Liver transplantation for Budd-Chiari syndrome: A European study on 248 patients from 51 centres

Transplantation Unit, Department of Surgery, University Hospitals, 1211 Geneva 14, Switzerland.
Journal of Hepatology (Impact Factor: 11.34). 04/2006; 44(3):520-8. DOI: 10.1016/j.jhep.2005.12.002
Source: PubMed


The results of liver transplantation for Budd-Chiari syndrome (BCS) are poorly known and the role and timing of the procedure are still controversial. The aim of this study was to investigate the results of transplantation for BCS, focusing on overall outcome, on prognostic factors and on the impact of the underlying disease.
An enquiry on 248 patients representing 84% of the patients transplanted for BCS in the European Liver Transplantation Registry between 1988 and 1999.
Of the 248 patients, 70.4% were female and 29.6% male. The mean age was 35.7 years. The overall actuarial survival was 76% at 1 year, 71% at 5 years and 68% at 10 years. 77% of deaths occurred in the first 3 months: 47% were due to infection and multiple organ failure, and 18% to graft failure or hepatic artery thrombosis. Late mortality (>1 year) occurred in nine patients, due to BCS recurrence in four of them. The only pre-transplant predictors of mortality on multivariate analysis (Cox) were impaired renal function and a history of a shunt.
Liver transplantation for BCS is an effective treatment, irrespective of the underlying cause, and should be considered before renal failure occurs.

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    • "However, invasive procedures and mechanical devices could trigger additional thrombotic events or enhance extension of preexisting ones and should be performed with caution. Furthermore, recurrence after liver transplantation is about 27% and, as might be expected, antithrombotic prophylaxis with aspirin and hydroxiurea for myeloproliferative syndromes or anticoagulation for patients with thrombophilia after the procedure has dramatically improved the results[27] [28] "
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    ABSTRACT: Budd-Chiari syndrome is characterized by supra-hepatic veins obstruction, leading to post-sinusoidal portal hypertension that often evolves to hepatic failure. It is usually related to prothrombotic conditions, such as trombophilia, myeloproliferative diseases or nocturnal paroxysmal hemoglobinuria. Spontaneous remissions are rare and less than a third of the patients survive one year without treatment. We recommend that anticoagulation should be started as soon as possible with full-dose subcutaneous heparin, postponing warfarin therapy until substantial improvement of ascites and liver con-gestion. This approach optimizes anticoagulation, decreasing the chances of bleeding. Since January 2000, among 350 pa-tients followed at the Anticoagulation Clinic, three fulfilled the criteria for primary Budd-Chiari syndrome and were started on scheduled anticoagulation protocol. During three to ten years follow-up, supra-hepatic thrombosis completely resolved in all patients and hepatic function normalized without resorting to invasive procedures or liver transplantation. Neither re-currence of thrombotic events, nor serious bleeding events were documented. Scheduled anticoagulation is safe and improves patient's outcomes.
    Full-text · Dataset · Nov 2013
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    • "In eastern countries, BCS is a chronic condition and patients are treated with balloon dilatation or stent placement (5). In addition, a transjugular intrahepatic portosystemic shunt (TIPS) or alternative surgical treatment (including shunting procedures and liver transplantation) may be performed in patients with diffuse hepatic venous occlusion (6,7). "
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    ABSTRACT: The aim of this study was to assess the efficacy and safety of catheter-directed thrombolysis combined with angioplasty in the treatment of hepatic vein obstruction in Budd-Chiari syndrome (BCS) complicated by thrombosis. In 14 cases of BCS, the patients with hepatic vein obstruction complicated by thrombosis who underwent catheter-directed urokinase thrombolysis, balloon dilatation and/or stent placement were followed up with an ultrasound examination of the liver. Among the 13 cases of successful treatment, one hepatic vein was recanalized in 12 patients (right hepatic vein, seven cases; left hepatic vein, three cases; middle hepatic vein, one case and accessory hepatic vein, one case) and two hepatic veins (right and left) were recanalized in one patient without serious complications, such as bleeding and pulmonary embolism. There was one patient in whom the treatment was unsuccessful. During an average follow-up period of 24.8±19.6 months, hepatic vein restenosis was observed in one patient in the sixth month after opperation; however, a successful result was obtained following a second balloon dilatation. The remaining 12 patients did not demonstrate any recurrence of restenosis or thrombosis. Catheter-directed thrombolysis combined with angioplasty was observed to be an effective and safe method for the treatment of hepatic vein obstruction in BCS complicated by thrombosis.
    Full-text · Article · Oct 2013 · Experimental and therapeutic medicine
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    • "Two were transplanted before TIPS had been introduced as a treatment option of BCS and two had cirrhosis with signs of chronic liver failure. Survival following OLT depends upon the underlying cause of BCS and the patients condition at the time of transplantation [41]. A large series with 510 patients [42] found a 3 year patient survival of 85% after introduction of the Model for End-Stage Liver Disease (MELD) score versus a 3 year patient survival of 73% in the pre-MELD era. "
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    ABSTRACT: Budd-Chiari syndrome (BCS) generally implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. Treatment depends on the underlying cause, the anatomic location, the extent of the thrombotic process and the functional capacity of the liver. It can be divided into medical treatment including anticoagulation and thrombolysis, radiological procedures such as angioplasty and transjugular intrahepatic porto-systemic shunt (TIPS) and surgical interventions including orthotopic liver transplantation (OLT). Controlled trials or reports on larger cohorts are limited due to rare disease frequency. The aim of this study was to report our single centre long term results of patients with BCS receiving one of three treatment options i.e. medication only, TIPS or OLT on an individually based decision of our local expert group. 20 patients with acute, subacute or chronic BCS were treated between 1988 and 2008. Clinical records were analysed with respect to underlying disease, therapeutic interventions, complications and overall outcome. 16 women and 4 men with a mean age of 34 +/- 12 years (range: 14-60 years) at time of diagnosis were included. Myeloproliferative disorders or a plasmatic coagulopathy were identified as underlying disease in 13 patients, in the other patients the cause of BCS remained unclear. 12 patients presented with an acute BCS, 8 with a subacute or chronic disease. 13 patients underwent TIPS, 4 patients OLT as initial therapy, 2 patients required only symptomatic therapy, and one patient died from liver failure before any specific treatment could be initiated. Eleven of 13 TIPS patients required 2.5 +/- 2.4 revisions (range: 0-8). One patient died from his underlying hematologic disease. The residual 12 patients still have stable liver function not requiring OLT. All 4 patients who underwent OLT as initial treatment, required re-OLT due to thrombembolic complications of the graft. Survival in the TIPS group was 92.3% and in the OLT group 75% during a median follow-up of 4 and 11.5 years, respectively. Our results confirm the role of TIPS in the management of patients with acute, subacute and chronic BCS. The limited number of patients with OLT does not allow to draw a meaningful conclusion. However, the underlying disease may generate major complications, a reason why OLT should be limited to patients who cannot be managed by TIPS.
    Full-text · Article · Mar 2010 · BMC Gastroenterology
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