Korean Circulation Journal

The Korean Society of Cardiology (대한심장학회)
권호 표지
DOI QR코드

DOI QR Code

Aggravation of Hepatopulmonary Syndrome after Sildenafil Treatment in a Patient with Coexisting Portopulmonary Hypertension

  • Chung, Seungmin (Division of Cardiology, Heart, Vascular and Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Kyungho (Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Chang, Sung-A (Division of Cardiology, Heart, Vascular and Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Duk-Kyung (Division of Cardiology, Heart, Vascular and Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • Received : 2014.03.25
  • Accepted : 2014.06.12
  • Published : 2015.01.30
⟨ Previous Next ⟩

Abstract

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are complications of portal hypertension and cirrhosis. Their pathophysiological mechanisms clearly differ. HPS is characterized by a defect in arterial oxygenation induced by pulmonary vascular dilatation. In contrast, PPHTN is predominantly due to excessive pulmonary vasoconstriction and vascular remodeling, but is rarely associated with hypoxia. We report a case of a patient who had both HPS and PPHTN at the time of presentation. HPS was aggravated after sildenafil administration for the treatment of PPHTN. We demonstrated increased amount of intrapulmonay shunt after sildenafil challenge by using agitated saline contrast transthoracic echocardiography.

Keywords

References

  1. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet 2004;363:1461-8. https://doi.org/10.1016/S0140-6736(04)16107-2
  2. Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome--a liverinduced lung vascular disorder. N Engl J Med 2008;358:2378-87. https://doi.org/10.1056/NEJMra0707185
  3. Rodriguez-Roisin R, Krowka MJ, Herve P, et al. Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J 2004;24:861-80. https://doi.org/10.1183/09031936.04.00010904
  4. Pham DM, Subramanian R, Parekh S. Coexisting hepatopulmonary syndrome and portopulmonary hypertension: implications for liver transplantation. J Clin Gastroenterol 2010;44:e136-40.
  5. Zopey R, Susanto I, Barjaktarevic I, Wang T. Transition from hepatopulmonary syndrome to portopulmonary hypertension: a case series of 3 patients. Case Rep Pulmonol 2013;2013:561870.
  6. Schwartz BG, Levine LA, Comstock G, Stecher VJ, Kloner RA. Cardiac uses of phosphodiesterase-5 inhibitors. J Am Coll Cardiol 2012;59:9-15. https://doi.org/10.1016/j.jacc.2011.07.051
  7. Reichenberger F, Voswinckel R, Steveling E, et al. Sildenafil treatment for portopulmonary hypertension. Eur Respir J 2006;28:563-7. https://doi.org/10.1183/09031936.06.00030206
  8. Gough MS, White RJ. Sildenafil therapy is associated with improved hemodynamics in liver transplantation candidates with pulmonary arterial hypertension. Liver Transpl 2009;15:30-6. https://doi.org/10.1002/lt.21533
  9. Kleinsasser A, Loeckinger A, Hoermann C, et al. Sildenafil modulates hemodynamics and pulmonary gas exchange. Am J Respir Crit Care Med 2001;163:339-43. https://doi.org/10.1164/ajrccm.163.2.2003021
  10. Castro PF, Greig D, Verdejo HE, et al. Intrapulmonary shunting associated with sildenafil treatment in a patient with idiopathic pulmonary arterial hypertension. Thorax 2011;66:1097-8. https://doi.org/10.1136/thx.2010.156711
  11. Olschewski H, Rose F, Schermuly R, et al. Prostacyclin and its analogues in the treatment of pulmonary hypertension. Pharmacol Ther 2004;102:139-53. https://doi.org/10.1016/j.pharmthera.2004.01.003
  12. Shao D, Park JE, Wort SJ. The role of endothelin-1 in the pathogenesis of pulmonary arterial hypertension. Pharmacol Res 2011;63:504-11. https://doi.org/10.1016/j.phrs.2011.03.003
  13. 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;39:1593-602. https://doi.org/10.1002/hep.20244
  14. Zhang J, Ling Y, Tang L, Luo B, Pollock DM, Fallon MB. Attenuation of experimental hepatopulmonary syndrome in endothelin B receptordeficient rats. Am J Physiol Gastrointest Liver Physiol 2009;296:G704-8. https://doi.org/10.1152/ajpgi.90627.2008
  15. Fallon MB. Mechanisms of pulmonary vascular complications of liver disease: hepatopulmonary syndrome. J Clin Gastroenterol 2005;39(4 Suppl 2):S138-42. https://doi.org/10.1097/01.mcg.0000155570.19340.61
  16. Gudavalli A, Kalaria VG, Chen X, Schwarz KQ. Intrapulmonary arteriovenous shunt: diagnosis by saline contrast bubbles in the pulmonary veins. J Am Soc Echocardiogr 2002;15:1012-4. https://doi.org/10.1067/mje.2002.121435

Cited by

  1. Sildenafil : Aggravation of hepatopulmonary syndrome and increased amount of intrapulmonary shunt: case report vol.1540, pp.1, 2015, https://doi.org/10.1007/s40278-015-8118-2
  2. Portopulomonary Hypertension and Hepatopulmonary Syndrome with Congenital Portosystemic Venous Shunt vol.31, pp.4, 2015, https://doi.org/10.9794/jspccs.31.223
  3. Mechanical characteristics of the pulmonary artery in beagle dogs with hepatopulmonary syndrome and portopulmonary hypertension vol.4, pp.1, 2015, https://doi.org/10.3892/br.2015.526
  4. Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil vol.23, pp.6, 2015, https://doi.org/10.1177/1093526620945951