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Expert Review of Clinical Pharmacology Volume 7, 2014 - Issue 1
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Therapeutic options in pulmonary hepatic vascular diseases

Thomas HorvatitsDepartment of Internal Medicine 3, Division of Gastroenterology and Hepatology, Medical University Vienna, Austria
&
Valentin FuhrmannDepartment of Internal Medicine 3, Division of Gastroenterology and Hepatology, Medical University Vienna, AustriaCorrespondence[email protected]
Pages 31-42 | Published online: 25 Nov 2013

References

  • Hourani JM, Bellamy PE, Tashkin DP, Batra P, Simmons MS. Pulmonary dysfunction in advanced liver disease: frequent occurrence of an abnormal diffusing capacity. Am. J. Med. 90, 693–700 (1991).
  • Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB. Pulmonary-Hepatic vascular Disorders (PHD). Eur. Respir. J. 24, 861–880 (2004).
  • Rodriguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome--a liver-induced lung vascular disorder. N. Engl. J. Med. 358, 2378–2387 (2008).
  • Schenk P, Fuhrmann V, Madl C et al. Hepatopulmonary syndrome: prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences. Gut 51, 853–859 (2002).
  • Berthelot P, Walker JG, Sherlock S, Reid L. Arterial changes in the lungs in cirrhosis of the liver--lung spider nevi. N. Engl. J. Med. 274, 291–298 (1966).
  • Schraufnagel DE, Kay JM. Structural and pathologic changes in the lung vasculature in chronic liver disease. Clin. Chest Med. 17, 1–15 (1996).
  • Gomez FP, Martinez-Palli G, Barbera JA, Roca J, Navasa M, Rodriguez-Roisin R. Gas exchange mechanism of orthodeoxia in hepatopulmonary syndrome. Hepatology 40, 660–666 (2004).
  • Rodriguez-Roisin R, Roca J, Agusti AG, Mastai R, Wagner PD, Bosch J. Gas exchange and pulmonary vascular reactivity in patients with liver cirrhosis. Am. Rev. Respir. Dis. 135, 1085–1092 (1987).
  • Moudgil R, Michelakis ED, Archer SL. Hypoxic pulmonary vasoconstriction. J. Appl. Physiol. 98, 390–403 (2005).
  • Schenk P, Schoniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Muller C. Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology 125, 1042–1052 (2003).
  • Fallon MB, Krowka MJ, Brown RS et al. Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterology 135, 1168–1175 (2008).
  • Fuhrmann V, Madl C, Mueller C et al. Hepatopulmonary syndrome in patients with hypoxic hepatitis. Gastroenterology 131, 69–75 (2006).
  • Regev A, Yeshurun M, Rodriguez M et al. Transient hepatopulmonary syndrome in a patient with acute hepatitis A. J. Viral Hepat. 8, 83–86 (2001).
  • De BK, Sen S, Biswas PKet al. Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression. Gastroenterology 122, 897–903 (2002).
  • Law YM, Mack CL, Sokol RJ, Rice M, Parsley L, Ivy D. Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: pulmonary hypertension and pulmonary vascular dilatation. Pediatr. Transplant. 15, E162–E168 (2011).
  • 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 109, 1283–1288 (1995).
  • Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: Impact of liver transplantation. Hepatology 41, 1122–1129 (2005).
  • Arguedas MR, Singh H, Faulk DK, Fallon MB. Utility of pulse oximetry screening for hepatopulmonary syndrome. Clin. Gastroenterol. Hepatol. 5, 749–754 (2007).
  • Krowka MJ, Tajik AJ, Dickson ER, Wiesner RH, Cortese DA. Intrapulmonary vascular dilatations (IPVD) in liver transplant candidates. Screening by two-dimensional contrast-enhanced echocardiography. Chest 97, 1165–1170 (1990).
  • Vedrinne JM, Duperret S, Bizollon T et al. Comparison of transesophageal and transthoracic contrast echocardiography for detection of an intrapulmonary shunt in liver disease. Chest 111, 1236–1240 (1997).
  • 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 114, 305–310 (1998).
  • Martinez G, Barbera JA, Navasa M, Roca J, Visa J, Rodriguez-Roisin R. Hepatopulmonary syndrome associated with cardiorespiratory disease. J. Hepatol. 30, 882–889 (1999).
  • Pham DM, Subramanian R, Parekh S. Coexisting hepatopulmonary syndrome and portopulmonary hypertension: implications for liver transplantation. J. Clin. Gastroenterol. 44, e136–e140 (2010).
