Hepatopulmonary syndrome historical perspective

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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

  • My refernces

[1] [2] [3] 4. Flückiger M. Vorkommen von trommelschlagel-formigen fingerendphalangen ohne chronische veranderungeng an den lungen oder am herzen. Wien Med Wochenschr. 1884;34:1457. [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23] [24] [25] [26] [27] [28] [29] [30] [31] [32] [33] [34] [35] [36] [37] [38] [39] [40] [41] [42]


Historical Perspective

Discovery

The first recorded description of a low level of oxygen in the blood () with was by in . It was not until nearly a century later,, that , and coined the term “” 5.

  • In 1884, Flückiger was the first to report the association between liver dysfunction and the development of hypoxemia.
  • The term "hepatopulmonary syndrome" was first suggested by Kennedy and Knudson almost 100 years later,in 1977 during describing a patient with the classic findings of hepatopulmonary syndrome.

Landmark Events in the Development of Treatment Strategies

  • Currently the only definitive treatment is liver transplantation. Alternative treatments such as supplemental oxygen or somatostatin to inhibit vasodilation remain anecdotal.
  • Here are the landmarks of liver transplantation pathway.
  • In the 1960s, Thomas Starzl used dogs as the first animals for research on liver transplantation in Boston and Chicago.
  • In 1963, the first liver transplant in humans was attempted by a surgical team led by Dr. Thomas Starzl of Denver, Colorado, United States.
  • Dr. Starzl performed many additional transplants until he was successful in 1967 with the first one-year survival post-transplantation.
  • In 1970, the regimen for immunosuppressive therapy following transplant was introduced, but azathioprine and steroids did not improve survival rates of patients.
  • In the 1980s, with the introduction of cyclosporine by Sir Roy Calne, there was an improvement in rejection rates.
  • In 1983, liver transplantation was no longer an experimental modality, but a clinically acceptable form of therapy for both adult and pediatric patients with appropriate indications.
  • In 1986, the introduction of monoclonal antibodies such as muromonab-CD3 [OKT3] further contributed to improvement of quality of immunosuppressive therapy used in patients, with significant decline in rejection rates.
  • In 1988, University of Wisconsin (UW) solution was developed, which ensured a smooth surgery and longer preservation period.
  • In 1992, the concept of xenotransplantation and cloning techniques were introduced by Starzl.
  • In 1999, approximately 5000 procedures were carried out, in contrast to 100 which had been performed a decade earlier.
  • Recently, the introduction of newer immunosuppressive agents such as IL-2 receptor blockers and tacrolimus, have drastically increased patient survival ratesto 1 and 5-year rates of approximately 85 and 70 percent respectively.
  • In December 2016, 147,128 liver transplants were performed in the US as compared to 7217 in 1998 based on data from the United Organ Sharing (UNOS) network.


Impact on Cultural History

Famous Cases

The following are a few famous cases of [disease name]:

