Budd-Chiari syndrome: A single-center experience

AIM:To investigate challenges,risk factors,prognostic indicators,and treatment outcomes associated with Budd-Chiari syndrome(BCS)at a tertiary care center.METHODS:A retrospective cohort study was conducted at the University of Pennsylvania in patients with a diagnosis of BCS or hepatic vein thrombosis.All patients receiving care at the University of Pennsylvania,and who had at least 2 clinical encounters in the University of Pennsylvania Health system from January1,2008 to September 10,2013 were eligible for study inclusion.Data were extracted from the electronic medical record of each patient,and recorded in a secure Research Electronic Data Capture database.Logistic regression analyses were applied to identify predictors of outcome of liver transplant(LT)or death.RESULTS:Between January 1,2008 and September10,2013,forty-seven patients were identified.Median age was 42.4 years.Thirty-one(66.0%)were women.A majority were Caucasian(68.1%).At diagnosis,43(91.5%)patients had ascites,27(57.4%)patients had a hematologic disorder associated with a hypercoagulable state and 26(55.3%)had cirrhosis.Forty(85.1%)patients were on anticoagulation(AC),30(63.8%)of whom were maintained on warfarin.Two patients(4.3%)underwent thrombolytic therapy.A transjugular intrahepatic portosystemic shunt(TIPS)was placed in21(44.7%)patients,19(90.5%)of whom were also on AC.Twenty-one(44.7%)received AC alone.Over a median of 974 d,8(17.0%)patients received LT,and10(21.3%)died.The median time from listing to death was 26 mo[interquartile range(IQR)=16,65)].TIPS with AC was utilized more frequently in younger patients(P=0.02).Age,cirrhosis and chronic kidney disease(CKD)were significant predictors of LT or death.CONCLUSION:AC alone was employed as frequently as TIPS with AC,though the latter was used more frequently in younger patients with polycythemia vera.There were no significant differences in treatment outcome regardless of the therapeutic intervention employed.Significant predictors of poor prognosis included age,cirrhosis and CKD.     

Transjugular intrahepatic portosystemic shunt for severe jaundice in patients with acute Budd-Chiari syndrome

AIM:To evaluate the feasibility of transjugular intrahepatic portosystemic shunt(TIPS)for severe jaundice secondary to acute Budd-Chiari syndrome(BCS).METHODS:From February 2009 to March 2013,37patients with severe jaundice secondary to acute BCS were treated.Sixteen patients without hepatic venule,hepatic veins(HV)obstruction underwent percutaneous angioplasty of the inferior vena cava(IVC)and/or HVs.Twenty-one patients with HV occlusion underwent TIPS.Serum bilirubin,liver function,demographic data and operative data of the two groups of patients were analyzed.RESULTS:Twenty-one patients underwent TIPS and the technical success rate was 100%,with no technical complications.Sixteen patients underwent recanalization of the IVC and/or HVs and the technical success rate was 100%.The mean procedure time for TIPS was 84.0±12.11 min and angioplasty was44.11±5.12 min(P0.01).The mean portosystemic pressure in the TIPS group decreased significantly from 40.50±4.32 to 16.05±3.50 mm Hg(P0.01).The mean portosystemic pressure gradient decreased significantly from 33.60±2.62 to 7.30±2.21 mm Hg(P0.01).At 8 wk after the procedures,in the TIPS group,total bilirubin(TBIL)decreased significantly from 266.24±122.03 before surgery to 40.11±3.52μmol/L(P0.01)and direct bilirubin(DBIL)decreased significantly from 194.22±69.82μmol/L to 29.82±3.10μmol/L(P0.01).In the angioplasty group,bilirubin returned to the normal range,with TBIL decreased significantly from 258.22±72.71μmol/L to 13.33±3.54μmol/L(P0.01)and DBIL from175.08±39.27 to 4.03±1.74μmol/L(P0.01).Liver function improved faster than TBIL.After 2 wk,in the TIPS group,alanine aminotransferase(ALT)decreased significantly from 50.33±40.61 U/L to 28.67±7.02U/L(P0.01)and aspartate aminotransferase(AST)from 49.46±34.33 U/L to 26.89±8.68 U/L(P0.01).In the angioplasty group,ALT decreased significantly from 51.56±27.90 to 14.22±2.59μmol/L(P0.01)and AST from 60.66±39.89μmol/L to 8.18±1.89μmol/L(P0.01).After mean follow-up of 12.6 mo,there was no recurrence of jaundice in either group.CONCLUSION:Severe jaundice is not a contraindication for TIPS in patients with acute BCS and TIPS is appropriate for severe jaundice due to BCS.     

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

Budd-Chiari syndrome(BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt(TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.     

