Abstract

Either acute or sub-acute Budd–Chiari syndrome (BCS) with diffuse occlusion of hepatic veins has a high mortality rate and remains challenging for clinical treatment. We aimed to evaluate the feasibility and safety of transjugular intrahepatic portosystemic shunt (TIPS) as a treatment for BCS with diffuse occlusion of hepatic veins. From January 2007 to December 2010, 100 patients were randomly recruited onto this study and 91 patients were treated with TIPS. 14 patients were defined as acute BCS group and 86 patients as sub-acute group. Patients with acute BCS had a significantly higher rate of jaundice whereas a lower rate of abdominal and chest varices, gastroesophageal variceal bleeding and refractory ascites than sub-acute group (P < 0.001). TIPS was technically successful in all 91 patients (12 in acute group). The portosystemic pressure gradient (PSG) was decreased to normal level, while total bilirubin (TBIL) and liver function were significantly improved. During follow-up period, the mortality rate of 91 patients who underwent TIPS was 6.59% (6/91), whereas 88.89% of 9 patients who didn’t receive TIPS procedure (2 in acute group). Collectively, TIPS is an effective and safe approach in treating BCS with diffuse occlusion of hepatic veins, which should be performed in time.

Highlights

  • Budd–Chiari syndrome (BCS) is an uncommon hepatic disease resulting from hepatic venous obstruction at the level of hepatic vein (HV), inferior vena cava (IVC), or hepatic venules[1]

  • From January 2007 to December 2010, 377 patients with BCS were randomly recruited in Beijing Shijitan Hospital, and 100 patients (F/M: 66/34) were included in this study according to the inclusion and exclusion criteria. 14 patients (F/M: 8/4) were defined as acute BCS group, 86 patients (F/M: 58/28) as sub-acute BCS group. 91 patients (12 in acute BCS group and 79 in sub-acute group) underwent transjugular intrahepatic portosystemic shunt (TIPS) procedure. 9 patients (F/M: 5/4) (2 in acute group and 7 in sub-acute group) received conservative treatment including liver protection therapy, hepatic encephalopathy (HE) prevention and supportive care

  • The rate of HE was significantly higher in acute BCS group (7.14%) than in sub-acute group (2.33%) (P < 0.001)

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Summary

Introduction

Budd–Chiari syndrome (BCS) is an uncommon hepatic disease resulting from hepatic venous obstruction at the level of hepatic vein (HV), inferior vena cava (IVC), or hepatic venules[1]. It occurs in 1 out of a million individuals in western countries and 10 out of a million persons in China[2], characterized by the classical triad of abdominal pain, ascites, and liver enlargement. From January 2007 and December 2010, 91 patients with acute and sub-acute BCS with diffuse occlusion of hepatic veins underwent transjugular intrahepatic portosystemic shunt (TIPS) in a single center, and a good clinical outcome was achieved. We compared the clinical features of patients in acute and sub-acute BCS group, and evaluated the feasibility and safety of TIPS as a promising avenue for these patients

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