Zusammenfassung
Die portopulmonale Hypertonie ist eine seltene Erkrankung, die durch das Vorhandensein eines erhöhten mittleren pulmonalarteriellen Druckes und dem gleichzeitigen Vorliegen einer portalen Hypertonie gekennzeichnet ist. Eine schwere portopulmonale Hypertonie gilt als Kontraindikation zur Durchführung einer Lebertransplantation. Zahlreiche Medikamente wie Prostazyklindrivate, Phosphodiesteraseinhibitoren wie auch duale Endothelinrezeptorantagonisten wurden in einzelnen Patienten versuchsweise eingesetzt. Die Wirkung eines selektiven Endothelinrezptorantagonisten wie Sitaxentan in diesem Kontext war bisher unbekannt. Wir berichten hier über die erfolgreiche Therapie mit oralem Sitaxentan über einen Zeitraum von drei Monaten, wobei sich sowohl die Leistungsfähigkeit wie auch die pulmonale Hämodynamik verbesserten. Zusätzlich kam es zu einer Verbesserung der portalen Hypertonie durch eine signifikanten Reduktion des Lebervenendruckes von 12 auf 8 mmHg. Der Einsatz selektiver Endothelinrezeptorantagonisten könnte somit eine neue Therapieoption für diese Patienten darstellen.
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.
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Kähler, C., Graziadei, I., Vogelsinger, H. et al. Successful treatment of portopulmonary hypertension with the selective endothelin receptor antagonist Sitaxentan. Wien Klin Wochenschr 123, 248–252 (2011). https://doi.org/10.1007/s00508-011-1540-4
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DOI: https://doi.org/10.1007/s00508-011-1540-4