title: Improvement of hepatopulmonary syndrome after transjugular intrahepatic portasystemic shunting: case report and review of literature.
abstract: The hepatopulmonary syndrome has been described in as many as 5-29% of patients with liver disease. Patients with this syndrome may suffer from chronic hypoxemia, and mortality rates of liver patients with this syndrome are as high as 41%. Early diagnosis of such patients is essential. Currently, liver transplantation is the only effective therapy for such patients, and reversal of this syndrome is seen in up to 80% of patients post-transplant. Transjugular intrahepatic portasystemic shunting (TIPS) as a therapeutic maneuver for this syndrome has been described in five patients to date with mixed results. Reduction in portal hypertension with consequent redistribution of blood flow and altered synthesis of vasodilatory chemicals have been postulated to help resolve this disease. In this report, we describe an 11-yr-old female with biliary atresia and hepatopulmonary syndrome. Her disease was complicated with recurrent variceal bleeding. TIPS achieved a therapeutic response of both her bleeding and respiratory complications.
authors.affiliation: The Recanati Miller Transplantation Institute, New York, NY 10029, USA.
authors.forename: Anil S
authors.initials: AS
authors.lastname: Paramesh
authors.forename: Sohail Z
authors.initials: SZ
authors.lastname: Husain
authors.forename: Benjamin
authors.initials: B
authors.lastname: Shneider
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Guller
authors.forename: Ikbal
authors.initials: I
authors.lastname: Tokat
authors.forename: Gabriel E
authors.initials: GE
authors.lastname: Gondolesi
authors.forename: Susan
authors.initials: S
authors.lastname: Moyer
authors.forename: Sukru
authors.initials: S
authors.lastname: Emre
mesh_terms: D002648:Child; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000209:etiology / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery; D008107:Liver Diseases / Q000150:complications; D019168:Portasystemic Shunt, Transjugular Intrahepatic