Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers.

BACKGROUND AND AIMS: Management of Budd-Chiari syndrome (BCS) has improved over the last decades. The main aim was to evaluate the contemporary post-liver transplant (post-LT) outcomes in Europe. APPROACH AND RESULTS: Data from all patients who underwent transplantation from 1976 to 2020 was obtained from the European Liver Transplant Registry (ELTR). Patients < 16 years, with secondary BCS or HCC were excluded. Patient survival (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression analysis identified predictors of PS and GS after 2000. Supplemental data was requested from all ELTR-affiliated centers and received from 44. In all, 808 patients underwent transplantation between 2000 and 2020. One-, 5- and 10-year PS was 84%, 77%, and 68%, and GS was 79%, 70%, and 62%, respectively. Both significantly improved compared to outcomes before 2000 ( p < 0.001). Median follow-up was 50 months and retransplantation rate was 12%. Recipient age (aHR:1.04,95%CI:1.02-1.06) and MELD score (aHR:1.04,95%CI:1.01-1.06), especially above 30, were associated with worse PS, while male sex had better outcomes (aHR:0.63,95%CI:0.41-0.96). Donor age was associated with worse PS (aHR:1.01,95%CI:1.00-1.03) and GS (aHR:1.02,95%CI:1.01-1.03). In 353 patients (44%) with supplemental data, 33% had myeloproliferative neoplasm, 20% underwent TIPS pre-LT, and 85% used anticoagulation post-LT. Post-LT anticoagulation was associated with improved PS (aHR:0.29,95%CI:0.16-0.54) and GS (aHR:0.48,95%CI:0.29-0.81). Hepatic artery thrombosis and portal vein thrombosis (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). CONCLUSIONS: LT for BCS results in excellent patient- and graft-survival. Older recipient or donor age and higher MELD are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.

A novel two-stage approach to the treatment of renal cell carcinoma with intra-cardiac tumour extension and Budd-Chiari syndrome.

OBJECTIVE: To present the early results of a new technique for the treatment of renal cell carcinoma with intra-cardiac tumour extension and Budd-Chiari syndrome. PATIENTS AND METHODS: The first stage involves transdiaphragmatic debulking of the right heart, inferior vena cava (IVC) and hepatic veins via median sternotomy, followed by a purse-string suture placed in the IVC below the hepatic veins. The second stage is performed separately and involves en bloc resection of the affected kidney, and IVC and vascular reconstruction via an abdominal incision. RESULTS: Three of five patients presented with clinical Budd-Chiari syndrome; two had radiological features only. The median time between surgical procedures was 12 days (IQR 13 days). Four of the five patients had a R0 resection. While all five patients successfully completed both operative stages, one patient died 22 days after the second stage. Of the remaining four, all survive with no disease recurrence. CONCLUSION: While we continue to compile longer-term data for a larger follow-up series, these preliminary findings show the feasibility of this technique and support the development of this programme of surgery.

Pediatric living donor liver transplant for Budd-Chiari syndrome using a cryopreserved pulmonary vein graft for retro-hepatic vena cava reconstruction: A case report.

INTRODUCTION: In pediatric patients with Budd-Chiari syndrome (BCS), living donor liver transplantation (LDLT) raises substantial challenges regarding IVC reconstruction. CASE PRESENTATION: We present a case of an 8-year-old girl with BCS caused by myeloproliferative syndrome with JAK2 V617F mutation. She had a complete thrombosis of the inferior vena cava (IVC) with multiple collaterals, developing a Budd-Chiari syndrome. She underwent LDLT with IVC reconstruction with a cryopreserved pulmonary vein graft obtained from a provincial biobank. The living donor underwent a laparoscopic-assisted left lateral hepatectomy. The reconstruction of the vena cava took place on the back table and the liver was implanted en bloc with the reconstructed IVC in the recipient. Anticoagulation was immediately restarted after the surgery because of her pro-thrombotic state. Her postoperative course was complicated by a biliary anastomotic leak and an infected biloma. The patient recovered progressively and remained well on outpatient clinic follow-up 32 weeks after the procedure. CONCLUSION: IVC reconstruction using a cryopreserved pulmonary vein graft is a valid option during LDLT for pediatric patients with BCS where reconstruction of the IVC entails considerable challenges. Early referral to a pediatric liver transplant facility with a multidisciplinary team is also important in the management of pediatric patients with BCS.

