title: Successful treatment of portopulmonary hypertension with the selective endothelin receptor antagonist Sitaxentan.
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.
authors: Department of Internal Medicine, Medical University of Innsbruck, Austria. C.M.Kaehler@i-med.ac.at
authors: Christian M
authors: CM
authors: Kähler
authors: Ivo
authors: I
authors: Graziadei
authors: Helene
authors: H
authors: Vogelsinger
authors: Susanna
authors: S
authors: Desole
authors: Katharina
authors: K
authors: Cima
authors: Wolfgang
authors: W
authors: Vogel
mesh_terms: D065128:Endothelin Receptor Antagonists; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy*; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D007555:Isoxazoles / Q000627:therapeutic use*; D008297:Male; D010865:Pilot Projects; D013577:Syndrome; D013876:Thiophenes / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D065128:Endothelin Receptor Antagonists; D007555:Isoxazoles; D013876:Thiophenes; C106276:sitaxsentan