Budd-Chiari syndrome and TIPS – 21 years’ experience

Czech version

Authors: V. Šafka ¹; P. Hůlek ^2,3; A. Krajina ⁴; T. Fejfar ²; P. Ďulíček ⁵; Š. Šembera ²; T. Vaňásek ²; V. Jirkovský ²; J. Žižka ⁴; V. Chovanec ⁴; M. Lojík ⁴; J. Raupach ⁴; O. Renc ⁴
Authors‘ workplace: Ústav fyziologie, LF UK, Hradec Králové ¹; II. interní gastroenterologická klinika LF UK a FN Hradec Králové ²; Interní klinika LF OU, Ostrava ³; Radiologická klinika LF UK a FN Hradec Králové ⁴; IV. interní hematologická klinika LF UK a FN Hradec Králové ⁵
Published in: Gastroent Hepatol 2014; 68(2): 116-123
Category: Hepatology: Original Article

Overview

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:
We consider TIPS an advantageous therapeutic approach in BCS due to 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 liver transplantation.

Key words:
Budd-Chiari syndrome – portal hypertension – transjugular intrahepatic – portosystemic shunt

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE „uniform requirements“ for biomedical papers.

Submitted:
12. 3. 2014

Accepted:
7. 4. 2014

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