An Update on the Management of Budd–Chiari Syndrome

Budd–Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advanc...

Full description

Saved in:
Bibliographic Details
Published in:Digestive diseases and sciences 2021-06, Vol.66 (6), p.1780-1790
Main Authors: Sharma, A., Keshava, S. N., Eapen, A., Elias, E., Eapen, C. E.
Format: Article
Language:English
Subjects:
Angioplasty - trends
Biochemistry
Budd-Chiari Syndrome - diagnostic imaging
Budd-Chiari Syndrome - physiopathology
Budd-Chiari Syndrome - therapy
Care and treatment
Development and progression
Disease
Disease Management
Endovascular Procedures - trends
Etiology
Gastroenterology
Hepatology
Humans
Hypertension
Liver
Liver Transplantation - trends
Medicine
Medicine & Public Health
Mutation
Oncology
Pathology
Patients
Review
Thrombolytic Therapy - trends
Thrombosis
Transplant Surgery
Veins & arteries
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Budd–Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.
ISSN:0163-2116
1573-2568
DOI:10.1007/s10620-020-06485-y