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Prognostic Assessment of Budd–Chiari Syndrome

  • Subrat Kumar AcharyaEmail author
  • Sawan Bopanna
Chapter

Abstract

Budd–Chiari syndrome, also termed as hepatic venous outflow tract obstruction (HVOTO), is a congestive disorder of liver due to obstruction in the hepatic outflow constituting small hepatic veins to insertion of inferior vena cava to right atrium. Such obstruction usually occurs due to abnormal coagulation associated with myeloproliferative disorder or primary defect in coagulation processes. Prolonged hepatic congestion results in hepatic fibrosis, cirrhosis, portal hypertension, liver failure, and primary liver cancer. While asymptomatic HVOTO carries a good long-term prognosis, symptomatic HVOTO can be life threatening. The treatment modalities in HVOTO have improved substantially over the years. Percutaneous catheter based recanalization of hepatic outflow by balloon angioplasty and/or stenting and transjugular intrahepatic portosystemic shunt (TIPSS) have improved the outcome in such patients as documented by many recent studies. However, despite such radiological interventions many patients deteriorate and are subjected to liver transplant. Therefore prognostic indices to identify high risk patients with HVOTO for mortality have been evaluated. Most prognostic indices have identified poor baseline liver functions indicating poor hepatic reserve as the main indicators of long-term outcome. However, by now there are 7 prognostic indices which have been described by various authors. They are Child–Pugh score, MELD score, Clichy score, the new Clichy score, Rotterdam-BCS index, BCS-TIPSS score, and AIIMS-HVOTO prognostic models. The latter two prognostic indices include the result of radiological intervention as a variable influencing outcome, while the former prognostic models were derived when such radiological interventions were not mainstay of therapy. The comparative studies on these prognostic indices indicate that while each of them can be used in a population as a whole, their use in individual patients may not be adequate. Most of these prognostic indices need external validation. Unfortunately, comparative studies have not been able to identify the most appropriate prognostic index to be used universally which is the unmet need. Multi-centric global study stratifying patients as per site of block and liver dysfunction is needed to identify the most appropriate, universally useful prognostic model.

Keywords

HVOTO Budd–Chiari syndrome Angioplasty Stenting Prognostic-models 

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Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.KIIT UniversityBhubaneswarIndia
  2. 2.Fortis HospitalVasant KunjIndia

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