Budd-Chiari syndrome: a single-center experience

Abstract

Aim: To investigate challenges, risk factors, prognostic indicators, and treatment outcomes associated with Budd-Chiari syndrome (BCS) at a tertiary care center.

Methods: A retrospective cohort study was conducted at the University of Pennsylvania in patients with a diagnosis of BCS or hepatic vein thrombosis. All patients receiving care at the University of Pennsylvania, and who had at least 2 clinical encounters in the University of Pennsylvania Health system from January 1, 2008 to September 10, 2013 were eligible for study inclusion. Data were extracted from the electronic medical record of each patient, and recorded in a secure Research Electronic Data Capture database. Logistic regression analyses were applied to identify predictors of outcome of liver transplant (LT) or death.

Results: Between January 1, 2008 and September 10, 2013, forty-seven patients were identified. Median age was 42.4 years. Thirty-one (66.0%) were women. A majority were Caucasian (68.1%). At diagnosis, 43 (91.5%) patients had ascites, 27 (57.4%) patients had a hematologic disorder associated with a hypercoagulable state and 26 (55.3%) had cirrhosis. Forty (85.1%) patients were on anticoagulation (AC), 30 (63.8%) of whom were maintained on warfarin. Two patients (4.3%) underwent thrombolytic therapy. A transjugular intrahepatic portosystemic shunt (TIPS) was placed in 21 (44.7%) patients, 19 (90.5%) of whom were also on AC. Twenty-one (44.7%) received AC alone. Over a median of 974 d, 8 (17.0%) patients received LT, and 10 (21.3%) died. The median time from listing to death was 26 mo [interquartile range (IQR) = 16, 65)]. TIPS with AC was utilized more frequently in younger patients (P = 0.02). Age, cirrhosis and chronic kidney disease (CKD) were significant predictors of LT or death.

Conclusion: AC alone was employed as frequently as TIPS with AC, though the latter was used more frequently in younger patients with polycythemia vera. There were no significant differences in treatment outcome regardless of the therapeutic intervention employed. Significant predictors of poor prognosis included age, cirrhosis and CKD.

Keywords: Anticoagulation; Budd-Chiari syndrome; Hepatic vein thrombosis; Liver transplantation; Transjugular intrahepatic portosystemic shunt.

Figure 1

Treatment and outcomes. BCS: Budd-Chiari syndrome; TIPS: Transjugular intrahepatic portosystemic shunt; AC: Anticoagulation; HE: Hepatic encephalopathy; OLT: Orthotopic liver transplantation.

Figure 2

Kaplan-Meier estimate of mortality: Effect of transplant status.

Figure 3

Kaplan-Meier estimate of mortality: Effect of transjugular intrahepatic portosystemic shunt + anticoagulation vs anticoagulation alone. TIPS: Transjugular intrahepatic portosystemic shunt.