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Vascular Liver Disease pp 197–211Cite as

Budd-Chiari Syndrome

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Abstract

The Budd-Chiari syndrome (BCS) is an uncommon and life-threatening disorder defined as the obstruction of hepatic venous outflow regardless of its causative mechanism or level of obstruction. The clinical presentation of BCS is highly variable and can range from asymptomatic cases to ­fulminant hepatic failure with encephalopathy. In the vast majority of cases, it is possible to identify an inherited or acquired prothrombotic risk factor as the underlying cause of thrombosis being chronic myeloproliferative diseases the most frequent etiological cause of BCS. In many cases, multiple factors are present. Anticoagulation is mandatory in patients with BCS. The need for an additional intervention, such as hepatic vein angioplasty, thrombolysis, transjugular intrahepatic portosystemic shunt, surgical shunts, or liver transplantation, depends on the severity of symptoms and response to treatment.

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Acknowledgments

Thanks to Dr. Cervantes, Dr. Reverter, Dra. Gilabert, and Dra. García-Criado for their precious and helpful collaboration. SSR is founded by “Río Hortega” Instituto de Salud Carlos III (CM08/00161). This work is supported in part by grants from Instituto de Salud Carlos III, Ministerio de Ciencia e Innovación (FIS 06/0623, FIS 09/01261 and SAF 07/61298). CIBERehd is funded by the Instituto de Salud Carlos III.

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  1. Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clinic Barcelona-CIBERehd-IDIBAPS-Institut Clinic de Malalties Digestives 1 Metaboliques, Villarroel 170 street, Barcelona, 08036, Spain

    Juan Carlos García-Pagán

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  1. Susana Seijo-Ríos
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  2. Puneeta Tandon
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  3. Jaime Bosch
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  4. Juan Carlos García-Pagán
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Correspondence to Juan Carlos García-Pagán .

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  1. Keck School of Medicine, Div. GI & Liver Diseases, University of Southern California, Los Angeles, 90033, California, USA

    Laurie D. DeLeve

  2. , Section of Digestive Diseases, Yale University School of Medicine, New Haven, 06520, Connecticut, USA

    Guadalupe Garcia-Tsao

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Seijo-Ríos, S., Tandon, P., Bosch, J., García-Pagán, J.C. (2011). Budd-Chiari Syndrome. In: DeLeve, L., Garcia-Tsao, G. (eds) Vascular Liver Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-8327-5_13

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