Skip to main content
Log in

Hepatopulmonary Syndrome in Noncirrhotic Portal Hypertensive Patients

  • Published:
Digestive Diseases and Sciences Aims and scope Submit manuscript

Abstract

Hepatopulmonary syndrome has yet not been sufficiently assessed in noncirrhotic portal hypertension. The prevalence of hepatopulmonary syndrome was determined in 31 consecutive patients with noncirrhotic portal hypertension (19 idiopathic portal hypertension, 7 portal vein thrombosis, 5 congenital hepatic fibrosis) and 46 patients with liver cirrhosis. Contrast echocardiography was carried out in all patients. Macroaggregated albumin lung perfusion scans were performed in patients with positive contrast echocardiogram. Hepatopulmonary syndrome was detected in 5 (10.8%) cirrhotic and 3 (9.7%) noncirrhotic portal hypertensive patients (2 idiopathic portal hypertension, 1 portal vein thrombosis). All patients with hepatopulmonary syndrome had an increased shunt fraction (13–62%) and a decreased diffusion capacity of carbon monoxide (40–79%), and 7 of them were hypoxemic (PaO2, 31.6–69.8 mm Hg). These findings show that hepatopulmonary syndrome may occur in both liver cirrhosis and noncirrhotic portal hypertension and that portal hypertension is the predominant etiopathogenic factor related to hepatopulmonary syndrome.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Rodriguez-Roisin R, Agusti AGN, Roca J: The hepatopulmonary syndrome: new name, old complexities. Thorax 47:897-902, 1992

    Google Scholar 

  2. Lange PA, Stoller JK: The hepatopulmonary syndrome. Ann Intern Med 122:521-529, 1995

    Google Scholar 

  3. Castro M, Krowka MJ: Hepatopulmonary syndrome: a pulmonary vascular complication of liver disease. Clin Chest Med 17:35-48, 1996

    Google Scholar 

  4. Krowka MJ, Tajik AJ, Dickson ER, Wiesner RH, Cortese DA: Intrapulmonary vascular dilatations (IPVD) in liver transplant candidates: screening by two-dimensional contrast-enhanced echocardiography. Chest 97:1165-1170, 1990

    Google Scholar 

  5. 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 109:1283-1288, 1995

    Google Scholar 

  6. Regev A, Yeshurun M, Rodriguez M, Sagie A, Neff GW, Molina EG, Schiff ER: Transient hepatopulmonary syndrome in a patient with acute hepatitis A. J Viral Hep 8:83-86, 2001

    Google Scholar 

  7. Scott VL, Dodson SF, Kang Y: The hepatopulmonary syndrome. Surg Clin North Am 79:23-41, 1999

    Google Scholar 

  8. Marchand EJ, De Jesus M, Biascoechea R: Cyanotic syndrome of portal hypertension in hepatosplenic schistosomiasis and portal cirrhosis. Am J Cardiol 10:496-506, 1962

    Google Scholar 

  9. Babbs C, Warnes TW, Haboubi NY: Noncirrhotic portal hypertension with hypoxaemia. Gut 29:129-131, 1988

    Google Scholar 

  10. Pugh RHN, Murray-Lyon IM, Dawson JL, Pietroni MC, Williams R: Transection of the oesophagus for bleeding oesophageal varices. Br J Surg 60:646-649, 1973

    Google Scholar 

  11. Okuda K: Idiopathic portal hypertension. In Recent Advances In Hepatology, 2nd ed. HC Thomas, EA Jones (eds). Edinburgh, Churchill Livingstone, 1986, pp 93-108

    Google Scholar 

  12. Kerr DN, Harrison CV, Sherlock S, Walker RM: Congenital hepatic fibrosis. Q J Med 30:91-117, 1961

    Google Scholar 

  13. Rodriguez-Roisin R, Roca J, Agusti AG, Mastai R, Wagner PD, Bosch J: Gas exchange and pulmonary vascular reactivity in patients with liver cirrhosis. Am Rev Respir Dis 135:1085-1092, 1987

    Google Scholar 

  14. Wolfe JD, Tashkin DP, Holly FE, Brachman MB, Genovesi MG: Hypoxemia of cirrhosis: detection of abnormal small pulmonary vascular channels by a quantitative radionuclide method. Am J Med 63:746-754, 1977

    Google Scholar 

  15. Kennedy KC, Knudson RJ: Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis. Chest 72:305-309, 1977

    Google Scholar 

  16. Krowka MJ, Cortese DA: Hepatopulmonary syndrome: current concepts in diagnostic and therapeutic considerations. Chest 105:1528-1537, 1994

    Google Scholar 

  17. Krowka MJ, Dickson ER, Cortese DA: Hepatopulmonary syndrome: clinical observations and lack of therapeutic response to somatostatin analogue. Chest 104:515-521, 1993

    Google Scholar 

  18. 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 114:305-310, 1998

    Google Scholar 

  19. Vachiery F, Moreau 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 27:492-495, 1997

    Google Scholar 

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

    Google Scholar 

  21. 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 272:G779-G784, 1997

    Google Scholar 

  22. Zhang M, Luo B, Chen S-J, Abrams GA, Fallon MB: Endothelin-1 stimulation of endothelial nitric oxide synthase in the pathogenesis of hepatopulmonary syndrome. Am J Physiol 277:G944-G952, 1999

    Google Scholar 

  23. Luo B, Abrams GA, Fallon MB: Endothelin-1 in the rat bile duct ligation model of hepatopulmonary syndrome: correlation with pulmonary dysfunction. J Hepatol 29:571-578, 1998

    Google Scholar 

Download references

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kaymakoglu, S., Kahraman, T., Kudat, H. et al. Hepatopulmonary Syndrome in Noncirrhotic Portal Hypertensive Patients. Dig Dis Sci 48, 556–560 (2003). https://doi.org/10.1023/A:1022549018807

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1023/A:1022549018807

Navigation