Abstract
The hepatopulmonary syndrome (HPS) occurs in as many as 15–20% of patients with cirrhosis; mortality is significantly increased compared to cirrhotic patients without HPS. The only proven effective therapy for HPS is orthotopic liver transplantation (OLT), which should be considered when severe hypoxemia is present. The natural history of HPS without liver transplantation is dismal. While post-OLT mortality is increased in patients with HPS relative to that reported in non-HPS patients, overall outcomes are favorable in properly selected patients. The higher mortality associated with HPS has led to the policy of increasing priority for OLT in selected HPS patients through a Model for End-Stage Liver Disease (MELD) score exception. There is currently no established protocol to screen for HPS in OLT candidates. However, a resting PaO2 < 65–60 mmHg identifies patients who qualify, or who may sufficiently deteriorate over a short time frame to qualify, for MELD exception criteria. In patients with HPS awaiting OLT, no specific therapies are available to improve intrapulmonary vasodilatation. The perioperative management of HPS patients presents particular clinical challenges.
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References
Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB, on behalf of the ERS Task Force Pulmonary-Hepatic Vascular Disorders Scientific Committee ERS Task Force PHD Scientific Committee. Pulmonary-hepatic vascular disorders (PHD). Eur Respir J 2004; 24:861–80.
Fallon MB, Abrams GA. Pulmonary dysfunction in chronic liver disease. Hepatology 2000; 32:859–65.
Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: Impact of liver transplantation. Hepatology 2005; 41:1122–9.
Schenk P, Schoniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Muller C. Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology 2003; 125:1042–52.
Krowka MJ, Dickson ER, Cortese DA. Hepatopulmonary syndrome: Clinical observations and lack of therapeutic response to somatostatin analogue. Chest 1993; 104:515–21.
Saunders KB, Fernando SSD, Dalton HR, Joseph A. Spontaneous improvement in a patient with the hepatopulmonary syndrome assessed by serial exercise tests. Thorax 1994; 49:725–7.
Herve P, Lebrec D, Brenot F, Simonneau G, Humbert M, Sitbon O, et al. Pulmonary vascular disorders in portal hypertension. Eur Respir J 1998; 11:1153–66.
Krowka MJ. Hepatopulmonary syndromes. Gut 2000; 40:1–4.
Lange PA, Stoller JK. The hepatopulmonary syndrome: Effect of liver transplantation. Clin Chest Med 1996; 17:115–23.
Philit F, Wiesendanger T, Gille D, Boillot O, Cordier JF. Late resolution of hepatopulmonary syndrome after liver transplantation. Respiration 1997; 6:173–175A.
Arguedas M, Abrams GA, Krowka MJ, Fallon MB. Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation. Hepatology 2003; 37:192–7.
Kaspar MD, Ramsay MA, Shuey CB, Levy MF, Klintmalm GG. Severe pulmonary hypertension and amelioration of hepatopulmonary syndrome after liver transplantation. Liver Transpl Surg 1998; 4:177–9.
Martinez-Palli G, Barbera JA, Taura P, Cirera I, Visa J, Rodriguez-Roisin R. Severe portopulmonary hypertension after liver transplantation in a patient with preexisting hepatopulmonary syndrome. J Hepatol 1999; 31:1075–9.
Corley DA, Scharschmidt B, Bass N, Somberg K, Gold W. Lack of efficacy of TIPS for hepatopulmonary syndrome. Gastroenterology 1997; 113:728–31.
Mandell MS, Groves BM, Duke J. Progressive plexogenic pulmonary hypertension following liver transplantation. Transplantation 1995; 59:1488–90.
Abrams GA, Rose K, Fallon MB, McGuire BM, Bloomer JR, van Leeuwen DJ, et al. Hepatopulmonary syndrome and venous emboli causing intracerebral hemorrhages after liver transplantation: A case report. Transplantation 1999; 68:1–3.
Taille C, Cadranel J, Bellocq A, Thabut G, Soubrane O, Durand F, Ichai P, et al. Liver transplantation for hepatopulmonary syndrome: A ten-year experience in Paris, France. Transplantation 2003; 79:1482–9.
Collison EA, Nourmand H, Fraiman MH. Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome. Liver Transpl 2002; 8:925–31.
Schiffer E, Majno P, Mentha G, Giostra E, Burri H, Klopfenstein E, et al. Hepatopulmonary syndrome increases the postoperative mortality rate following liver transplantation: A prospective study in 90 patients. Am J Transpl 2006; 6:1430–7.
Sulieman B, Voigt M, Katz D, Hunsicker L. Data driven policy for points allocation in hepatopulmonary syndrome. Hepatology 2005; 42:347A.
Schenk P, Fuhrmann V, Madl C, Funk G, Lehr S, Kandel O, Muller C. Hepatopulmonary syndrome: Prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences. Gut. 2002; 51:853–9.
Krowka MJ, Mandell S, Ramsay MA, Kawut S, Fallon MB, Manzarbeitia C, Pardo M, et al. Hepatopulmonary syndrome and portopulmonary hypertension: A report of the Multicenter Liver Transplant Database. Liver Transpl 2004; 10:174–82.
Martinez G, Barbera J, Visa J, Rimola A, Parc J, Roca J, Navasa M, et al. Hepatopulmonary syndrome in candidates for liver transplantation. J Hepatol 2001; 34:756–8.
Abrams GA, Sanders MK, Fallon MB. Utility of pulse oximetry in the detection of arterial hypoxemia in liver transplant candidates. Liver Transpl 2002; 8:391–6.
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 1995; 109:1283–8.
Abrams G, Nanda N, Dubovsky E, Krowka M, Fallon M. Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome: A new approach. Gastroenterology 1998; 114: 305–10.
Abrams GA, Fallon MB. Treatment of hepatopulmonary syndrome with Allium sativum (garlic): A pilot trial. J Clin Gastroenterol 1998; 27:232–5.
Anel RM, Sheagren JN. Novel presentation and approach to management of hepatopulmonary syndrome with use of antimicrobial agents. Clin Infect Dis 2001; 32:E131–E136A.
Rabiller A, Nunes H, Lebrec D, Tazi KA, Wartski M, Dulmet E, et al. Prevention of Gram-negative translocation reduces the severity of hepatopulmonary syndrome. Am J Respir Crit Care Med 2002; 166:514–7.
Meyers C, Low L, Kaufman L, Druger G, Wong LL. Trendelenburg positioning and continuous lateral rotation improve oxygenation in hepatopulmonary syndrome after liver transplantation. Liver Transpl Surg 1998; 6:510–2.
Alexander J, Greenough A, Baker A, Rela M, Heaton N, Potter D. Nitric oxide treatment of severe hypoxemia after liver transplantation in hepatopulmonary syndrome. Liver Transpl Surg 1997; 3:54–5.
Durand P, Baujard C, Grosse AL. Reversal of hypoxemia by inhaled nitric oxide in children with severe hepatopulmonary syndrome, type 1, during and after liver transplantation. Transplantation 1998; 65:437–9.
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Palma, D.T., Fallon, M.B. (2009). Liver Transplantation and the Hepatopulmonary Syndrome. In: Trotter, J., Everson, G. (eds) Liver Transplantation. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-60327-028-1_7
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DOI: https://doi.org/10.1007/978-1-60327-028-1_7
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