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Abstract：
BACKGROUND: There has been significant improvement in understanding the etiology and management of Budd-Chiari Syndrome (BCS). Patients with chronic or acute-on-chronic BCS need radiological interventions in the form of angioplasty, hepatic vein/inferior vena cava stenting or Transjugular Intrahepatic Portosystemic Shunt (TIPS). Data regarding the long term follow up of patients undergoing TIPS is limited. We thus prospectively followed-up BCS patients who underwent TIPS at our center.
METHODS: This study included 42 patients with BCS who underwent TIPS with a covered stent between 2004 and 2014. We analyzed the etiology, symptoms, severity, laboratory parameters and imaging pre and post TIPS. All patients underwent surveillance for hepatocellular carcinoma.
RESULTS: Patients demographics included 26 males and 16 females with a mean age of 40.5 years (19-68 years). The mean Model for End-Stage Liver Disease score of the entire cohort was 15.38 (range: 9-25). Thirty-four patients were grouped into Rotterdam Class 2 and remaining into Class 3. There was significant improvement in ascites, gastrointestinal bleed, renal function and transaminase levels post TIPS. 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 2.1 weeks and median survival till follow-up was 45.5 months (0-130 months). 33/42 patients underwent TIPS prior to 2013, and their median survival till follow-up was 55 months. Six out of eleven deaths that occurred within six months post-TIPS were before 2006; when the technique of TIPS creation was evolving. The cumulative 1 year, 5 years and 10 years OLT-free survival was 86%, 81% and 76%, respectively. Two patients underwent a liver transplant at 4 and 7 years after TIPS.
CONCLUSIONS: Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, the requirement for liver transplantation has become rare.
METHODS: This study included 42 patients with BCS who underwent TIPS with a covered stent between 2004 and 2014. We analyzed the etiology, symptoms, severity, laboratory parameters and imaging pre and post TIPS. All patients underwent surveillance for hepatocellular carcinoma.
RESULTS: Patients demographics included 26 males and 16 females with a mean age of 40.5 years (19-68 years). The mean Model for End-Stage Liver Disease score of the entire cohort was 15.38 (range: 9-25). Thirty-four patients were grouped into Rotterdam Class 2 and remaining into Class 3. There was significant improvement in ascites, gastrointestinal bleed, renal function and transaminase levels post TIPS. 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 2.1 weeks and median survival till follow-up was 45.5 months (0-130 months). 33/42 patients underwent TIPS prior to 2013, and their median survival till follow-up was 55 months. Six out of eleven deaths that occurred within six months post-TIPS were before 2006; when the technique of TIPS creation was evolving. The cumulative 1 year, 5 years and 10 years OLT-free survival was 86%, 81% and 76%, respectively. Two patients underwent a liver transplant at 4 and 7 years after TIPS.
CONCLUSIONS: Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, the requirement for liver transplantation has become rare.
摘要：
背景：在了解Budd-Chiari综合征（BCS）的病因和管理方面有了显着改善。慢性或慢性BCS患者需要以血管成形术的形式进行放射学干预，肝静脉/下腔静脉支架置入术或经颈静脉肝内门体分流术（TIPS）。有关接受TIPS的患者的长期随访数据有限。因此，我们对在我们中心接受TIPS的BCS患者进行了前瞻性随访。
方法：本研究纳入了42例BCS患者，这些患者在2004年至2014年间接受了带覆膜支架的TIPS治疗。我们分析了病因,症状,严重程度,TIPS前后的实验室参数和成像。所有患者均接受肝细胞癌监测。
结果：患者的人口统计学包括26名男性和16名女性，平均年龄为40.5岁（19-68岁）。整个队列的终末期肝病评分的平均模型为15.38（范围：9-25）。34名患者被分为鹿特丹2级，其余为3级。腹水有显著改善，胃肠出血,TIPS后肾功能和转氨酶水平。随访期间有11例死亡-一个月内4例，2在6个月内，其余在TIPS后3年。从临床表现到TIPS的中位持续时间为2.1周，随访的中位生存期为45.5个月（0-130个月）。33/42例患者在2013年之前接受了TIPS，随访的中位生存期为55个月。在TIPS后六个月内发生的11例死亡中，有6例发生在2006年之前；当TIPS创建技术不断发展时。累计1年,5年和10年无OLT生存率为86%，81%和76%，分别。两名患者在TIPS后4年和7年接受了肝移植。
结论：我们的结果验证了TIPS在治疗BCS患者中的作用。随着TIPS的可访问性，肝移植的需求已经变得罕见。
方法：本研究纳入了42例BCS患者，这些患者在2004年至2014年间接受了带覆膜支架的TIPS治疗。我们分析了病因,症状,严重程度,TIPS前后的实验室参数和成像。所有患者均接受肝细胞癌监测。
结果：患者的人口统计学包括26名男性和16名女性，平均年龄为40.5岁（19-68岁）。整个队列的终末期肝病评分的平均模型为15.38（范围：9-25）。34名患者被分为鹿特丹2级，其余为3级。腹水有显著改善，胃肠出血,TIPS后肾功能和转氨酶水平。随访期间有11例死亡-一个月内4例，2在6个月内，其余在TIPS后3年。从临床表现到TIPS的中位持续时间为2.1周，随访的中位生存期为45.5个月（0-130个月）。33/42例患者在2013年之前接受了TIPS，随访的中位生存期为55个月。在TIPS后六个月内发生的11例死亡中，有6例发生在2006年之前；当TIPS创建技术不断发展时。累计1年,5年和10年无OLT生存率为86%，81%和76%，分别。两名患者在TIPS后4年和7年接受了肝移植。
结论：我们的结果验证了TIPS在治疗BCS患者中的作用。随着TIPS的可访问性，肝移植的需求已经变得罕见。
