Subscribe to RSS
DOI: 10.1055/s-0032-1301730
Hepatopulmonary Syndrome
Publication History
Publication Date:
23 March 2012 (online)
![](https://www.thieme-connect.de/media/srccm/201201/lookinside/thumbnails/10.1055-s-0032-1301730-1.jpg)
Abstract
Hepatopulmonary syndrome (HPS) is characterized by an oxygenation defect induced by pulmonary vascular dilatation in the setting of liver cirrhosis or portal hypertension. It is defined by an alveolar–arterial gradient > 15 mm Hg measured at sea level. This syndrome is seen in 15 to 30% of cirrhotic patients and has been associated with worse survival. Most HPS patients are either asymptomatic or develop the insidious onset of dyspnea. The key event in its pathogenesis is the development of intrapulmonary vascular dilatation (IPVD), which has been linked to increased pulmonary levels of nitric oxide. Pulse oximetry is a useful screening test for HPS, which can guide subsequent use of arterial blood gases. Contrast-enhanced transthoracic echocardiography is the most effective test to demonstrate IPVD. Another method for detecting IPVD is the radionuclide lung perfusion scanning, using technetium-labeled macroaggregated albumin particles. Liver transplantation is the only available treatment for HPS, resulting in complete resolution or significant improvement in gas exchange in over 85% of patients.
-
References
- 1 Rodríguez-Roisin R, Krowka MJ, Hervé P, Fallon MB ; ERS Task Force Pulmonary-Hepatic Vascular Disorders (PHD) Scientific Committee. Pulmonary-hepatic vascular disorders (PHD). Eur Respir J 2004; 24 (5) 861-880
- 2 Martinez G, Barberà JA, Navasa M, Roca J, Visa J, Rodriguez-Roisin R. Hepatopulmonary syndrome associated with cardiorespiratory disease. J Hepatol 1999; 30 (5) 882-889
- 3 Schraufnagel DE, Kay JM. Structural and pathologic changes in the lung vasculature in chronic liver disease. Clin Chest Med 1996; 17 (1) 1-15
- 4 Degano B, Mittaine M, Hervé P , et al. Nitric oxide production by the alveolar compartment of the lungs in cirrhotic patients. Eur Respir J 2009; 34 (1) 138-144
- 5 Delclaux C, Mahut B, Zerah-Lancner F , et al. Increased nitric oxide output from alveolar origin during liver cirrhosis versus bronchial source during asthma. Am J Respir Crit Care Med 2002; 165 (3) 332-337
- 6 Rolla G, Brussino L, Colagrande P , et al. Exhaled nitric oxide and impaired oxygenation in cirrhotic patients before and after liver transplantation. Ann Intern Med 1998; 129 (5) 375-378
- 7 Fallon MB, Abrams GA, Luo B, Hou Z, Dai J, Ku DD. The role of endothelial nitric oxide synthase in the pathogenesis of a rat model of hepatopulmonary syndrome. Gastroenterology 1997; 113 (2) 606-614
- 8 Ling Y, Zhang J, Luo B , et al. The role of endothelin-1 and the endothelin B receptor in the pathogenesis of hepatopulmonary syndrome in the rat. Hepatology 2004; 39 (6) 1593-1602
- 9 Carter EP, Hartsfield CL, Miyazono M, Jakkula M, Morris Jr KG, McMurtry IF. Regulation of heme oxygenase-1 by nitric oxide during hepatopulmonary syndrome. Am J Physiol Lung Cell Mol Physiol 2002; 283 (2) L346-L353
- 10 Zhang J, Ling Y, Luo B , et al. Analysis of pulmonary heme oxygenase-1 and nitric oxide synthase alterations in experimental hepatopulmonary syndrome. Gastroenterology 2003; 125 (5) 1441-1451
- 11 Zhang J, Luo B, Tang L , et al. Pulmonary angiogenesis in a rat model of hepatopulmonary syndrome. Gastroenterology 2009; 136 (3) 1070-1080
- 12 Fallon MB, Krowka MJ, Brown RS , et al; Pulmonary Vascular Complications of Liver Disease Study Group. Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterology 2008; 135 (4) 1168-1175
