Budd-Chiari syndrome

Czech version

Authors: T. Takáč; F. Danček; Ľ. Skladaný
Authors‘ workplace: II. interná klinika SZU a FNsP F. D. Roosevelta Banská Bystrica
Published in: Gastroent Hepatol 2026; 80(3): 255-259
Category: Hepatology: Rewiev Article
doi: https://doi.org/10.48095/ccgh2026255

Overview

Budd-Chiari syndrome (BCS) is a rare vascular liver disorder characterized by obstruction of hepatic venous outflow at the level of hepatic veins and/or inferior vena cava, most commonly due to thrombotic processes. Estimated incidence is approximately 1 case per million inhabitants per year. Most frequent etiological factors include myeloproliferative neoplasms, inherited and acquired thrombophilic conditions, and antiphospholipid syndrome. Clinical presentation is highly heterogeneous, ranging from asymptomatic forms to acute liver failure. Most common manifestations include ascites, hepatomegaly, and abdominal pain. Diagnosis is primarily based on imaging modalities, with Doppler ultrasonography as the first-line method, complemented by CT or MR angiography to accurately assess the extent of vascular involvement. Management of BCS requires an individualized, stepwise approach. Early initiation and long-term administration of anticoagulation represent the cornerstone of therapy, together with management of portal hypertension-related complications. In patients with insufficient response to conservative treatment, interventional procedures are indicated, including percutaneous angioplasty, stenting, or creation of a transjugular intrahepatic portosystemic shunt (TIPS or DIPS). Liver transplantation remains the definitive therapeutic option in patients with fulminant presentation or advanced liver disease. Prognosis of patients with BCS has significantly improved over recent decades due to advances in diagnostic and therapeutic strategies, with current 5-year survival exceeding 70–80%. Early diagnosis, systematic evaluation of underlying etiological factors, and appropriate stepwise management are essential for optimizing clinical outcomes.

Keywords:

Budd-Chiari syndrome – liver cirrhosis – ascites – portal hypertension

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