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 Fig. 1. Two year follow up shunt venogram demonstrates intratract and hepatic vein stenoses. When measured from multiple oblique venograms, the mean luminal reduction exceeded 50%. |
 Fig. 2. Prograde intrahepatic flow is visible. |
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A 22-year-old woman underwent TIPS for treatment of acute Budd-Chiari syndrome over 2 years ago. At the time, she had marked ascites and related symptoms. The etiology was never clearly identified, though it may have been related to ulcerative colitis and azathioprine use - diagnoses of exclusion in the face of an extensive hematologic workup. The initial TIPS gradient was 46 mm Hg, reduced to 19 mm Hg with a 12 mm diameter Wallstent-lined shunt. Several days later after diuresis of most of her ascitic and third space fluids, her portosystemic gradient dropped further to 8 mm Hg. She has undergone periodic venography and serial laboratory studies during follow-up and remains on anticoagulation. Two follow up venograms from 2-year follow up are shown. Both intra-tract and hepatic vein stenoses are seen, exceeding 50% in measured luminal reduction. This translates to a great reduction in the trans-shunt flow (proportional to the fourth). Despite this, her portosystemic gradient measured 7 mm Hg and a transjugular liver biopsy, performed through the mesh of stent, revealed no evidence of significant hepatic fibrosis. The shunt stenoses have not changed in appearance during several iterative exams. We have elected to leave this shunt in this stenotic, yet 'stable' venographic appearance as it appears to provide sufficient decompression, as evidenced by a satisfactory liver biopsy. We follow a number of such patients with Budd-Chiari treated with TIPS in whom different degrees of shunt patency are accepted as necessary to maintain hepatic decompression and prevent hepatic fibrosis in an otherwise normal liver. In recent years, we have begun performing serial biopsies in all these patients to assess the efficacy of the TIPS in this setting and, accordingly, assist in providing the patients long term prognostic information about the likelihood of progressive liver disease. To date, only one patient has required liver transplantation-a patient with hepatic fibrosis when initially referred for TIPS. Questions: 1) What are "suitable" gradients for decompression of TIPS in Budd-Chiari syndrome? 2) How should we best follow these patients? 3) How has the role of TIPS in Budd-Chiari syndrome evolved and what patients should currently undergo TIPS, and when? 4) Have others seen and dealt with acute shunt thromboses during shunt creation in these patients, as I have? I have taken to administering very high doses of heparin during TIPS formation and high degrees of anticoagulation for the first month after TIPS. Related Cases: Case 0498_09 --Hepatic vein occlusion Case 597_10-- Fine needle TIPS for Budd-Chiari Syndrome MMVI_06 TIPS-Mismanagement of Budd-Chiari Syndrome Case 0599_02 -- Budd-Chiari Syndrome Presenting with Right Hydrothorax: Treatment with TIPS |
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