Successful treatment of portopulmonary hypertension with the selective endothelin receptor antagonist Sitaxentan

Summary Portopulmonary hypertension (POPH) is a rare complication of portal hypertension. Prostanoids have been shown to be effective in the treatment of POPH and have been used as a bridge to liver transplantation. More recently, case series revealed beneficial effects of both the dual endothelin r...

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Bibliographic Details
Published in:Wiener Klinische Wochenschrift 2011-04, Vol.123 (7-8), p.248-252
Main Authors: Kähler, Christian M., Graziadei, Ivo, Vogelsinger, Helene, Desole, Susanna, Cima, Katharina, Vogel, Wolfgang
Format: Article
Language:English
Subjects:
Case Report
Endocrinology
Endothelin Receptor Antagonists
Gastroenterology
Humans
Hypertension, Portal - drug therapy
Hypertension, Pulmonary - drug therapy
Internal Medicine
Isoxazoles - therapeutic use
Male
Medicine
Medicine & Public Health
Pilot Projects
Pneumology/Respiratory System
Syndrome
Thiophenes - therapeutic use
Treatment Outcome
Young Adult
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Summary:Summary Portopulmonary hypertension (POPH) is a rare complication of portal hypertension. Prostanoids have been shown to be effective in the treatment of POPH and have been used as a bridge to liver transplantation. More recently, case series revealed beneficial effects of both the dual endothelin receptor antagonist bosentan and the phosphodiesterase-5 inhibitor sildenafil. The efficacy of sitaxentan, a selective endothelin receptor A (ERA) antagonist in the reversal of POPH, is still unclear. We report a case of POPH that was successfully treated with oral sitaxentan. Haemodynamic and symptomatic improvements were maintained after a 12-week long-term treatment period. Additionally, hepatic vein pressure gradient significantly decreased from 12 mmHg to 8 mm after treatment with sitaxentan. This is the first reported case of a successful therapy with a selective ERA antagonist in a patient suffering from POPH. Oral sitaxentan therapy might be a promising new option for patients suffering from POPH.
ISSN:0043-5325
1613-7671
DOI:10.1007/s00508-011-1540-4