Budd-Chiari syndrome natural history, complications and prognosis

The symptoms of Budd-Chiari syndrome usually develop in the third or fourth decade of life and start with symptoms such as hepatomegaly, ascites, and abdominal pain. Without treatment, the patient will develop complications such as hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, portal hypertension, complications secondary to hepatic decompensation, which may eventually lead to intractable ascites with emaciation, gastrointestinal bleeding, and liver failure. Complications that can develop as a result of Budd-Chiari syndrome are portal hypertension, hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, hepatic decompensation, bacterial peritonitis, especially following paracentesis. If left untreated, patients with Budd-Chiari syndrome have a high mortality rate. About 90% die within 3 years as the disease may progress to develop intractable ascites with emaciation, gastrointestinal bleeding and liver failure. Prognosis is generally good with treatment, and the 5-year survival rate of patients with treatment of Budd-Chiari syndrome is approximately 74 %. Poor prognostic factors include older age at diagnosis, chronic disease, severe liver failure and associated refractory ascites.

• The symptoms of Budd-Chiari syndrome usually develop in the third or fourth decade of life and start with symptoms such as hepatomegaly, ascites, and abdominal pain.^[1]
• Without treatment, the patient will develop complications like hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, portal hypertension, Complications secondary to hepatic decompensation, which may eventually lead to intractable ascites with emaciation, gastrointestinal bleeding, and liver failure.

• Complications that can develop as a result of Budd-Chiari syndrome are:^[1][2]
  • Portal hypertension
  • Hepatic encephalopathy
  • Variceal hemorrhage
  • Hepatorenal syndrome
  • Hepatic decompensation
  • Bacterial peritonitis especially the following paracentesis

• The prognosis of Budd-Chiari syndrome (BCS) is good with treatment.^[3][4][5]
• Without treatment, Budd-Chiari syndrome will result in death as a result of progressive liver failure within 3 years from diagnosis.
• Budd-Chiari syndrome is associated with a 10-year survival rate of 55% with treatment. Among patientd with liver transplantation, the 5-year survival rate is 70%.
• In patients treated by endovascular recanalization the 5-year survival is 86% in patients with intermediate disease severity and 77% in patients with severe BCS.

• Transjugular intrahepatic portosystemic shunt (TIPS) has a 5-year survival of 78% without the need for liver transplantation.
• Calculation of prognostic index for Budd-Chiari can be done using the following formula:
• Prognostic index = (ascites score x 0.75) + (Pugh score x 0.28) + (age x 0.037) + (creatinine level x 0.0036).
• Prognostic index score less than 5.4 is associated with a good prognosis.

Good prognostic factors include:

• Younger age at diagnosis
• Low Child-Pugh score
• Absence of ascites or easily controlled ascites
• Low serum creatinine level

Poor prognostic factors include:

• Older age at diagnosis
• Chronic disease
• Severe liver failure
• Refractory ascites

Template:WS Template:WH