Transjugular intrahepatic portosystemic shunt (TIPS) augments hyperinsulinemia in patients with cirrhosis

BACKGROUND/AIMS: Cirrhosis of the liver is characterized by glucose intolerance and hyperinsulinemia. Both increased insulin secretion and decreased insulin clearance appear to contribute to hyperinsulinemia in cirrhotic patients. A decrease in hepatic insulin extraction rate may be due either to hepatocellular dysfunction or to portosystemic shunting with decreased first-pass insulin clearance. METHODS: To specifically address the contribution of portosystemic shunting to the pathogenesis of hyperinsulinemia in cirrhotic patients, we analyzed glycemic control and insulin levels in fasting serum in 23 cirrhotic patients before and after transjugular intrahepatic portosystemic shunt (TIPS). RESULTS: Compared to respective values in healthy controls, C-peptide, insulin and proinsulin concentrations at baseline were increased by 340%, 120% and by 100% in cirrhotic patients (all p<0.05). In cirrhotic patients insulin levels before TIPS averaged 104+/-73 pmol/l and increased by more than 50% to 163+/-118 pmol/l after TIPS (p<0.01), whereas levels of C-peptide and proinsulin showed no significant change. Glucose and fructosamin levels also remained unchanged after TIPS. CONCLUSION: Our data demonstrate that TIPS does not impair glycemic control in cirrhotic patients and that an increase in portosystemic shunting augments hyperinsulinemia, most likely by decreasing hepatic insulin clearance.     

能谱CT成像定量评估Budd-Chiari综合征患者TIPS术治疗前后肝功能状态临床价值研究*

目的 探讨应用能谱CT成像定量评估Budd-Chiari综合征（BCS）患者在经颈静脉肝内门体静脉分流术（TIPS）治疗前后肝功能状态的临床价值。方法 2012年5月~2018年4月我院收治的BCS患者58例,其中Child-Pugh A级、B级和C级分别为21例、18例和19例。病理学类型为下腔静脉阻塞型15例,肝静脉阻塞型17例和混合型26例。所有患者均接受TIPS治疗,手术前后行能谱CT扫描,采用Couinaud肝脏分段,在I~VIII段及相同层面的腹主动脉勾画感兴趣区（ROI）,计算肝脏各段标准化碘基值。结果 治疗前,Child A级患者I~VIII段标准化碘基值分别为（0.32±0.21）、（0.33±0.12）、（0.31±0.07）、（0.31±0.02）、（0.31±0.02）、（0.31±0.03）、（0.31±0.09）和（0.31±0.11）,均显著高于Child B级患者【分别为（0.28±0.14）、（0.28±0.13）、（0.28±0.08）、（0.27±0.09）、（0.22±0.09）、（0.25±0.10）、（0.27±0.13）和（0.28±0.10）,P<0.05】或Child C级患者【分别为（0.21±0.10）、（0.20±0.09）、（0.19±0.07）、（0.19±0.11）、（0.19±0.12）、（0.19±0.10）、（0.20±0.11）和（0.19±0.10）,P<0.05】;治疗后,随着肝功能的恢复,不同分级的BCS患者不同肝段标准化碘基值也有所升高,并且Child A级患者碘基值升高更明显。结论 应用能谱CT成像定量评估Budd-Chiari综合征患者肝功能状态有重要的临床价值,可以通过标准化碘基值间接反映肝脏的血供状况,对BCS患者治疗疗效进行定量评估,为临床后续治疗提供更多有用的信息。     

Hepatic venous pressure gradient measurement before TIPS for acute variceal bleeding

Hepatic venous pressure gradient (HVPG) is an independent predictor of variceal rebleeding in patients with cirrhosis. After pharmacological and/or endoscopic therapy, the use of a transjugular intrahepatic portosystemic shunt (TIPS) may be necessary in HVPG non-responders, but not in responders. Thus, HVPG measurement may be incorporated into the treatment algorithm for acute variceal bleeding, which further identifies the candidates that should undergo early insertion of TIPS or maintain the traditional pharmacological and/or endoscopic therapy. The potential benefits are to reduce the cost and prevent TIPS-related complications.     

Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt

BACKGROUND: 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. RESULTS: 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. CONCLUSIONS: 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.     

Rebleeding rates following TIPS for variceal hemorrhage in the Viatorr era: TIPS alone versus TIPS with variceal embolization

Purpose  

To compare rebleeding rates following treatment of variceal hemorrhage with TIPS alone versus TIPS with variceal embolization in the covered stent-graft era.     

