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      Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

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          Abstract

          Budd-Chiari syndrome (BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt (TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.

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          Vascular disorders of the liver.

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            Etiology, management, and outcome of the Budd-Chiari syndrome.

            The Budd-Chiari syndrome (BCS) is hepatic venous outflow obstruction. What is known about the syndrome is based on small studies of prevalent cases. To characterize the causes and treatment of incident BCS. Consecutive case series of patients with incident BCS, enrolled from October 2003 to October 2005 and followed until May 2006. Academic and nonacademic hospitals in France, Spain, Italy, Great Britain, Germany, Belgium, the Netherlands, Portugal, and Switzerland. Persons older than 16 years with definite hepatic outflow obstruction diagnosed by imaging. Persons with hepatic outflow obstruction due to heart failure, sinusoidal obstruction syndrome, cancer, or liver transplantation were excluded. Signs and symptoms; laboratory and imaging findings; diagnosis; treatment; and overall, transplantation-free, and intervention-free survival. 163 incident cases of BCS were identified. Median follow-up was 17 months (range, 0.1 to 31 months). Most patients (84%) had at least 1 thrombotic risk factor, and many (46%) had more than 1; the most common was myeloproliferative disorders (49% of 103 tested patients). Patients were mainly treated with anticoagulation (140 patients [86%]), transjugular intrahepatic portosystemic shunting (56 patients [34%]), or liver transplantation (20 patients [12%]), and 80 patients (49%) were managed noninvasively. Only 3 patients underwent surgical shunting. The survival rate was 87% (95% CI, 82% to 93%) at 1 year and 82% (CI, 75% to 88%) at 2 years. Treatment was not standardized across all centers, and data on important clinical variables were missing for some patients. Most patients with BCS have at least 1 thrombotic risk factor, and many have more than 1; myeloproliferative disorders are most common. One- and 2-year survival rates are good with contemporary management, which includes noninvasive therapies (anticoagulation and diuretics) and invasive techniques. Transjugular intrahepatic portosystemic shunting seems to have replaced surgical shunting as the most common invasive therapeutic procedure. Fifth Framework Programme of the European Commission.
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              The Budd-Chiari syndrome.

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                Author and article information

                Contributors
                Journal
                World J Gastroenterol
                World J. Gastroenterol
                WJG
                World Journal of Gastroenterology
                Baishideng Publishing Group Inc
                1007-9327
                2219-2840
                14 September 2020
                14 September 2020
                : 26
                : 34
                : 5060-5073
                Affiliations
                Interventional Radiology Unit, "F. Miulli" Regional Hospital, Acquaviva delle Fonti 70021, Italy
                Department of Radiology, King´s College Hospital, London SE5 9RS, United Kingdom. riccardoin@ 123456hotmail.it
                Department of Radiology, Gemelli Hospital, Roma 00135, Italy
                Interventional Radiology Department, Aberdeen Royal Infirmary Hospital, Aberdeen AB25 2ZN, United Kingdom
                Interventional Radiology Department, Universidade Federal de Mato Grosso do Sul, Campo Grande 79070-900, Brazil
                Interventional Radiology Department, Universidade Federal de Mato Grosso do Sul, Campo Grande 79070-900, Brazil
                2 nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Chaidari Athens GR 12461, Greece
                2 nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Chaidari Athens GR 12461, Greece
                Author notes

                Author contributions: All authors equally contributed to this paper with conception and design of the study, literature review and analysis, drafting and critical revision and editing, and final approval of the final version.

                Corresponding author: Riccardo Inchingolo, MD, Doctor, Interventional Radiology Unit, "F. Miulli" Regional Hospital, Acquaviva delle Fonti 70021, Italy. riccardoin@ 123456hotmail.it

                Article
                jWJG.v26.i34.pg5060
                10.3748/wjg.v26.i34.5060
                7495032
                32982109
                ae322da2-53de-4c88-94a9-28724a6dd244
                ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.

                This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial.

                History
                : 19 April 2020
                : 6 June 2020
                : 25 August 2020
                Categories
                Review

                budd-chiari syndrome,liver,transjugular intrahepatic portosystemic shunt,orthotopic liver transplantation,interventional radiology,portal hypertension

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