Long-Term Follow-Up After Successful Transjugular Intrahepatic Portosystemic Shunt Placement in a Pediatric Patient with Budd-Chiari Syndrome

The role of portosystemic shunting in the treatment of the Budd-Chiari syndrome is still under debate. Medical therapy and liver transplantation are alternative treatments. The aim of this study was to determine the outcome of a transjugular intrahepatic portosystemic shunt implantation. Thirty-five patients with severe Budd-Chiari syndrome and a Child-Pugh score of 9.2+/-1.9, who were not responsive to medical therapy, were elected for the transjugular shunt treatment, which was successfully accomplished in 33. Eleven patients had a fulminant/acute (history <2 months); 13, a subacute (<6 months); and 11, a chronic course of the disease. The shunt was established by using conventional self-expandable stents in 25 patients and polytetrafluoroethylene-covered stents in 8 patients. The mean follow-up was 37+/-29 months. The shunt reduced the portosystemic pressure gradient from 29+/-7 to 10+/-4 mm Hg and improved the portal flow velocity from 9.2+/-11 to 51+/-17 cm/s. Clinical symptoms as well as the biochemical test results improved significantly during 4 weeks after shunt treatment. Three patients died and 2 received liver transplants. The cumulative 1- and 5-year survival rate without transplantation in all patients was 93% and 74%, respectively, and in patients with fulminant/acute disease 91% and 91% respectively (no deaths in this time period). On the average, 1.4+/-2.2 revisions per patient were needed during the mean follow-up of 3 years with a 1-year probability of 47%. The transjugular shunt provides an excellent outcome in patients with severe fulminant/acute, subacute, and chronic Budd-Chiari syndrome. It may be regarded as a treatment for the acute and long-term management of these patients.

The long-term outcome of Budd-Chiari syndrome (BCS) with transjugular intrahepatic portosystemic shunts (TIPS) is not well studied. To address this, the records of 47 consecutive patients with BCS evaluated in one center from January 1989 to April 2004, were analyzed. Seven patients with liver tumors were excluded from analyses. Eleven patients had Bechet's disease, 14 had thrombophiliac disorders, four had myeloproliferative diseases and 11 patients had other or unknown causes. The site of block was hepatic vein in 16 patients, in the suprhepatic inferior vena cava in 19 and not known in five. The majority of patients (21/40; 52.5%) presented with subacute disease with massive ascites and abdominal pain as the dominant manifestations. Eight patients with membranes or segemental block were treated with transluminal angiopalsty, and six were treated with clinical and biochemical recovery. The TIPS was placed through a transcaval puncture in eight patients with progressive liver disease who were on medical therapy and had thrombosis limited to hepatic veins. One patient bled from portal vein puncture, which was managed by placing stent across the punctured site. The TIPS was very effective in decreasing portal pressure gradient, improving synthetic functions, reducing transaminase levels and controlling ascites. Five patients had TIPS dysfunction needing revision. In two patients it was difficult to maintain TIPS patency due to repeated TIPS dysfunction. However, both these patients were asymptomatic with normal liver function tests. Long-term follow up revealed that patients with TIPS had significantly better survival than those treated with medical therapy alone (log-rank test, P = 0.04). In a multivariate Cox-model analysis four variables, namely, more florid presentation, male sex, no treatment with TIPS and increasing Child-Pugh-Turcotte score, adversely affected the survival. Budd-Chiari syndrome needs an individualized multidisciplinary approach and TIPS is indicated in a subgroup of patients with progressive liver disease. It is safe, feasible and improves survival.

To retrospectively compare primary patency and reintervention rates, clinical outcome, and improvement in hepatic dysfunction (Child-Pugh score) in patients with Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt (TIPS) using bare stents or expanded polytetrafluoroethylene (PTFE)-covered stents. Approval of study and treatment protocol and waiver of informed consent for the retrospective study were obtained from institutional review board. Informed consent was obtained from each patient before procedure. Four male and nine female consecutive patients (mean age, 35.7 years) with Budd-Chiari syndrome treated with TIPS from January 1994 to November 2003 were included. In six patients, TIPS creation was performed with bare stents (group A). After April 2001, TIPS creation was performed in seven patients with expanded PTFE-covered stent-grafts (group B). Follow-up (group A mean, 22.7 months +/- 10.53 [standard deviation]; group B mean, 23.1 months +/- 7.81) was performed at 7 days, 30 days, and 3 months and subsequently every 3 months. Liver function was evaluated every 3 months. Primary end points were 6- and 12-month TIPS patency rates. Secondary end points were reintervention rate and clinical outcome. Patency rates were estimated (Kaplan-Meier method) and compared (log-rank test). Continuous (Mann-Whitney U test) and categoric (chi(2) test) data were compared. Significant differences before and after treatment were determined (Wilcoxon test). Mean primary patency duration was 4.46 months +/- 3.40 in group A and 22.29 months +/- 8.15 in group B. In group B, a significant increase in primary patency rate (100% and 85.7% vs 16.7% and 0% at 6 and 12 months; P < .001, log-rank test) and a decrease in reintervention rate (0.29 +/- 0.49 vs 6.17 +/- 1.72, P < .001) were observed. Both groups had a significantly decreased Child-Pugh score; group B had a more persistent and significantly higher reduction. Expanded PTFE-covered stent-grafts significantly increased primary patency rate of TIPS in patients with Budd-Chiari syndrome, with reduced reintervention rate and prolonged clinical improvement. (c) RSNA, 2006.