Transjugular intrahepatic portosystemic shunt for hepatorenal syndrome: A systematic review and meta-analysis

BackgroundHepatorenal syndrome is a severe complication of advanced liver diseases with a dismal prognosis.AimsThis systematic review and meta-analysis aims to explore the efficacy and safety of transjugular intrahepatic portosystemic shunt for the treatment of hepatorenal syndrome.MethodPublications were searched via PubMed and EMBASE databases. The pooled proportion and mean difference were calculated by using a random-effect model.ResultsNine publications were included, in which 128 patients with hepatorenal syndrome were treated with transjugular intrahepatic portosystemic shunt. The pooled short-term and 1-year survival rates were 72% and 47% in type 1 hepatorenal syndrome and 86% and 64% in type 2 hepatorenal syndrome. No lethal procedure-related complications were observed. The pooled rate of hepatic encephalopathy after transjugular intrahepatic portosystemic shunt was 49%. The pooled rate of renal function improvement after transjugular intrahepatic portosystemic shunt was 93% in type 1 hepatorenal syndrome and 83% in any type of hepatorenal syndrome. After transjugular intrahepatic portosystemic shunt, serum creatinine, blood urea nitrogen, serum sodium, sodium excretion, and urine volume were significantly improved; by comparison, serum bilirubin slightly increased, but the difference was not statistically significant.ConclusionLimited evidence suggested a potential survival benefit of transjugular intrahepatic portosystemic shunt in patients with hepatorenal syndrome but with a high incidence of hepatic encephalopathy.     

Portal vein aneurysm associated with Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt: A case report

A 65-year-old woman with Budd-Chiari syndrome(BCS) presented with right upper quadrant pain.A computed tomography(CT) scan showed a saccular aneurysm located at the extrahepatic portal vein main branch measuring 3.2 cm in height and 2.5 cm × 2.4 cm in diameter.The aneurysm was thought to be associatedwith BCS as there was no preceding history of trauma and it had not been present on Doppler ultrasound examination performed 3 years previously.Because of increasing pain and concern for complications due to aneurysm size, the decision was made to relieve the hepatic venous outflow obstruction.Transjugular intrahepatic portosystemic shunt(TIPS) was created without complications.She had complete resolution of her abdominal pain within 2 d and remained asymptomatic after 1 year of follow-up.CT scans obtained after TIPS showed that the aneurysm had decreased in size to 2.4 cm in height and 2.0 cm × 1.9 cm in diameter at 3 mo, and had further decreased to 1.9 cm in height and 1.6 cm × 1.5 cm in diameter at 1 year.     

Transjugular intrahepatic portosystemic shunt (TIPS) for budd-chiari syndrome

Summary The transjugular intrahepatic portosystemic shunt (TIPS) is widely used in the treatment of variceal hemorrhage and portal hypertension associated with cirrhosis. Its potential as a therapy for Budd-Chiari syndrome is less well-known. We report a case of a 15-year-old girl with Budd-Chiari syndrome who was successfully treated with a TIPS, leading to resolution of her ascites and improvement in liver function.     

Transjugular intrahepatic portosystemic shunt (TIPS) for the treatment of refractory ascites

Background: The Transjugular Intrahepatic Portosystemic Shunt (TIPS) corrects portal hypertension and has proven to be effective in controlling variceal bleeding in patients with cirrhosis. Several reports have now appeared suggesting a possible role in patients with refractory ascites.
Aims: To examine the outcome of TIPS for the treatment of refractory ascites in patients with cirrhosis.
Methods: Fifteen patients underwent TIPS for ascites between April 1992 and December 1996. The clinical findings, response to treatment, complications, shunt patency and survival of these patients were analysed.
Results: TIPS was successfully placed in all patients. The mean period of follow-up was 375 days (range: 14–1165 days). In eight patients (53%) there was a reduction in the degree of ascites after shunt insertion, with six patients (40%) having complete resolution. Age, Child-Pugh class or portal pressure gradient, before or after the procedure, were not predictive of response. Of five patients with renal insufficiency (serum creatinine >130 umol/L), only one had improvement in ascites control. Six patients (40%) required shunt revision during follow-up, either for acute thrombotic occlusion (two) or stent stenosis (four). New or worsening encephalopathy developed in ten patients (67%). Two patients (13%) died of liver failure within 30 days. Cumulative survival was 46% at one year and 18% at two years. Treatment response was associated with increased survival (ρ=0.02), with median survival of 658 days as compared with 71 days for treatment failure.
Conclusions: TIPS can be effective in the treatment of refractory ascites in patients with cirrhosis. Our experience suggests the benefit may be less for patients with advanced liver disease and renal impairment. Controlled trials are needed to compare TIPS with other treatment modalities such as large volume paracentesis or peritoneovenous shunting.     

