Published online Nov 21, 2014. doi: 10.3748/wjg.v20.i43.16236
Revised: May 30, 2014
Accepted: July 11, 2014
Published online: November 21, 2014
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.
Core tip: Budd-Chiari syndrome (BCS) is a challenging disease with a spectrum of clinical manifestations. It is evident from our experience that this condition has heterogeneous causes. Once specific prognostic factors are considered, treatment courses should be determined on a case-by-case basis but are most consistently instituted in a stepwise fashion. Moreover, in certain patients with visible hepatic decompensation, systemic anticoagulation may need to be fortified with portal decompression procedures such as transjugular intrahepatic portosystemic shunt earlier in the disease process. The liver transplant waitlist mortality was 15.6%; therefore, it is evident that BCS patients have high mortality despite being considered candidates for liver transplantation.