Liver transplantation for hepatopulmonary syndrome due to noncirrhotic portal hypertension

K. Maganty, R. Ghanta, P. Bejarano, D. Weppler, Akin Tekin, J. Moon, Seigo Nishida, A. Tzakis, Paul Martin

Research output: Contribution to journalArticle

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Abstract

Background: Hepatopulmomary syndrome is defined by the triad of chronic liver disease, increased alveolar-arterial gradient, and evidence of intrapulmonary vasodilation. It is commonly seen in association with cirrhosis (90%). Four percent to 8% of the hepatopulmomary syndrome cases are reported in noncirrhotic portal hypertension. The management of patients with hepatopulmomary syndrome due to noncirrhotic portal hypertension is not well described. Methods: We report a case of a 26-year-old woman who underwent liver transplantation for hepatopulmomary syndrome due to noncirrhotic portal hypertension. The patient presented with dyspnea and platypnea, requiring home oxygen therapy. She had orthodexia, severe hypoxemia, and positive bubble echocardiography consistent with hepatopulmomary syndrome. Her Model for End-stage Liver Disease score was 10. Liver biopsy revealed diffuse nodular regenerative hyperplasia. Results: The patient underwent liver transplantation with Model for End-stage Liver Disease exception points. Her oxygen requirements gradually improved during the postoperative period. The patient's symptoms and hypoxemia resolved at 15-month follow-up posttransplantation. Conclusion: We suggest hepatopulmonary syndrome in this setting is an indication for liver transplantation despite the absence of cirrhosis.

Original languageEnglish
Pages (from-to)2814-2816
Number of pages3
JournalTransplantation Proceedings
Volume43
Issue number7
DOIs
StatePublished - Sep 1 2011

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Hepatopulmonary Syndrome
Portal Hypertension
Liver Transplantation
End Stage Liver Disease
Fibrosis
Oxygen
Postoperative Period
Vasodilation
Dyspnea
Hyperplasia
Echocardiography
Liver Diseases
Chronic Disease
Biopsy
Liver

ASJC Scopus subject areas

  • Surgery
  • Transplantation

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Liver transplantation for hepatopulmonary syndrome due to noncirrhotic portal hypertension. / Maganty, K.; Ghanta, R.; Bejarano, P.; Weppler, D.; Tekin, Akin; Moon, J.; Nishida, Seigo; Tzakis, A.; Martin, Paul.

In: Transplantation Proceedings, Vol. 43, No. 7, 01.09.2011, p. 2814-2816.

Research output: Contribution to journalArticle

Maganty, K. ; Ghanta, R. ; Bejarano, P. ; Weppler, D. ; Tekin, Akin ; Moon, J. ; Nishida, Seigo ; Tzakis, A. ; Martin, Paul. / Liver transplantation for hepatopulmonary syndrome due to noncirrhotic portal hypertension. In: Transplantation Proceedings. 2011 ; Vol. 43, No. 7. pp. 2814-2816.
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AU - Maganty, K.

AU - Ghanta, R.

AU - Bejarano, P.

AU - Weppler, D.

AU - Tekin, Akin

AU - Moon, J.

AU - Nishida, Seigo

AU - Tzakis, A.

AU - Martin, Paul

PY - 2011/9/1

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