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: 10.4). 04/2006; 44(3):520-8. DOI: 10.1016/j.jhep.2005.12.002
Source: PubMed

ABSTRACT 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.
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    • "We would like to suggest that high diagnostic accuracy, availability, low side-effect profile and economic advantages make ultrasound the optimum method to follow-up patients with BCS. Based on technical applicability and local expertise we believe that the therapeutic procedures should be introduced by order of increasing invasiveness (medical therapy, interventional shunting, surgical shunting and finally OLT) based on response to previous therapy [20] [21]. "
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    ABSTRACT: We analyzed sonomorphological signs of Budd-Chiari syndrome (BCS) and their potential for prognosis prediction. Forty-five consecutive patients were included. Analysis included the frequencies of sonomorphological signs and their predictive value for diagnosis of BCS, mean values of laboratory and color Doppler data in different therapeutic groups and survival. Specific ultrasound signs were identified at the level of the hepatic veins in 71% of the patients and in 33% at the level of the caval vein, i.e. thrombosis, stenosis, fibrotic cord or insufficient recanalization of the vessels. The frequent non-specific signs were splenomegaly (78%), inhomogeneous liver parenchyma (76%), intrahepatic collaterals (73%), caudate lobe hypertrophy (67%), ascites (56%) and extrahepatic collaterals (44%). The combination of specific signs and "caudate lobe hypertrophy" offered the highest predictive value to identify patients with BCS (p=0.014) with a specificity of 100%. Mean survival was significantly different between the patients with or without portal hypertension (n=25, 41.1+/-7.6, 95% CI (26.2-55.9) versus n=20, 89.4+/-4.5, 95% CI (80.5-98.2), p=0.004) and with or without portal vein thrombosis (n=12, 29.8+/-10.7, 95% CI (8.9-50.7) versus n=33, 79.3+/-6.1, 95% CI (67.4-91.1), p=0.003). We present a comprehensive description of sonomorphological signs in BCS. The combination of ultrasound signs "altered hepatic and/or caval veins" and "caudate lobe hypertrophy" was the best strategy to diagnose BCS. Patients with portal vein thrombosis or portal hypertension have a poor prognosis.
    Journal of Hepatology 07/2008; 49(4):572-80. DOI:10.1016/j.jhep.2008.04.025 · 10.40 Impact Factor
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    • "Liver transplantation for Budd–Chiari syndrome Author Year Country Years included # patients PreTx shunt PreTx TIPS 1-year patient survival (%) 3-year patient survival (%) 5-year patient survival (%) Recur- rence % retrans- plant Campbell 1988 UK 1976–1986 17 NS NS 88 88 NS 1 12 Halff 1990 US 1974–1988 23 5 0 69 45 45 3 13 Jamieson 1991 UK 1986–1990 26 3 0 69 69 50 2 8% Shaked 1992 US 1984–1990 14 2 NS 86 76 NS 0 21 Knoop 1994 Germany 1988–1994 8 NS NS 88 NS NS 1 25 Sakai 1994 Canada 1977–1990 11 3 NS 64 64 64 0 0% Ringe 1995 Germany 1981–1993 43 4 0 69 69 69 0 21 Hemming 1996 Canada 1987–1995 10 3 NS NS NS 59 NS 10 Rao 2000 Hong Kong 1986–1997 8 3 NS NS 63 NS 0 0% Srinivasan 2002 UK 1988–1999 19 5 1 95 95 95 2 21 Ulrich 2002 Germany 1988–2001 27 NS NS NS NS 87 NS 15 Attwell 2004 US 1992–2001 10 0 5 90 80 80 0 0% Cruz 2005 US 1988–2002 11 1 4 81 NS 65 3 9% Mentha 2006 Europe 1988–1999 248 49 11 76 71 68 6 15 Yamada 1 2006 Japan 1990–2005 9 0 1 88 71 67 2 0 Plessier 2006 France 1997–2004 11 0 2 91 91 NS 1 9 Segev 2006 US 2002–2006 Ã 100 NS 23 88 85 NS NS NS 1 "
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    ABSTRACT: Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atriocaval junction. BCS is a complex disease with a wide spectrum of aetiologies and presentations. This article reviews the current literature with respect to presentation, management and prognosis of the disease. Medical, interventional and surgical management of BCS is discussed. Particular attention is paid to interventional and surgical aspects of management. The review is augmented by images, which provide a clinical corollary to the text.
    Liver international: official journal of the International Association for the Study of the Liver 05/2008; 28(4):455-66. DOI:10.1111/j.1478-3231.2008.01684.x · 4.41 Impact Factor
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