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- W4235288735 abstract "Background & Aims: Budd–Chiari syndrome (BCS) is an uncommon disorder defined as a hepatic venous outflow obstruction at any level between the hepatic veins and the right atrium. It usually involves thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. Treatment of BCS includes medical, interventional radiological (TIPS) and surgical interventions. As the disease is rare, there is limited data regarding the long term survival of patients undergoing TIPS. The aim of this study was to report our single centre long term follow-up results of patients with BCS who underwent TIPS.Methods: 42 BCS patients underwent TIPS with covered stent between 2004 and 2014. Retrospective analysis was performed regarding the presenting symptoms and laboratory workup pre- and post-TIPS, etiology of BCS, extent of thrombosis, status of varices, therapeutic interventions, complications, symptom relief and overall outcome.Results: 16 women and 26 men with a mean age of 40 years (range: 14–60 years) at time of diagnosis were included. Myeloproliferative disorder and JAK 2 mutation were the commonest etiology followed by MTHFR mutation. The cause of BCS was unclear in 24 patients. There was significant improvement in ascites, GI bleed, HRS and transaminase levels post TIPS. 3 patients required a TIPS plasty and one patient underwent TIPS reduction. There were 11 deaths over the follow-up period, 4 within one month, 2 within six months and the rest after 3 years following TIPS. Median duration from clinical presentation to TIPS was 6 weeks and median survival till follow-up was 55.3 months (0–130 months). 33/42 patients underwent TIPS prior to 2013, their median survival till follow-up was 64.2 months.Conclusion: Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, requirement for liver transplantation has become rare. Background & Aims: Budd–Chiari syndrome (BCS) is an uncommon disorder defined as a hepatic venous outflow obstruction at any level between the hepatic veins and the right atrium. It usually involves thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. Treatment of BCS includes medical, interventional radiological (TIPS) and surgical interventions. As the disease is rare, there is limited data regarding the long term survival of patients undergoing TIPS. The aim of this study was to report our single centre long term follow-up results of patients with BCS who underwent TIPS. Methods: 42 BCS patients underwent TIPS with covered stent between 2004 and 2014. Retrospective analysis was performed regarding the presenting symptoms and laboratory workup pre- and post-TIPS, etiology of BCS, extent of thrombosis, status of varices, therapeutic interventions, complications, symptom relief and overall outcome. Results: 16 women and 26 men with a mean age of 40 years (range: 14–60 years) at time of diagnosis were included. Myeloproliferative disorder and JAK 2 mutation were the commonest etiology followed by MTHFR mutation. The cause of BCS was unclear in 24 patients. There was significant improvement in ascites, GI bleed, HRS and transaminase levels post TIPS. 3 patients required a TIPS plasty and one patient underwent TIPS reduction. There were 11 deaths over the follow-up period, 4 within one month, 2 within six months and the rest after 3 years following TIPS. Median duration from clinical presentation to TIPS was 6 weeks and median survival till follow-up was 55.3 months (0–130 months). 33/42 patients underwent TIPS prior to 2013, their median survival till follow-up was 64.2 months. Conclusion: Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, requirement for liver transplantation has become rare." @default.
- W4235288735 created "2022-05-12" @default.
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- W4235288735 date "2015-06-01" @default.
- W4235288735 modified "2023-10-16" @default.
- W4235288735 title "Long Term Survival of Patients Undergoing Tips in Budd–Chiari Syndrome" @default.
- W4235288735 doi "https://doi.org/10.1016/j.jceh.2015.07.105" @default.
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