Eighteen years of liver transplantation experience in patients with advanced Budd-Chiari syndrome

Abstract

The long-term results of liver transplantation for Budd-Chiari syndrome (BCS) and timely indication for the procedure are still under debate. Innovations in interventional therapy and better understanding of underlying diseases have improved therapy strategies. The aim of this study was the analysis of patient and disease characteristics, outcome, and specific complications. Between September 1988 and December 2006 we performed 42 orthotopic liver transplantations (OLTs) in 39 patients with BCS. A total of 29 (74%) women and 10 men (26%) had a median age of 35 years; the median follow-up period was 96 months. Etiologically, 27 patients had a preoperative diagnosis of hematologic disease, including myeloproliferative disorders (MPD), followed by factor V Leiden mutation and antiphospholipid syndrome. The actuarial 5-year and 10-year survival rates were 89.4% and 83.5%, respectively, compared to 80.7% and 71.4%, respectively, for other indications (n ϭ 1742). Retransplantation was necessary in 3 patients (7.1%) with portal vein thrombosis or recurrent BCS. Although the number of bleeding events was similar, incidence of vascular complications was significantly higher in patients with BCS. Thrombosis of the portal vein was observed in 4.8% versus 0.8% of the patients, whereas liver veins were affected in 7.1% versus 0.2%. Our data shows that severe acute or chronic forms of BCS with liver failure can be successfully treated by OLT. Despite higher rates of vascular complications, patient and graft survival are similar or even better compared to other indication groups. In conclusion, patients with reversible hepatic damage should be treated by combined strategies, including medical therapy and surgical or interventional shunting.

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“…These findings are similar to those from larger series of patients published in this era. Ulrich and associates also reported the same rate of recurrent BCS in their study (7.1%), 9 whereas Yamada and colleagues reported a higher rate of recurrence in their series (33.3%). 10 After OLT, the most important strategy for preventing recurrent BCS is anticoagulant or antiaggregant therapy, depending on the underlying cause of BCS.…”

“…They found higher rates of vascular complications in patients who had received a transplant for BCS than for other indications. 9 In our study, overall patient survival rates were 87%, 71%, and 71% at 1-, 3-, and 5-year follow-up, and 3 of 4 patient deaths (75%) occurred in the early postoperative period. In this study, the causes of mortality were primary graft nonfunction, cardiac failure, and intra-abdominal sepsis.…”

“…4 Therapeutic procedures are introduced in order of increasing invasiveness, based on the patient's response to previous therapy. 1,[7][8][9] Orthotopic liver transplant (OLT) should be considered in patients with fulminant BCS with irreparable necrosis of the liver parenchyma, as well as in patients with chronic forms of BCS accompanied by established cirrhosis and hepatic decompensation. 5,7 The aim of this study was to evaluate the results of 15 OLTs over a period of 7 years in 14 patients diagnosed with BCS.…”

Budd‐Chiari syndrome

“…In this study the five-year survival rate was 89.4%, which does not differ that much from the survival rate of an untreated Child-Pugh class A. 77,82 This indicates that the survival benefit of OLT is most pronounced in those BCS patients with worse baseline characteristics. The survival rate and graft function after OLT in BCS patients are similar 78 or even superior 77 to those transplanted for other indications.…”

“…77,82 This indicates that the survival benefit of OLT is most pronounced in those BCS patients with worse baseline characteristics. The survival rate and graft function after OLT in BCS patients are similar 78 or even superior 77 to those transplanted for other indications. A previous TIPS placement is not associated with a poor outcome after OLT.…”