492–495

    Article  PubMed  Google Scholar 

  56. Whyte, M.K.B., Hughes, J.M.B., Peters, A.M., Ussow, W., Patel, S., Burroughs, A.K.: Analysis of intrapulmonary right to left shunt in the hepatopulmonary syndrome. J. Hepatol. 1998; 29: 85–93

    Article  PubMed  CAS  Google Scholar 

  57. Williams, A., Trewby, P., Williams, R., Reid, L.: Structural alterations to the pulmonary circulation in fulminant hepatic failure. Thorax 1979; 34: 447–453

    Article  PubMed  CAS  Google Scholar 

  58. Yao, E.H., Kong, B., Hsue, G., Zhou, A., Wang, H.: Pulmonary function changes in cirrhosis of the liver. Amer. J., Gastroenterology. 1987; 82: 352–354

    CAS  Google Scholar 

  59. Zhang, J.L., Ling, Y.Q., Luo, B., Tang, L.P., Ryter, S.W., Stockard, C.R., Grizzle, W.E., Fallon, M.B.: Analysis of pulmonary heme oxygenase-1 and nitric oxide synthase alterations in experimental hepatopulmonary syndrome. Gastroenterology 2003; 125: 1441–1451

    Article  PubMed  CAS  Google Scholar 

Portopulmonary hypertension

  1. Aucejo, F., Miller, C., Vogt, D., Eghtesad, B., Nakagawa, S., Stoller, J.K.: Pulmonary hypertension after liver transplantation in patients with antecedent hepatopulmonary syndrome. A report of 2 cases and review of the literature. Liver Transplant. 2006; 12: 1278–1282

    Article  Google Scholar 

  2. Bayraktar, Y., Tanaci, N., Egesel, T., Gököz, A., Balkanci, F.: Antiphospholipid syndrome presenting as portopulmonary hypertension. J. Clin. Gastroenterol. 2001; 32: 359–361

    Article  PubMed  CAS  Google Scholar 

  3. Colle, I., Moreau, R., Godinho, E., Belghiti, J., Ertori, F., Cohen-Solai, A., Mal, H., Bernuau, J., Marty, J., Lebrec, D., Valla, D., Durand, F.: Diagnosis of portopulmonary hypertension in candidates for liver transplantation: a prospective study. Hepatology 2003; 37: 401–409

    Article  PubMed  Google Scholar 

  4. Halank, M., Ewert, R., Seyfarth, H.J., Hoeffken, G.: Portopulmonary hypertension. J. Gastroenterol. 2006; 41: 837–847

    Article  PubMed  Google Scholar 

  5. Hinterhuber, L., Graziadei, I.W., Kähler, E.M., Jaschke, W., Vogel, W.: Endothelin — receptor antagonist treatment of portopulmonary hypertension. Clin. Gastroenterol. Hepatol. 2004; 2: 1039–1042

    Article  PubMed  Google Scholar 

  6. Kähler, C.M., Graziadei, I., Wiedermann, C.J., Kneussl, M.P., Vogel, W.: Successful use of continuous intravenous prostacyclin in a patient with severe portopulmonary hypertension. Wien. Klin. Wschr. 2000; 112: 637–640

    PubMed  Google Scholar 

  7. Krowka, M.J., Frantz, R.P., McGoon, M.D., Severson, C., Plevak, D.J., Wiesner, R.H.: 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 

  8. Krowka, M.J.: Evolving dilemmas and management of portopulmonary hypertension. Semin. Liver. Dis. 2006; 26: 265–272

    Article  PubMed  Google Scholar 

  9. Kuo, P.C., Plotkin, J.S., Gaine, S., Schroeder, R.A., Rustgi, V.K., Rubin, L.R., Johnson, L.B.: Portopulmonary hypertension and the liver transplant candidate. Transplantation 1999; 67: 1087–1093

    Article  PubMed  CAS  Google Scholar 

  10. Levy, M.T., Torzillo, P., Bookallil, M., Sheil, A.G.R., McCaughan, G.W.: Case report: Delayed resolution of severe pulmonary hypertension after isolated liver transplantation in a patient with cirrhosis. J. Gastroenterol. Hepatol. 1996; 11: 734–737

    Article  PubMed  CAS  Google Scholar 

  11. Mantz, F., Craige, E.: Portal axis thrombosis with spontaneous portocaval shunt and resultant cor pulmonale. AMA Arch. Pathol. 1951; 52: 91–97

    PubMed  Google Scholar 

  12. Masaki, T.: The discovery of endothelins. Cardiovasc. Res. 1998; 39: 530–533

    Article  PubMed  CAS  Google Scholar 

  13. Passarella, M., Fallon, M.B., Kawut, S.M.: Portopulmonary hypertension. Clin. Liver Dis. 2006; 10: 653–663

    Article  PubMed  Google Scholar 

  14. Rubin, L.J., Badesch, D.B., Barst, R.J., Galie, N., Black, C.M., Keogh, A., Pulido, T., Frost, A., Roux, S., Leconte, I., Landzberg, M., Simonneau, G.: Bosentan therapy for pulmonary arterial hypertension. New Engl. J. Med. 2002; 346: 896–903

    Article  PubMed  CAS  Google Scholar 

  15. Schraufnagel, D.E., Kay, J.M.: Structural and pathologic changes in the lung vasculature in chronic liver disease. Clin. Chest Med. 1996; 17: 1–15

    Article  PubMed  CAS  Google Scholar 

  16. Starkel, P., Vera, A., Gunson, B., Mutimer, D.: Outcome of liver transplantation for patients with pulmonary hypertension. Liver Transplant. 2002; 8: 382–388

    Article  Google Scholar 

  17. Yang, Y.Y., Lin, H.C., Lee, W.C., Hou, M.C., Lee, F.Y., Chang, F.Y., Lee, S.D.: Portopulmonary hypertension: distinctive hemodynamic and clinical manifestations. J. Gastroenterol. 2001; 36: 181–186

    Article  PubMed  CAS  Google Scholar 

  18. Yokomori, H., Oda, M., Yasogawa, Y., Nishi, Y., Ogi, M., Takahashi, M., Ishii, H.: Enhanced expression of endothelin B receptor at protein and gene levels in human cirrhotic liver. J. Pathol. 2001; 159: 1353–1362

    CAS  Google Scholar 

Download references

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer Medizin Verlag Heidelberg

About this chapter

Cite this chapter

(2008). Hepatopulmonary syndrome. In: Hepatology Textbook and Atlas. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-76839-5_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-540-76839-5_18

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-76838-8

  • Online ISBN: 978-3-540-76839-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics