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Budd-Chiari Syndrome: Presentation, Management, and Prognosis.

Waseem Amjad1,2, Paul J Thuluvath1,2

  • 1Institute of Digestive Health & Liver Disease, Mercy Medical Center, Baltimore, Maryland, USA.

The American Journal of Gastroenterology
|December 12, 2025
PubMed
Summary

Budd Chiari syndrome (BCS) involves hepatic vein obstruction, leading to liver issues. Management includes anticoagulation, shunting, and transplantation, with lifelong anticoagulation often necessary for Budd Chiari syndrome patients.

Keywords:
Budd-Chiari syndromehepatitic venous thrombosisliver transplantationprognosis

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Area of Science:

  • Hepatology
  • Vascular Medicine
  • Thrombosis Research

Background:

  • Budd Chiari syndrome (BCS) is a rare hepatic vein obstruction causing liver congestion and dysfunction.
  • It can manifest acutely, subacutely, or chronically, often affecting younger individuals.
  • Risk factors include prothrombotic conditions, myeloproliferative disorders, oral contraceptive use, lower socioeconomic status, and malnutrition.

Purpose of the Study:

  • To review current understanding of Budd Chiari syndrome (BCS) etiologies.
  • To discuss diagnostic options, prognostic scores, and natural history, including pregnancy outcomes.
  • To summarize treatment strategies and outcomes for BCS patients.

Main Methods:

  • Literature review of Budd Chiari syndrome (BCS) research.
  • Analysis of diagnostic criteria and prognostic scoring systems.
  • Evaluation of treatment pathways including anticoagulation, shunting, and liver transplantation.

Main Results:

  • BCS treatment follows a stepwise approach: anticoagulation, portosystemic shunting, and liver transplantation.
  • Liver transplant outcomes for BCS, even with acute liver failure, are comparable to other indications.
  • Pregnancy in BCS has favorable maternal but poor fetal outcomes; lifelong anticoagulation is common.

Conclusions:

  • Budd Chiari syndrome (BCS) management requires a tailored, stepwise approach.
  • Lifelong anticoagulation is frequently necessary for BCS patients, including transplant recipients.
  • Further research is needed for chronic BCS, portal hypertension complications, and liver cancer.