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- Title
Successful treatment of portopulmonary hypertension with the selective endothelin receptor antagonist Sitaxentan.
- Authors
Kähler, Christian M; Graziadei, Ivo; Vogelsinger, Helene; Desole, Susanna; Cima, Katharina; Vogel, Wolfgang
- Abstract
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.
- Subjects
PORTAL hypertension; LIVER transplantation; TRANSPLANTATION of organs, tissues, etc.; PHOSPHODIESTERASES; HYPERTENSION
- Publication
Wiener Klinische Wochenschrift, 2011, Vol 123, Issue 7/8, p248
- ISSN
0043-5325
- Publication type
Report
- DOI
10.1007/s00508-011-1540-4