  • Rolla G, Brussino L, Colagrande P et al. Exhaled nitric oxide and oxygenation abnormalities in hepatic cirrhosis. Hepatology 26, 842–847 (1997).
  • La Villa G, Barletta G, Pantaleo P et al. Hemodynamic, renal, and endocrine effects of acute inhibition of nitric oxide synthase in compensated cirrhosis. Hepatology 34, 19–27 (2001).
  • Cremona G, Higenbottam TW, Mayoral V et al. Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. Eur. Respir. J. 8, 1883–1885 (1995).
  • Koch DG, Bogatkevich G, Ramshesh V, Lemasters JJ, Uflacker R, Reuben A. Elevated levels of endothelin-1 in hepatic venous blood are associated with intrapulmonary vasodilatation in humans. Dig. Dis. Sci. 57, 516–523 (2012).
  • 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 39, 1593–1602 (2004).
  • Rolla G, Bucca C, Brussino L. Methylene blue in the hepatopulmonary syndrome. N. Engl. J. Med. 331, 1098 (1994).
  • Schenk P, Madl C, Rezaie-Majd S, Lehr S, Muller C. Methylene blue improves the hepatopulmonary syndrome. Ann. Intern. Med. 133, 701–706 (2000).
  • Gomez FP, Barbera JA, Roca J, Burgos F, Gistau C, Rodriguez-Roisin R. Effects of nebulized N(G)-nitro-L-arginine methyl ester in patients with hepatopulmonary syndrome. Hepatology 43, 1084–1091 (2006).
  • Nunes H, Lebrec D, Mazmanian M et al. Role of nitric oxide in hepatopulmonary syndrome in cirrhotic rats. Am. J. Respir. Crit. Care Med. 164, 879–885 (2001).
  • Zhang J, Ling Y, Tang L, Luo B, Pollock DM, Fallon MB. Attenuation of experimental hepatopulmonary syndrome in endothelin B receptor-deficient rats. Am. J. Physiol. Gastrointest. Liver Physiol. 296, G704–G708 (2009).
  • Krug S, Seyfarth HJ, Hagendorff A, Wirtz H. Inhaled iloprost for hepatopulmonary syndrome: improvement of hypoxemia. Eur. J. Gastroenterol. Hepatol. 19, 1140–1143 (2007).
  • Sztrymf B, Rabiller A, Nunes H et al. Prevention of hepatopulmonary syndrome and hyperdynamic state by pentoxifylline in cirrhotic rats. Eur. Respir. J. 23, 752–758 (2004).
  • Liu L, Liu N, Zhao Z et al. TNF-alpha neutralization improves experimental hepatopulmonary syndrome in rats. Liver Int. 32, 1018–1026 (2012).
  • Gupta LB, Kumar A, Jaiswal AK et al. Pentoxifylline therapy for hepatopulmonary syndrome: a pilot study. Arch. Intern. med. 168, 1820–1823 (2008).
  • Tanikella R, Philips GM, Faulk DK, Kawut SM, Fallon MB. Pilot study of pentoxifylline in hepatopulmonary syndrome. Liver transpl. 14, 1199–1203 (2008).
  • Rabiller A, Nunes H, Lebrec D et al. Prevention of gram-negative translocation reduces the severity of hepatopulmonary syndrome. Am. J. Respir. Crit. Care Med. 166, 514–517 (2002).
  • 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. 8, 1095–1098 (2010).
  • Arguedas MR, Drake BB, Kapoor A, Fallon MB. Carboxyhemoglobin levels in cirrhotic patients with and without hepatopulmonary syndrome. Gastroenterology 128, 328–333 (2005).
  • Zhang J, Ling Y, Luo B et al. Analysis of pulmonary heme oxygenase-1 and nitric oxide synthase alterations in experimental hepatopulmonary syndrome. Gastroenterology 125, 1441–1451 (2003).
  • Sumanovski LT, Battegay E, Stumm M, van der Kooij M, Sieber CC. Increased angiogenesis in portal hypertensive rats: role of nitric oxide. Hepatology 29, 1044–1049 (1999).
  • Zhang J, Luo B, Tang L et al. Pulmonary angiogenesis in a rat model of hepatopulmonary syndrome. Gastroenterology 136, 1070–1080 (2009).
