Liver Transplantation for Budd-Chiari Syndrome in the MELD Era

To evaluate clinical characteristics and factors associated with survival among liver transplantation (LT) recipients with Budd-Chiari syndrome (BCS), with or without transjugular intrahepatic portosystemic shunt (TIPS), in the post-Model for End-stage Liver Disease era.

We extracted data from the United Network for Organ Sharing database on all adult (≥18 y old) waitlisted candidates and recipients of LT with BCS in the United States between 2002 and 2019. Multivariable Cox regression was used to determine predictors of mortality and hazard ratios (HRs).

A total of 647 BCS patients were waitlisted between 2002 and 2019. BCS was an indication for LT in 378 (0.2%) of all adult LT recipients during the study period. Of BCS patients who received LT, approximately three-fourths (72.3%) were alive for up to 10 y. We found no significant difference in LT outcomes in BCS patients with or without TIPS. Longer length of hospital stay following LT (HR, 1.32; 95% confidence interval [CI], 1.19-1.47), Black/African American race (HR, 2.24; 95% CI, 1.38-3.64), diabetes (HR, 3.17; 95% CI, 1.62-6.21), donor risk index (HR, 1.44; 95% CI, 1.05-1.99), and lower albumin levels at the time of transplantation (HR, 0.66; 95% CI, 0.50-0.88) were negatively associated with survival after LT. Interestingly, neither the Model for End-stage Liver Disease nor prior TIPS showed a significant association with survival after LT.

These findings demonstrate good comparable survival among TIPS versus no TIPS in LT recipients with BCS. The decision for TIPS versus LT should be individualized on a case-by-case basis.