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Abstract

Objectives: Budd-Chiari syndrome is a low-prevalence, life-threatening disorder characterized by hepatic venous outflow obstruction at the hepatic venules, the large hepatic veins, the inferior vena cava, or the right atrium. Orthotopic liver transplant should be considered for patients with fulminant and chronic forms of the syndrome. Materials and Methods: Fourteen patients received 15 orthotropic liver transplants at our center from September 2006 to March 2013. This study retrospectively reviewed the prospectively collected data from these 14 patients. Results: The mean age of the patients was 33 years; only 1 patient was female. The severity of liver disease was Child-Pugh score A in 1 patient, B in 4 patients, and C in 9 patients. Mean calculated Model for EndStage Liver Disease score was 18 (range, 6-30). The cause of Budd-Chiari syndrome was factor 5 Leiden mutation in 3 patients, polycythemia vera in 2 patients, factor 2 and 3 deficiency in 1 patient, fulminant essential throm bocytosis in 1 patient, and protein C deficiency in 2 patients. We performed 15 transplants in 14 patients. Five grafts were obtained from deceased donors, and 10 grafts were from livingrelated donors. Mean graft-to-recipient weight ratio was 1,12 for patients receiving a living-donor liver transplant. Median follow-up was 29 months. Patient survival rates were 87%, 71%, and 71% at 1, 3, and 5 years. Conclusions: Liver transplant is an option for treating Budd-Chiari syndrome in cases of fulminant presentation and cirrhosis. Living-donor liver trans plant is a viable choice in countries where procuring organ donations is still a problem. To manage the long-term medical therapy and follow-up for these patients, a careful evaluation is necessary to determine the cause of Budd-Chiari syndrome. Anticoagulant and antiaggregant therapy remains the mainstay of treatment for this syndrome.

Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis

“…Since patients with MPN have mature and well-differentiated granulocytes, the risk of infectious complications after LT is no higher than in LT patients without MPN. Several case series and retrospective studies 142,145,148 did not reveal evidence of accelerated MPN progression (leukemic transformation) after LT in a 10year follow-up period, and the survival rates were excellent (71->90% over 3 years, comparable to non-MPN patients with BCS).…”

“…In patients with BCS, LT represents the last therapeutic option following treatment failure on other less-invasive therapies. Although it may be a first step in patients who initially present with acute hepatic failure, 142 TIPS should be considered while waiting for LT as it may foster fast improvement and potentially avoid transplantation. It may be acknowledged that LT in patients with BCS represents a technical challenge mainly because of the presence of retroperitoneal fibrosis…”

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

“…Prior TIPS does not compromise the results of liver transplantation. After OLT, given that BCS often has a prothrombotic state, long-term anticoagulation after liver transplantation should be considered as the most important strategy for preventing recurrent BCS[ 48 ].…”