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Budd–Chiari syndrome/hepatic venous outflow tract obstruction

  • Special Issue - Portal Hypertension
  • Published:
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Abstract

Background

Budd–Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction (HVOTO).

Methods

Recent literature has been analyzed for this narrative review.

Results

Primary BCS/HVOTO is a result of thrombosis. The same patient often has multiple risk factors for venous thrombosis and most have at least one. Presentation and etiology may differ between Western and certain Eastern countries. Myeloproliferative neoplasms are present in 40% of patients and are usually associated with the V617F-JAK2 mutation in myeloid cells, in particular peripheral blood granulocytes. Presentation and symptoms vary, thus this diagnosis must be considered in any patient with acute or chronic liver disease. Doppler ultrasound, computed tomography, or magnetic resonance imaging of the hepatic veins and inferior vena cava usually successfully provide noninvasive identification of the obstruction or its consequences in the collaterals of hepatic veins or the inferior vena cava. The reported life expectancy in these patients is 3 years after the first symptoms. The therapeutic strategy includes first, anticoagulation, correction of risk factors, diuretics, and prophylaxis for portal hypertension, then angioplasty for short-length venous stenosis followed by transjugular intrahepatic portosystemic shunt (TIPS) and finally liver transplantation. The progression of treatment is based on the response to therapy at each step. This strategy results in a 5-year survival rate of nearly 85%. The medium-term prognosis depends upon the severity of liver disease, and the long-term outcome can be jeopardized by transformation of underlying conditions and hepatocellular carcinoma.

Conclusion

BCS/HVOTO hepatic manifestations of BCS/HVOTO can be controlled in most patients with medical or radiological interventions. Underlying disease has become the major determinant of patient outcome.

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  1. Hepatology, University Paris-Diderot and Inserm UMR1149, Paris, France

    Dominique-Charles Valla

  2. DHU UNITY and Centre National de Référence Maladies Rares, Maladies Vasculaires du Foie, Service d’hépatologie, Hôpital Beaujon, APHP, Clichy-la-Garenne, France

    Dominique-Charles Valla

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Valla, DC. Budd–Chiari syndrome/hepatic venous outflow tract obstruction. Hepatol Int 12 (Suppl 1), 168–180 (2018). https://doi.org/10.1007/s12072-017-9810-5

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