Hepatopulmonary syndrome surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

Surgery is the mainstay of treatment for hepatopulmonary syndrome. Orthotopic liver transplantation is the only available treatment for patients with hepatopulmonary syndrome.

Indications

  • There are a number of indications for liver transplantation in cirrhosis divided to relative and absolute indications.
  • Among them, severe cardiac and/or pulmonary diseases and severe pulmonary hypertension (mPAP >45 mm Hg) is an absolute indication.
  • Plenty of patients who develop HPS are already candied for liver transplantation.

Surgery

[1][2] [3][4] [5][6] [7][8][9][10]

Complications

  • Severe post-transplant hypoxemia and subsequent prolonged mechanical ventilation
  • Post-operative infections
  • Anastomotic bile duct leaks due to delayed wound healing because of hypoxemia
  • Post-operative portal vein and hepatic artery thrombosis due to polycythemia induced by chronic hypoxemia
  • Post-transplant HPS recurrence because of graft dysfunction
  • Progressive pulmonary hypertension

References

  1. Swanson KL, Wiesner RH, Krowka MJ (2005) Natural history of hepatopulmonary syndrome: Impact of liver transplantation. Hepatology 41 (5):1122-9. DOI:10.1002/hep.20658 PMID: 15828054
  2. Nayyar D, Man HS, Granton J, Gupta S (2014) Defining and characterizing severe hypoxemia after liver transplantation in hepatopulmonary syndrome. Liver Transpl 20 (2):182-90. DOI:10.1002/lt.23776 PMID: 24142412
  3. Nayyar D, Man HS, Granton J, Lilly LB, Gupta S (2015) Proposed management algorithm for severe hypoxemia after liver transplantation in the hepatopulmonary syndrome. Am J Transplant 15 (4):903-13. DOI:10.1111/ajt.13177 PMID: 25649047
  4. Park TJ, Ahn KS, Kim YH, Kim H, Park UJ, Kim HT et al. (2014) Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia. Clin Mol Hepatol 20 (1):76-80. DOI:10.3350/cmh.2014.20.1.76 PMID: 24757662
  5. Egawa H, Kasahara M, Inomata Y, Uemoto S, Asonuma K, Fujita S et al. (1999) Long-term outcome of living related liver transplantation for patients with intrapulmonary shunting and strategy for complications. Transplantation 67 (5):712-7. PMID: 10096527
  6. Rodríguez-Roisin R, Krowka MJ (2008) Hepatopulmonary syndrome--a liver-induced lung vascular disorder. N Engl J Med 358 (22):2378-87. DOI:10.1056/NEJMra0707185 PMID: 18509123
  7. Fallon MB, Abrams GA (2000) Pulmonary dysfunction in chronic liver disease. Hepatology 32 (4 Pt 1):859-65. DOI:10.1053/jhep.2000.7519 PMID: 11003635
  8. Krowka MJ, Mandell MS, Ramsay MA, Kawut SM, Fallon MB, Manzarbeitia C et al. (2004) Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database. Liver Transpl 10 (2):174-82. DOI:10.1002/lt.20016 PMID: 14762853
  9. Kennedy TC, Knudson RJ (1977) Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis. Chest 72 (3):305-9. DOI:10.1378/chest.72.3.305 PMID: 891282
  10. Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA et al. (2016) International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation 100 (7):1440-52. DOI:10.1097/TP.0000000000001229 PMID: 27326810

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