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. ↑ Rodríguez-Roisin R, Agustí AG, Roca J (1992) The hepatopulmonary syndrome: new name, old complexities. Thorax 47 (11):897-902. DOI:10.1136/thx.47.11.897 PMID: 1465744
  9. ↑ Cremona G, Higenbottam TW, Mayoral V, Alexander G, Demoncheaux E, Borland C et al. (1995) Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. Eur Respir J 8 (11):1883-5. PMID: 8620957
  10. ↑ Rolla G, Brussino L, Colagrande P, Dutto L, Polizzi S, Scappaticci E et al. (1997) Exhaled nitric oxide and oxygenation abnormalities in hepatic cirrhosis. Hepatology 26 (4):842-7. DOI:10.1053/jhep.1997.v26.pm0009328302 PMID: 9328302
  11. ↑ Nunes H, Lebrec D, Mazmanian M, Capron F, Heller J, Tazi KA et al. (2001) Role of nitric oxide in hepatopulmonary syndrome in cirrhotic rats. Am J Respir Crit Care Med 164 (5):879-85. DOI:10.1164/ajrccm.164.5.2009008 PMID: 11549549
  12. ↑ Luo B, Abrams GA, Fallon MB (1998) Endothelin-1 in the rat bile duct ligation model of hepatopulmonary syndrome: correlation with pulmonary dysfunction. J Hepatol 29 (4):571-8. PMID: 9824266
  13. ↑ Zhang J, Yang W, Hu B, Wu W, Fallon MB (2014) Endothelin-1 activation of the endothelin B receptor modulates pulmonary endothelial CX3CL1 and contributes to pulmonary angiogenesis in experimental hepatopulmonary syndrome. Am J Pathol 184 (6):1706-14. DOI:10.1016/j.ajpath.2014.02.027 PMID: 24731444
  14. ↑ Gómez FP, Barberà JA, Roca J, Burgos F, Gistau C, Rodríguez-Roisin R (2006) Effects of nebulized N(G)-nitro-L-arginine methyl ester in patients with hepatopulmonary syndrome. Hepatology 43 (5):1084-91. DOI:10.1002/hep.21141 PMID: 16628648
  15. ↑ Schenk P, Fuhrmann V, Madl C, Funk G, Lehr S, Kandel O et al. (2002) Hepatopulmonary syndrome: prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences. Gut 51 (6):853-9. DOI:10.1136/gut.51.6.853 PMID: 12427789
  16. ↑ Abrams GA, Jaffe CC, Hoffer PB, Binder HJ, Fallon MB (1995) Diagnostic utility of contrast echocardiography and lung perfusion scan in patients with hepatopulmonary syndrome. Gastroenterology 109 (4):1283-8. PMID: 7557096
  17. ↑ Lima BL, França AV, Pazin-Filho A, Araújo WM, Martinez JA, Maciel BC et al. (2004) Frequency, clinical characteristics, and respiratory parameters of hepatopulmonary syndrome. Mayo Clin Proc 79 (1):42-8. DOI:10.4065/79.1.42 PMID: 14708947
  18. ↑ Martínez GP, Barberà JA, Visa J, Rimola A, Paré JC, Roca J et al. (2001) Hepatopulmonary syndrome in candidates for liver transplantation. J Hepatol 34 (5):651-7. PMID: 11434610
  19. ↑ Schenk P, Schöniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Müller C (2003) Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology 125 (4):1042-52. PMID: 14517788
  20. ↑ Hopkins WE, Waggoner AD, Barzilai B (1992) Frequency and significance of intrapulmonary right-to-left shunting in end-stage hepatic disease. Am J Cardiol 70 (4):516-9. DOI:10.1016/0002-9149(92)91200-n PMID: 1642191
  21. ↑ Arguedas MR, Singh H, Faulk DK, Fallon MB (2007) Utility of pulse oximetry screening for hepatopulmonary syndrome. Clin Gastroenterol Hepatol 5 (6):749-54. DOI:10.1016/j.cgh.2006.12.003 PMID: 17392034
  22. ↑ Rodríguez-Roisin R, Krowka MJ, Hervé P, Fallon MB, ERS Task Force Pulmonary-Hepatic Vascular Disorders (PHD) Scientific Committee (2004) Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J 24 (5):861-80. DOI:10.1183/09031936.04.00010904 PMID: 15516683
  23. ↑ Castro M, Krowka MJ (1996) Hepatopulmonary syndrome. A pulmonary vascular complication of liver disease. Clin Chest Med 17 (1):35-48. PMID: 8665789
  24. ↑ Hurtado-Cordovi JM, Lipka S, Singh J, Shahzad G, Mustacchia P (2011) Diagnostic challenge of hepatopulmonary syndrome in a patient with coexisting structural heart disease. Case Reports Hepatol 2011 ():386709. DOI:10.1155/2011/386709 PMID: 25954542
  25. ↑ Abrams GA, Nanda NC, Dubovsky EV, Krowka MJ, Fallon MB (1998) Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome: a new approach. Gastroenterology 114 (2):305-10. PMID: 9453490
  26. ↑ Chen YA, Prabhudesai V, Castel H, Gupta S (2016) CT Scan Does Not Differentiate Patients with Hepatopulmonary Syndrome from Other Patients with Liver Disease. PLoS One 11 (7):e0158637. DOI:10.1371/journal.pone.0158637 PMID: 27384058
  27. ↑ 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
  28. ↑ 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
  29. ↑ 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
  30. ↑ 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
  31. ↑ 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
  32. ↑ 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
  33. ↑ 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
  34. ↑ 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
  35. ↑ 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
  36. ↑ 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
  37. ↑ Martinez-Palli G, Drake BB, Garcia-Pagan JC, Barbera JA, Arguedas MR, Rodriguez-Roisin R et al. (2005) Effect of transjugular intrahepatic portosystemic shunt on pulmonary gas exchange in patients with portal hypertension and hepatopulmonary syndrome. World J Gastroenterol 11 (43):6858-62. DOI:10.3748/wjg.v11.i43.6858 PMID: 16425397
  38. ↑ Skinner HA, Maley O, Norman CD (2006) Developing internet-based eHealth promotion programs: the Spiral Technology Action Research (STAR) model. Health Promot Pract 7 (4):406-17. DOI:10.1177/1524839905278889 PMID: 16840770
  39. ↑ Winker MA, Flanagin A, Chi-Lum B, White J, Andrews K, Kennett RL et al. (2000) Guidelines for medical and health information sites on the internet: principles governing AMA web sites. American Medical Association. JAMA 283 (12):1600-6. DOI:10.1001/jama.283.12.1600 PMID: 10735398
  40. ↑ Boyer C, Baujard V, Geissbuhler A (2011) Evolution of health web certification through the HONcode experience. Stud Health Technol Inform 169 ():53-7. PMID: 21893713
  41. ↑ Ghajar-Khosravi S, Tarlo SM, Liss GM, Chignell M, Ribeiro M, Levinson AJ et al. (2013) Development of a web-based, work-related asthma educational tool for patients with asthma. Can Respir J 20 (6):417-23. DOI:10.1155/2013/562104 PMID: 24137573
  42. ↑ Marianayagam J, Griffin K, Sykes J, Gupta S (2017) Development and Evaluation of an Online Resource for Hepatopulmonary Syndrome Patients and Caregivers: www.hpscare.com. Clin Gastroenterol Hepatol 15 (8):1302-1304. DOI:10.1016/j.cgh.2017.03.002 PMID: 28302500
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