- 13 Martínez GP, Barberà JA, Visa J , et al. Hepatopulmonary syndrome in candidates for liver transplantation. J Hepatol 2001; 34 (5) 651-657
- 14 Schenk P, Schöniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Müller C. Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology 2003; 125 (4) 1042-1052
- 15 Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatology 2005; 41 (5) 1122-1129
- 16 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 (4) 1283-1288
- 17 Tanikella R, Kochar R, Kawut SM, Machicao VI, Fallon MB. Intrapulmonary vasodilatation and survival in liver transplant candidates [abstract]. Hepatology 2010; 52 (S1) A1207
- 18 Palma DT, Philips GM, Arguedas MR, Harding SM, Fallon MB. Oxygen desaturation during sleep in hepatopulmonary syndrome. Hepatology 2008; 47 (4) 1257-1263
- 19 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 1977; 63 (5) 746-754
- 20 Lima BL, França AV, Pazin-Filho A , et al. Frequency, clinical characteristics, and respiratory parameters of hepatopulmonary syndrome. Mayo Clin Proc 2004; 79 (1) 42-48
- 21 Abrams GA, Sanders MK, Fallon MB. Utility of pulse oximetry in the detection of arterial hypoxemia in liver transplant candidates. Liver Transpl 2002; 8 (4) 391-396
- 22 Arguedas MR, Singh H, Faulk DK, Fallon MB. Utility of pulse oximetry screening for hepatopulmonary syndrome. Clin Gastroenterol Hepatol 2007; 5 (6) 749-754
- 23 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 1998; 114 (2) 305-310
- 24 Roberts DN, Arguedas MR, Fallon MB. Cost-effectiveness of screening for hepatopulmonary syndrome in liver transplant candidates. Liver Transpl 2007; 13 (2) 206-214
- 25 Sztrymf B, Rabiller A, Nunes H , et al. Prevention of hepatopulmonary syndrome and hyperdynamic state by pentoxifylline in cirrhotic rats. Eur Respir J 2004; 23 (5) 752-758
- 26 Zhang J, Ling Y, Tang L , et al. Pentoxifylline attenuation of experimental hepatopulmonary syndrome. J Appl Physiol 2007; 102 (3) 949-955
- 27 Tanikella R, Philips GM, Faulk DK, Kawut SM, Fallon MB. Pilot study of pentoxifylline in hepatopulmonary syndrome. Liver Transpl 2008; 14 (8) 1199-1203
- 28 Gupta LB, Kumar A, Jaiswal AK , et al. Pentoxifylline therapy for hepatopulmonary syndrome: a pilot study. Arch Intern Med 2008; 168 (16) 1820-1823
- 29 Gupta S, Faughnan ME, Lilly L, Hutchison S, Fowler R, Bayoumi AM. Norfloxacin therapy for hepatopulmonary syndrome: a pilot randomized controlled trial. Clin Gastroenterol Hepatol 2010; 8 (12) 1095-1098
- 30 Abrams GA, Fallon MB. Treatment of hepatopulmonary syndrome with Allium sativum L. (garlic): a pilot trial. J Clin Gastroenterol 1998; 27 (3) 232-235
- 31 Najafi Sani M, Kianifar HR, Kianee A, Khatami G. Effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome. World J Gastroenterol 2006; 12 (15) 2427-2431
- 32 De BK, Dutta D, Pal SK, Gangopadhyay S, Das Baksi S, Pani A. The role of garlic in hepatopulmonary syndrome: a randomized controlled trial. Can J Gastroenterol 2010; 24 (3) 183-188
- 33 Krowka MJ, Mandell MS, Ramsay MA , et al. Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database. Liver Transpl 2004; 10 (2) 174-182
- 34 Arguedas MR, 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 (1) 192-197
- 35 Gupta S, Castel H, Rao RV , et al. Improved survival after liver transplantation in patients with hepatopulmonary syndrome. Am J Transplant 2010; 10 (2) 354-363
- 36 Sulieman BM, Hunsicker LG, Katz DA, Voigt MD. OPTN policy regarding prioritization of patients with hepatopulmonary syndrome: does it provide equitable organ allocation?. Am J Transplant 2008; 8 (5) 954-964