  • Zhang J, Yang W, Luo B, Hu B, Maheshwari A, Fallon MB. The role of CX(3)CL1/CX(3)CR1 in pulmonary angiogenesis and intravascular monocyte accumulation in rat experimental hepatopulmonary syndrome. J. Hepatol. 57, 752–758 (2012).
  • Roberts KE, Kawut SM, Krowka MJ et al. Genetic risk factors for hepatopulmonary syndrome in patients with advanced liver disease. Gastroenterology 139, 130–139, e24 (2010).
  • Chang CC, Chuang CL, Lee FY et al. Sorafenib treatment improves hepatopulmonary syndrome in rats with biliary cirrhosis. Clin. Sci. (Lond.) 124, 457–466 (2013).
  • Moreira Silva H, Reis G, Guedes M et al. A case of hepatopulmonary syndrome solved by mycophenolate mofetil (an inhibitor of angiogenesis and nitric oxide production). J. Hepatol. 58, 630–633 (2013).
  • 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. 12, 2427–2431 (2006).
  • 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. 24, 183–188 (2010).
  • Peck-Radosavljevic M, Angermayr B, Datz C et al. Austrian consensus on the definition and treatment of portal hypertension and its complications (Billroth II). Wien. Kli. Wochenschr. 125, 200–219 (2013).
  • Fritz JS, Fallon MB, Kawut SM. Pulmonary vascular complications of liver disease. Am. J. Respir. Crit. Care Med. 187, 133–143 (2013).
  • Fukushima KY, Yatsuhashi H, Kinoshita A et al. Two cases of hepatopulmonary syndrome with improved liver function following long-term oxygen therapy. J. Gastroenterol. 42, 176–180 (2007).
  • Riegler JL, Lang KA, Johnson SP, Westerman JH. Transjugular intrahepatic portosystemic shunt improves oxygenation in hepatopulmonary syndrome. Gastroenterology 109, 978–983 (1995).
  • Boyer TD, Haskal ZJ. The role of transjugular intrahepatic portosystemic shunt (TIPS) in the management of portal hypertension: update 2009. Hepatology 51, 306 (2010).
  • Poterucha JJ, Krowka MJ, Dickson ER, Cortese DA, Stanson AW, Krom RA. Failure of hepatopulmonary syndrome to resolve after liver transplantation and successful treatment with embolotherapy. Hepatology 21, 96–100 (1995).
  • Gupta S, Castel H, Rao RV et al. Improved survival after liver transplantation in patients with hepatopulmonary syndrome. Am. J. Transplant. 10, 354–363 (2010).
  • 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 37, 192–197 (2003).
  • Fallon MB, Mulligan DC, Gish RG, Krowka MJ. Model for end-stage liver disease (MELD) exception for hepatopulmonary syndrome. Liver Transpl. 12, S105–S107 (2006).
  • Iyer VN, Swanson KL, Cartin-Ceba R et al. Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era. Hepatology 57, 2427–2435 (2013).
  • Galie N, Hoeper MM, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur. Heart J. 30, 2493–2537 (2009).
  • McLaughlin VV, Archer SL, Badesch DB et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on expert consensus documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation 119, 2250–2294 (2009).
  • Berzigotti A, Seijo S, Reverter E, Bosch J. Assessing portal hypertension in liver diseases. Exp. Rev. Gastroenterol. Hepatol. 7, 141–155 (2013).
  • Pellicelli AM, Barbaro G, Puoti C et al. Plasma cytokines and portopulmonary hypertension in patients with cirrhosis waiting for orthotopic liver transplantation. Angiology 61, 802–806 (2010).
  • Benjaminov FS, Prentice M, Sniderman KW, Siu S, Liu P, Wong F. Portopulmonary hypertension in decompensated cirrhosis with refractory ascites. Gut 52, 1355–1362 (2003).
  • Roberts KE, Fallon MB, Krowka MJ et al. Genetic risk factors for portopulmonary hypertension in patients with advanced liver disease. Am. J. Respir. Crit. Care Med. 179, 835–842 (2009).
  • Pietra GG, Capron F, Stewart S et al. Pathologic assessment of vasculopathies in pulmonary hypertension. J. Am. Coll. Cardiol. 43, 25S–32S (2004).
  • Tuder RM, Abman SH, Braun T et al. Development and pathology of pulmonary hypertension. J. Am. Coll. Cardiol. 54, S3–S9 (2009).
  • Lebrec D, Capron JP, Dhumeaux D, Benhamou JP. Pulmonary hypertension complicating portal hypertension. Am. Rev. Respir. Dis. 120, 849–856 (1979).
