Budd-Chiari syndrome and TIPS - 21 years' experience
Václav Šafka Orcid.org , Petr Hůlek1,2, Antonín Krajina Orcid.org 3, Tomáš Fejfar Orcid.org 1, Petr Dulíček Orcid.org 4, Štěpán Šembera Orcid.org 1, Tomáš Vaňásek Orcid.org 5, Václav Jirkovský Orcid.org 1, Jan Žižka Orcid.org , Vendelín Chovanec Orcid.org 3, Ondřej Renc Orcid.org 3, Miroslav Lojík3, Jan Raupach3
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Summary
Budd-Chiari syndrome (BCS) often has an acute or fulminant course leading to ischaemia and necrosis of the liver parenchyma as consequence of venostasis. Restitution of blood drainage is then a key measure. In the chronic course, symptoms of portal hypertension are dominant. TIPS as a portosystemic shunt ensures both venous drainage as well as corrected portal hypertension.
Patients and methods: TIPS has already been available for 21 years in the Czech Republic. Using this method we also treated 52 patients with BCS from thrombosis of hepatic veins. The median age was 37 years (13 to 82 years), 10% were children, 16 patients (31%) were men, 31% procedures were urgent. Myeloproliferative syndrome was the cause in 60%, another thrombophilic disorder was detected in 15%, and in 25% of them the cause was not elucidated. In the first five years, uncovered stents were used, but since 1997 different types of ePTFE covered stents have been available and since 2001 dedicated ePTFE covered stents became the standard.
Results: 13 patients died: three of fulminant liver failure, one of extensive tumorous thrombosis, three of early septic complications, two of liver failure due to later acute shunt occlusion, two of progressions of haemato-oncological disease, one tragically, and one of unknown cause. Two patients later underwent OLTx. In 39 patients the shunt remains patent on anticoagulant therapy and occasional reinterventions, liver functions are stable and no portal hypertension complications occur. We have not noticed significant hepatic encephalopathy. The use of ePTFE covered stents reduced a number of mainly early occlusions. In imaging we often find nodular hyperplasia.
Conclusion: WeconsiderTIPS an advantageous therapeutic approach in BCS dueto thrombosis of the hepatic veins. If the follow-up treatment is rigorous, the TIPS usually ensures sufficient perfusion of the liver and prevents portal hypertension complications, saving most patients from livertransplantation.
Keywords
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