[Analysis of risk factors of short-term prognosis in patients with severe Budd-Chiari syndrome].

Objective: To explore the risk factors of short-term prognosis of severe Budd-Chiari syndrome (BCS) patients,established and verified the nomogram prediction model for these BCS patients and evaluated its clinical application value. Methods: This study is a retrospective cohort study. The clinical data of 171 patients with severe BCS diagnosed were retrospectively analyzed in the Department of Hepatopancreatobiliary Surgery First Affiliated Hospital of Zhengzhou University from January 2018 to December 2023. There were 105 males and 66 females, aged (52.1±12.8) years (range: 18 to 79 years). The patients were divided into two groups based on whether they died within 28 days: the death group (n=38) and the survival group (n=133). The risk factors for short-term death of patients were analyzed,and independent risk factors were screened by univariate and multivariate analysis. Furthermore,these factors were used to establish the nomogram prediction model. The area under the curve(AUC),the Bootstrap Resampling,the Hosmer-Lemeshow test and the Decision Curve Analysis(DCA) were used to verify the model's differentiation,internal verification,calibration degree and clinical effectiveness,respectively. Results: Univariate and multivariate Logistics regression analysis showed that the history of hepatic encephalopathy,white blood cell,glomerular filtration rate and prothrombin time were independent risk factors (P<0.05). The above factors were used to successfully establish the prediction model with 0.908 of AUC and 0.895 of the internal verification of AUC,indicating that the predictive model was valuable. The 0.663 P-values in the Hosmer-Lemeshow test indicated the high calibration degree of the model. The clinical effectiveness of the model was proved by the 18% clinical benefit population using the DCA curve with the 17% probability threshold. Conclusions: The independent risk factors are the history of hepatic encephalopathy,white blood cell,glomerular filtration rate and prothrombin time. An adequate basis was acquired by establishing a nomogram prediction model of the short-term prognosis of severe BCS,which was helpful for early clinical screening and identification of high-risk patients with severe BCS who could die in the short term and timely providing timely intervention measures for improving the prognosis.

A Systematic Review of the Safety and Efficacy of Inferior Vena Cava Stenting.

OBJECTIVE: Inferior vena cava (IVC) stenting may provide benefit to patients with symptomatic obstruction; however, there are no devices currently licensed for use in the IVC and systematic reviews on the topic are lacking. The aim of this study was to carry out a systematic review of the literature and meta-analysis to investigate the safety and efficacy of IVC stenting in all adult patient groups. DATA SOURCES: The Medline and Embase databases were searched for studies reporting outcomes for safety and effectiveness of IVC stenting for any indication in series of 10 or more patients. REVIEW METHODS: A systematic review of the literature was carried out according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. RESULTS: Thirty-three studies were included describing 1 575 patients. Indications for stenting were malignant IVC syndrome (229 patients), thrombotic disease (807 patients), Budd-Chiari syndrome (501 patients), and IVC stenosis post liver transplantation (47 patients). The male:female ratio was 2:1 and the median age ranged from 30 to 61 years. The studies included were not suitable for formal meta-analysis as 30/33 were single centre retrospective studies with no control groups and there was considerable inconsistency in outcome reporting. There was significant risk of bias in 94% of studies. Median reported technical success was 100% (range 78 - 100%), primary patency was 75% (38 - 98%), and secondary patency was 91.5% (77 - 100%). Major complications were pulmonary embolism (three cases), stent migration (12 cases), and major bleeding (15 cases), and there were three deaths in the immediate post-operative period. Most studies reported improvement in clinical symptoms but formal reporting tools were not used consistently. CONCLUSION: The evidence base for IVC stenting consists of predominantly single centre, retrospective, observational studies that have a high risk of bias. Nonetheless the procedure appears safe with few major adverse events, and studies that reported clinical outcomes demonstrate improvement in symptoms and quality of life. Randomised controlled trials and prospective registry based studies with larger patient numbers and standardised outcome are required to improve the evidence base for this procedure.