  • Portmann B, Stewart S, Higenbottam TW, Clayton PT, Lloyd JK, Williams R. Nodular transformation of the liver associated with portal and pulmonary arterial hypertension. Gastroenterology 104, 616–621 (1993).
  • Humbert M, Sitbon O, Chaouat A et al. Pulmonary arterial hypertension in France: results from a national registry. Am. J. Respir. Crit. Care Med. 173, 1023–1030 (2006).
  • Krowka MJ, Miller DP, Barst RJ et al. Portopulmonary hypertension: a report from the US-based REVEAL Registry. Chest 141, 906–915 (2012).
  • Krowka MJ, Swanson KL, Frantz RP, McGoon MD, Wiesner RH. Portopulmonary hypertension: Results from a 10-year screening algorithm. Hepatology 44, 1502–1510 (2006).
  • Castro M, Krowka MJ, Schroeder DR et al. Frequency and clinical implications of increased pulmonary artery pressures in liver transplant patients. Mayo Clinic Proc. 71, 543–551 (1996).
  • Colle IO, Moreau R, Godinho E et al. Diagnosis of portopulmonary hypertension in candidates for liver transplantation: a prospective study. Hepatology 37, 401–409 (2003).
  • Plevak D, Krowka M, Rettke S, Dunn W, Southorn P. Successful liver transplantation in patients with mild to moderate pulmonary hypertension. Transplant. Proc. 25, 1840 (1993).
  • Ramsay MA, Simpson BR, Nguyen AT, Ramsay KJ, East C, Klintmalm GB. Severe pulmonary hypertension in liver transplant candidates. Liver Transpl. Surg. 3, 494–500 (1997).
  • Robalino BD, Moodie DS. Association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations. J. Am. Coll. Cardiol. 17, 492–498 (1991).
  • Taura P, Garcia-Valdecasas JC, Beltran J et al. Moderate primary pulmonary hypertension in patients undergoing liver transplantation. Anesth. Analg. 83, 675–680 (1996).
  • Kawut SM, Krowka MJ, Trotter JF et al. Clinical risk factors for portopulmonary hypertension. Hepatology 48, 196–203 (2008).
  • Raevens S, Colle I, Reyntjens K et al. Echocardiography for the detection of portopulmonary hypertension in liver transplant candidates: an analysis of cutoff values. Liver Transpl. 19, 602–610 (2013).
  • Kawut SM, Taichman DB, Ahya VN et al. Hemodynamics and survival of patients with portopulmonary hypertension. Liver Transpl. 11, 1107–1111 (2005).
  • Swanson KL, Wiesner RH, Nyberg SL, Rosen CB, Krowka MJ. Survival in portopulmonary hypertension: mayo clinic experience categorized by treatment subgroups. Am. J. Transplant. 8, 2445–2453 (2008).
  • Le Pavec J, Souza R, Herve P et al. Portopulmonary hypertension: survival and prognostic factors. Am. J. Respir. Crit. Care Med. 178, 637–643 (2008).
  • Provencher S, Herve P, Jais X et al. Deleterious effects of beta-blockers on exercise capacity and hemodynamics in patients with portopulmonary hypertension. Gastroenterology 130, 120–126 (2006).
  • Montani D, Savale L, Natali D et al. Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension. Eur. Heart J. 31, 1898–1907 (2010).
  • Humbert M, Segal ES, Kiely DG, Carlsen J, Schwierin B, Hoeper MM. Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur. Respir. J. 30, 338–344 (2007).
  • Savale L, Magnier R, Le Pavec J et al. Efficacy, safety and pharmacokinetics of bosentan in portopulmonary hypertension. Eur. Respir. J. 41, 96–103 (2013).
  • Cartin-Ceba R, Swanson K, Iyer V, Wiesner RH, Krowka MJ. Safety and efficacy of ambrisentan for the treatment of portopulmonary hypertension. Chest 139, 109–114 (2011).
  • Reichenberger F, Voswinckel R, Steveling E et al. Sildenafil treatment for portopulmonary hypertension. Eur. Respir. J. 28, 563–567 (2006).
  • Hoeper MM, Seyfarth HJ, Hoeffken G et al. Experience with inhaled iloprost and bosentan in portopulmonary hypertension. Eur. Respir. J. 30, 1096–1102 (2007).
  • Ghofrani HA, Galie N, Grimminger F et al. Riociguat for the treatment of pulmonary arterial hypertension. N. Engl. J. Med. 369, 330–340 (2013).

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