Budd-Chiari syndrome (BCS) is a rare, life-threatening disease characterized by hepatic venous outflow obstruction. Liver transplantation (LT) is widely accepted as an effective therapeutic measure for irreversible liver failure due to BCS. There is debate on differences in the post LT course and complications in patients with BCS as compared to non-Budd-Chiari (NBC) patients.
In this retrospective study, data on all patients who received a liver transplant for BCS at the Shiraz Organ Transplantation Center between January 1996 and September 2017 were reviewed and compared to data of a control group who had received liver transplants over the same period but due to other causes (NBC).
Out of 4225 patients who received liver transplants in the study period, 108 had BCS and an age- and gender-matched control group consisted of 108 NBC cases. The mean ± standard deviation (SD) of model for end-stage liver disease (MELD) scores were 19.1 ± 3 and 20 ± 3 for BCS and NBC groups, respectively (p = 0.33). One-, 3-, 5-, and 10-year survival rates in the BCS group were as follows: 82%, 78%, 76%, and 76% compared with the NBC rates of 83%, 83%, 83%, and 76%, respectively (p = 0.556). There was no difference between the two groups in complication rates after 6 months. In the later period, vascular thrombosis was more common in BCS.
Whole-organ LT from deceased donors in patients with BCS had comparable outcomes with LT due to other causes of end-stage liver disease. In most instances, these patients should receive lifelong anticoagulation.