Hepatopulmonary syndrome medical therapy

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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

There is no medical treatment for hepatopulmonary syndrome. The mainstay of treatment for hepatopulmonary syndrome is surgery. Orthotopic liver transplantation is the only available treatment for patients with hepatopulmonary syndrome. Supportive therapy for hepatopulmonary syndrome includes oxygen therapy.

Medical Therapy

  • There is no medical treatment for hepatopulmonary syndrome.[1]

[2][3][4][5][6][7]

  • The mainstay of treatment for hepatopulmonary syndrome is surgery.
  • Orthotopic liver transplantation is the only available treatment for patients with hepatopulmonary syndrome.
  • Supportive therapy for hepatopulmonary syndrome includes oxygen therapy.
  • Nevertheless, norfloxacin, pentoxifylline, inhaled N(G)-nitro-L-arginine methyl ester, nitric oxide inhalation and methylene blue injection, are among suggested and studied medical treatments for HPS. Most of them failed to show efficacy and hence, non of them yet approved as a standard treatment for HPS.[8][9][10][11][12][13]

References

  1. 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
  2. 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
  3. 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
  4. 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
  5. 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
  6. Krowka MJ, Dickson ER, Cortese DA (1993) Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue. Chest 104 (2):515-21. DOI:10.1378/chest.104.2.515 PMID: 8101797
  7. 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
  8. Gupta S, Faughnan ME, Lilly L, Hutchison S, Fowler R, Bayoumi AM (2010) Norfloxacin therapy for hepatopulmonary syndrome: a pilot randomized controlled trial. Clin Gastroenterol Hepatol 8 (12):1095-8. DOI:10.1016/j.cgh.2010.08.011 PMID: 20816858
  9. Gupta LB, Kumar A, Jaiswal AK, Yusuf J, Mehta V, Tyagi S et al. (2008) Pentoxifylline therapy for hepatopulmonary syndrome: a pilot study. Arch Intern Med 168 (16):1820-3. DOI:10.1001/archinte.168.16.1820 PMID: 18779471
  10. Tanikella R, Philips GM, Faulk DK, Kawut SM, Fallon MB (2008) Pilot study of pentoxifylline in hepatopulmonary syndrome. Liver Transpl 14 (8):1199-203. DOI:10.1002/lt.21482 PMID: 18668653
  11. Brussino L, Bucca C, Morello M, Scappaticci E, Mauro M, Rolla G (2003) Effect on dyspnoea and hypoxaemia of inhaled N(G)-nitro-L-arginine methyl ester in hepatopulmonary syndrome. Lancet 362 (9377):43-4. DOI:10.1016/S0140-6736(03)13807-X PMID: 12853200
  12. Schenk P, Madl C, Rezaie-Majd S, Lehr S, Müller C (2000) Methylene blue improves the hepatopulmonary syndrome. Ann Intern Med 133 (9):701-6. DOI:10.7326/0003-4819-133-9-200011070-00012 PMID: 11074903
  13. Jounieaux V, Leleu O, Mayeux I (2001) Cardiopulmonary effects of nitric oxide inhalation and methylene blue injection in hepatopulmonary syndrome. Intensive Care Med 27 (6):1103-4. PMID: 11497151

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