Document Detail

Endothelin-receptor antagonist treatment of portopulmonary hypertension.
MedLine Citation:
PMID:  15551258     Owner:  NLM     Status:  MEDLINE    
BACKGROUND & AIMS: Portal hypertension leading to variceal bleeding and ascites is a severe complication of liver cirrhosis and is associated with a high mortality rate. Endogenous vasoconstrictors including endothelin might play a role in control of intrahepatic vascular tone. Few patients develop pulmonary hypertension in the setting of portal hypertension, known as portopulmonary hypertension.
METHODS/RESULTS: In this case report, we describe a patient with portopulmonary hypertension in the setting of cryptogenic liver cirrhosis. The patient received the dual antagonist of receptors A and B of endothelin-1 bosentan. After 16 and 31 weeks the pulmonary arterial pressure dropped significantly from the baseline value of 88 mm Hg to 63 and 58 mm Hg, respectively. Hepatic venous portal gradient was measured before and after bosentan treatment. Hepatic venous portal gradient significantly decreased from 26 mm Hg (baseline) to 7 and 17 mm Hg at 16 and 31 weeks after treatment with bosentan, respectively.
CONCLUSIONS: The present report demonstrates the efficacy of bosentan in the treatment of portopulmonary and portal hypertension in the clinical setting. There are ongoing prospective studies to confirm these data.
Lukas Hinterhuber; Ivo W Graziadei; Christian M Kähler; Werner Jaschke; Wolfgang Vogel
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association     Volume:  2     ISSN:  1542-3565     ISO Abbreviation:  Clin. Gastroenterol. Hepatol.     Publication Date:  2004 Nov 
Date Detail:
Created Date:  2004-11-19     Completed Date:  2005-02-17     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  101160775     Medline TA:  Clin Gastroenterol Hepatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1039-42     Citation Subset:  IM    
Department of Gastroenterology & Hepatology, Medical University Innsbruck, Austria.
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MeSH Terms
Antihypertensive Agents / therapeutic use*
Hypertension, Portal / complications*,  drug therapy*,  etiology,  physiopathology
Hypertension, Pulmonary / drug therapy*,  physiopathology
Liver Cirrhosis / complications
Middle Aged
Sulfonamides / therapeutic use
Reg. No./Substance:
0/Antihypertensive Agents; 0/Sulfonamides; Q326023R30/bosentan

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