Accessory hepatic vein recanalization for Budd-Chiari syndrome: a systematic review and meta-analysis.

BACKGROUND: Budd-Chiari syndrome (BCS) results when the outflow of the hepatic vein (HV) is obstructed. BCS patients exhibiting an accessory HV (AHV) that is dilated but obstructed can achieve significant alleviation of liver congestion after undergoing AHV recanalization. This meta-analysis was developed to explore the clinical efficacy of AHV recanalization in patients with BCS. MATERIALS AND METHODS: PubMed, Embase, and Wanfang databases were searched for relevant studies published as of November 2022, and RevMan 5.3 and Stata 12.0 were used for pooled endpoint analyses. RESULTS: Twelve total studies were identified for analysis. Pooled primary clinical success, re-stenosis, 1- and 5-year primary patency, 1- and 5-year secondary patency, 1-year overall survival (OS), and 5-year OS rates of patients in these studies following AHV recanalization were 96%, 17%, 91%, 75%, 98%, 91%, 97%, and 96%, respectively. Patients also exhibited a significant reduction in AHV pressure after recanalization relative to preoperative levels (P < 0.00001). Endpoints exhibiting significant heterogeneity among these studies included, AHV pressure (I2 = 95%), 1-year primary patency (I2 = 51.2%), and 5-year primary patency (I2 = 62.4%). Relative to HV recanalization, AHV recanalization was related to a lower rate of re-stenosis (P = 0.002) and longer primary patency (P < 0.00001), but was not associated with any improvements in clinical success (P = 0.88) or OS (P = 0.29) relative to HV recanalization. CONCLUSIONS: The present meta-analysis highlights AHV recanalization as an effective means of achieving positive long-term outcomes in patients affected by BCS, potentially achieving better long-term results than those associated with HV recanalization.

Secondary Budd-Chiari syndrome occurred after adjuvant radiotherapy for perihilar cholangiocarcinoma: a case report.

BACKGROUND: Budd-Chiari syndrome (BCS) is a rare vascular disorder of the liver, and acute and secondary BCS is even rarer. CASE PRESENTATION: A 62-year-old man with perihilar cholangiocarcinoma of Bismuth type IIIa underwent right hemi-hepatectomy with caudate lobectomy and pancreatoduodenectomy. Adjuvant chemoradiotherapy was performed due to a positive hepatic ductal margin. Subsequently, the disease passed without recurrence. The patient visited for acute onset abdominal pain at the 32nd postoperative month. Multidetector-row computed tomography (MDCT) showed stenosis of the left hepatic vein (LHV) root, which was the irradiated field, and thrombotic occlusion of the LHV. The patient was diagnosed with acute BCS caused by adjuvant radiotherapy. Although anticoagulation therapy was performed, the patient complained of sudden upper abdominal pain again. MDCT showed an enlarged LHV thrombus and hepatomegaly. The patient was diagnosed with exacerbated acute BCS, and stenting for the stenotic LHV root was performed with a bare stent. Although stenting for the LHV root was very effective, restenosis occurred twice due to thrombus in the existing stent, so re-stenting was performed twice. The subsequent clinical course was acceptable without recurrence or restenosis of the LHV root as of 6 months after the last stenting using a stent graft. CONCLUSION: Although no case of BCS caused by radiotherapy has yet been reported, the present case showed that late side effect of radiotherapy can cause hepatic vein stenosis and secondary BCS.

Innovation in direct intrahepatic portosystemic shunt; a developing country experience.