Selection of treatment modalities for Budd-Chiari Syndrome in China:A preliminary survey of published literature

AIM:To evaluate the frequency of use of various treatment modalities for Budd-Chiari syndrome(BCS)in China by conducting a preliminary survey of relevant literature.METHODS:All papers regarding the treatment of BCS in Chinese patients were identified by searching PubMed,Chinese Scientific and Technological Journal,and China National Knowledge Infrastructure databases.Data regarding the number of BCS patients treated with different treatment modalities over time were collected.The proportions of BCS patients undergoing various treatment modalities were calculated.RESULTS:Overall,300 of 3005 papers initially retrieved were included.These papers included 23352BCS patients treated with different treatment modalities.The treatment modalities include surgery(n=8625),interventional treatment(n=13940),surgery combined with interventional treatment(n=363),medical therapy(n=277),other treatments(n=91),and no treatment(n=56).After 2005,the number of BCS patients treated with surgery was drastically decreased,but the number of BCS patients who underwent interventional treatment was almost maintained.Shunt surgery was the most common type of surgery(n=3610).Liver transplantation was rarely employed(n=2).Balloon angioplasty with or without stenting was the most common type of interventional treatment(n=13747).Transjugular intrahepatic portosystemic shunt was rarely employed(n=81).CONCLUSION:Selection of treatment modalities for BCS might be different between China and Western countries.Further work should be necessary to establish a unanimous therapeutic strategy for BCS in China.     

应用利伐沙班抗凝治疗经皮血管内介入治疗的布加综合征患者疗效和安全性研究

目的 探讨应用利伐沙班作为口服抗凝剂在布加综合征患者接受经皮血管内介入治疗后长期治疗的有效性和安全性.方法 2017年12月～2020年4月我科收治的103例布加氏综合征患者,均接受经皮血管内介入治疗,术后服用利伐沙班抗凝治疗49例,服用阿司匹林54例.术后随访.结果 术后,利伐沙班组和阿司匹林组平均随访时间分别为20...     

Liver failure after an uncovered TIPS procedure associated with hepatic infarction

Transjugular intrahepatic portosystemic shunt (TIPS) is a safe and effective procedure for the treatment of complications of liver cirrhosis, such as refractory ascites, hepatic hydrothorax and refractory variceal bleeding. The aim of this paper is to describe a rare case of liver failure after a TIPS procedure. A 38-year-old diabetic male with Child-Pugh C liver cirrhosis due to chronic hepatitis C infection who had developed refractory ascites was scheduled for a TIPS procedure. Within 24 h following TIPS placement, the patient developed distributive shock, jaundice, persistentgrade 3 hepatic encephalopathy, severe coagulopathy and acute renal failure. He was treated with lactulose enemas, broad-spectrum antibiotics and blood-derived products. Laboratory data revealed a 100-fold increase in aminotransferases and a non-enhanced computed tomography showed an irregular hypodense area in the right posterior segment of the liver. Despite being initially being in a stable condition, the patient developed progressive liver failure and died 2 mo later. Hepatic infarction is an uncommon phenomenon after a TIPS procedure; however, it can greatly complicate the course of a disease in a patient with an already compromised liver function.     

Viatorr覆膜支架用于TIPS术治疗肝硬化门静脉高压症患者预后研究

目的 研究Viatorr覆膜支架用于经颈静脉肝内门体静脉分流术(TIPS)治疗肝硬化门静脉高压症患者的疗效和转归.方法 2018年1月～2020年1月我院收治的肝硬化并发门静脉高压症患者146例,采用随机数字表法将患者分为对照组73例和观察组73例,在接受TIPS术治疗时分别使用裸支架联合Fluency覆膜支架或Via...     

Stent-grafts for the treatment of TIPS dysfunction:Fluency stent vs Wallgraft stent

AIM:To evaluate the clinical efficacy of an expanded polytetrafluoro-ethylene-covered Fluency stent compared with that of a polyethylene terephthalate-covered Wallgraft stent for the management of transjugular intrahepatic portosystemic shunt(TIPS)dysfunction.METHODS:A retrospective review of patients who underwent TIPS revision with stent-grafts between May 2007 and June 2011 was conducted.The patients were divided into two groups according to the stentgrafts implanted:the Fluency stent(Bard Incorporated,Karlsruhe,Germany)and the Wallgraft stent(Boston Scientific,Galway,Ireland).The primary patency rates were calculated and compared using the Kaplan-Meier method.RESULTS:A total of 73 patients were evaluated in this study:33 with Fluency stents and 40 with Wallgraft stents.The primary patency rates at 12 and 24 mo were 91% and 85%,respectively,in the Fluency stent group and 78% and 63%,respectively,in the Wallgraft stent group.The primary shunt patency rates after TIPS revision were significantly better with the Fluency stent than with the Wallgraft stent(P = 0.033).CONCLUSION:TIPS revision with the Fluency stent has higher medium-term patency rates than that with the Wallgraft stent.