Anticoagulants are the first-line treatment option for patients with Budd-Chiari syndrome (BCS); however, intervention is required when this fails. Although, the ultimate treatment is liver transplant, other radiological procedures are for the management of the disease and bridge to definitive therapy. TIPS (trans jugular intrahepatic portosystemic shunt) is a method used by interventional radiologists to create a shunt between portal vein and hepatic vein. At times it is technically not possible, in such cases, direct intrahepatic portosystemic shunt (DIPS) is performed. This patient underwent a successful DIPS procedure for BCS along with balloon dilatation (venoplasty) for inferior vena cava (IVC) stenosis.

Percutaneous recanalization in hepatic vein-type Budd-Chiari syndrome: hepatic or accessory hepatic vein.

PURPOSE: This study was designed to assess the clinical efficiency and long-term outcomes of hepatic vein (HV) and accessory hepatic vein (AHV) recanalization in patients with HV-type Budd-Chiari syndrome (BCS). MATERIAL AND METHODS: A total of 27 patients with HV-type BCS underwent AHV recanalization and 94 patients had HV recanalization at our center from January 2012 to December 2019. The treatment effectiveness and long-term outcomes were compared. RESULTS: Technical success was accomplished in all patients, without any procedure-related complications. The clinical success rates were 96.3% (26/27) and 95.7% (90/94) (p = 1.000). In the AHV and HV groups, re-obstruction was observed in 5 and 36 patients, respectively (p = 0.056). The median primary durations of AHV and HV patency were 64 and 49 months, respectively (p = 0.036), while the median secondary durations of AHV and HV patency were 70 and 64 months, respectively (p = 0.134). The median overall survival after AHV and HV recanalization was 73 and 78 months, respectively (p = 0.263). CONCLUSIONS: Our findings suggest that AHV could be employed as a replacement for HV, as a hepatic drainage vein, in HV-type BCS patients.

Efficacy and Safety of Transjugular Intrahepatic Portosystemic Shunt Creation for Budd-Chiari Syndrome: A Systematic Review and Meta-Analysis.

PURPOSE: To assess the critical role of transjugular intrahepatic portosystemic shunt (TIPS) in the management of Budd-Chiari syndrome (BCS), as the data with respect to the safety and outcome of TIPS in patients with BCS are scarce because of the rarity of the disease. MATERIALS AND METHODS: A comprehensive search of literature of various databases from 2000 to October 2021 was conducted for studies evaluating the outcome of TIPS in patients with BCS. The primary outcomes of the analysis were technical and clinical success, adverse events and mortality associated with TIPS, dysfunction of TIPS, need for TIPS revision, need for liver transplantation (LT), and 1-year survival. RESULTS: A total of 33 studies (1,395 patients) were included in this meta-analysis. The pooled rates and 95% confidence intervals of various outcomes were 98.6% (97.6-99.7) for technical success, 90.3% (86.0-94.6) for clinical success, 10.0% (6.5-13.6) for major adverse events, 0.5% (0.2-1.0) for TIPS-related mortality, 11.6% (7.8-15.4) for post-TIPS hepatic encephalopathy (HE), 40.1% (32.5-47.7) for TIPS dysfunction, 8.6% (4.9-12.4) for the need for TIPS revision, 4.5% (2.8-6.2) for the need for LT, and 94.6% (93.1-96.1) for 1-year survival. Publication bias was seen with all outcomes except for post-TIPS HE, TIPS dysfunction, and the need for LT. CONCLUSIONS: The existing literature supports the feasibility, safety, and efficacy of TIPS in the treatment of BCS. Deciding the optimal timing of TIPS in BCS needs further studies.

Living Donor Liver Transplantation for Budd-Chiari Syndrome: A Propensity Score-Matched Analysis.

INTRODUCTION: There are unique technical and management challenges associated with living donor liver transplantation (LDLT) for Budd-Chiari Syndrome (BCS). The outcomes of LDLT for BCS in comparison to other indications remain unclear and warrant elucidation. METHODS: Data of 24 BCS patients who underwent LDLT between January 2012 and June 2019 were analyzed. There were 20 adults and 4 children. The early and long-term outcomes of adult LDLT BCS patients were compared to a control group of LDLT patients for other indications and matched using propensity scoring methodology. RESULTS: Primary BCS was observed in 18 (90%) patients. Caval replacement was performed in 7 (35%) patients. Early and late hepatic venous outflow tract (HVOT) complications were seen in 1 (5%) and 3 (15%) patients. Preoperative acute kidney injury was identified as a risk factor for mortality in the BCS cohort (p = 0.013). On comparison, BCS recipients were younger with fewer comorbidities, more large volume ascites and higher rates of PVT. They also had longer cold ischemia time, increased blood loss and transfusion requirements, increased hospital stay, and higher late outflow complications. The 1-year and 3-year survivals were similar to non-BCS cohort (84.2% vs. 94% and 71.3% vs. 91.9%, respectively, log rank test p = 0.09). CONCLUSION: LDLT is a good option for symptomatic BCS who have failed non-transplant interventions. The clinical and risk factor profile of BCS recipients is distinct from non-BCS recipients. By following an algorithmic management protocol, we show on propensity-score matched analysis that outcomes of LDLT for BCS are similar to non-BCS indications.

[SOLVING A MEDICAL MYSTERY THROUGH THE EYES OF A YOUNG PATIENT WITH BUDD-CHIARI SYNDROME AND A NEW VISION LOSS].

INTRODUCTION: Budd-Chiari syndrome is a heterogeneous group of disorders characterized by venous drainage obstruction of the liver and is extremely rare. The clinical manifestations are usually ascites, varicose veins and in severe cases - hepatic insufficiency. Behcet's disease is a chronic, idiopathic, inflammatory disease that manifests as obstructive vasculitis and affects a variety of organ systems. Ocular involvement occurs in approximately 70% of the patients, and is a major clinical criterion in the diagnosis. Rarely, Behcet's disease can be a cause of Budd-Chiari syndrome. In these cases, the diagnosis has a crucial impact on the treatment and prognosis of the patients since patients with Budd-Chiari syndrome secondary to Behcet's disease, will usually improve under systemic medications with no need for surgery. In addition, in these patients there is a higher chance for developing hepatocellular carcinoma so they need to have a tight and a long follow-up. DISCUSSION: In this article we discuss a case of a young patient with Budd-Chiari syndrome, who was examined due to acute vision loss in his left eye. Left eye examination revealed panuveitis presenting with anterior uveitis, intermediate uveitis and an occlusive retinal vasculitis. The ocular examination raised suspicion that the diagnosis was Behcet's disease. Actually, Budd-Chiari syndrome was part of the presentation of Behcet's disease, which was not diagnosed until he was examined by us. The patient was treated with corticosteroid therapy and biological treatment with adalimumab, an anti-TNF drug. During follow-up, a complete resolution of the intraocular inflammation was achieved, as well as stabilization of its general condition, with the disappearance of the clinical signs indicative of liver failure.

Outcomes of status 1 liver transplantation for Budd-Chiari Syndrome with fulminant hepatic failure.

There is a paucity of data on the outcome of liver transplantation (LT) in Budd-Chiari Syndrome (BCS) patients who are listed as status 1. The objective of our study was to determine patient or graft survival following LT in status 1 BCS patients. We utilized United Network for Organ Sharing (UNOS) database to identify all adult patients (> 18 years of age) listed as status 1 with a primary diagnosis of BCS in the United States from 1998 to 2018, and analyzed their outcomes and compared it to non-status 1 BCS patients. Four hundred and forty-six patients with BCS underwent LT between 1998 and 2018, and of these 55 (12.3%) were listed as status 1. There was no difference in long-term post-liver transplant or "intention-to-treat" survival from the time of listing to death or the last day of follow-up between status 1 and non-status 1 groups. Graft and patient survival at 5 years for status 1 patients were 75% and 82%, respectively. Cox regression analysis showed that patients listed as status 1 (aHR: 0.45, p < .02) were associated with a better survival. BCS patients listed as status 1 have excellent survival following emergency LT.

Techniques and Outcomes of Transjugular Intrahepatic Portosystemic Shunting in Infants with Budd-Chiari Syndrome.

PURPOSE: To describe the technical aspects, feasibility, and outcomes in children with Budd-Chiari syndrome (BCS) undergoing transjugular intrahepatic portosystemic shunt (TIPS) creation during infancy. MATERIALS AND METHODS: A retrospective review of infants with BCS undergoing TIPS creation between January 2012 and December 2018 was performed. Eight infants (5 males) underwent TIPS creation (7 for refractory ascites and 1 for refractory variceal bleeding) during the study period. The median age at TIPS creation was 10.5 months (range, 8-16 months). The median elapsed time between presentation and TIPS creation was 6.5 months (range, 0-13 months). The median weight and median pediatric end-stage liver disease score of the infants at the time of TIPS creation were 6.7 kg (range, 5.4-10 kg) and 13 kg (range, 8-18 kg), respectively. RESULTS: TIPS creation was successful in all patients. There were no immediate postprocedural complications. An 18-gauge hollow needle was manually curved, through which a 21-gauge Chiba needle was inserted to access the portal vein. All patients received 1 or 2 overlapping bare metal stents. One patient was lost to follow-up after the procedure. The median follow-up duration was 32 months (range, 14-51 months). Four of 7 infants needed reintervention. Two children died during the follow-up period. Two children successfully underwent living donor liver transplant, whereas the remaining 3 children were asymptomatic at the follow-up. CONCLUSIONS: TIPS creation was found to be safe and efficacious in improving portal hypertension and growth in these children, although, with a higher rate of reinterventions, possibly due to the use of small, bare metal stents.

Recanalization of portal axis after cavoportal hemitransposition in a liver transplant recipient with complete splanchnic thrombosis.

BACKGROUND: Diffuse splanchnic thrombosis may render standard LTx difficult or even technically impossible. A 19-year-old woman with acute-on-chronic Budd-Chiari syndrome and complete splanchnic thrombosis underwent conventional LTx. Only limited anatomical portal inflow could be restored, and urgent re-transplantation for recurrent splanchnic vein thrombosis became necessary. METHODS: At re-transplant, and in addition to the reestablishment of some portal inflow through the preserved original porto (native)-portal (graft) connection, a cavoportal shunt was created (first partial via 30% tapering of the vena cava, but eventually complete by total occlusion of the vena cava). RESULTS: The postoperative course was then uneventful, and interestingly, the native portomesenteric axis gradually reopened. Two years post-transplant, the liver graft is perfused via both physiological and non-physiological sources. Liver function is normal. There is no IVC syndrome and no residual PHT. She is leading a normal life. CONCLUSION: Creation of CPHT, in addition to the preservation of portal inflow from the native splanchnic system, should be considered in patients with diffuse splanchnic thrombosis, when sufficient physiological portal inflow cannot be restored at the time of LTx, but in whom the splanchnic circulation may reopen up later.

Preoperative successful thrombectomy and thrombolysis of acute extensive splanchnic venous system and TIPSS thrombosis in a child with Budd-Chiari syndrome-Creating a window to enable living donor liver transplantation.

Preoperative extensive PV thrombosis can pose a technical challenge during liver transplantation surgery. Several strategies adopted to mitigate this problem include creation of a superior mesenteric vein-PV jump graft, use of a polytetrafluoroethylene graft, renoportal anastomosis, or cavoportal hemitransposition. Extensive and diffuse thrombosis of the splanchnic venous system may even necessitate multivisceral transplantation. We describe the case of a pediatric patient with Budd-Chiari syndrome and decompensated cirrhosis, who developed extensive thrombosis of the porto-spleno-mesenteric venous system prior to liver transplantation. We used a combination technique of thrombus aspiration by a novel trans-TIPPS approach followed by thrombolysis. Complete preoperative resolution of the extensive thrombosis was achieved. This allowed the creation of a brief window to enable planned LDLT. In prudently selected patients, performing an early mechanical and chemical thrombolysis of an extensive acute splanchnic venous thrombosis can thus help expedite a planned LDLT.

Open Cardiac Surgery in a Patient With Chronic Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) is a rare congestive hepatopathy arising from hepatic venous outflow obstruction. The clinical presentation of BCS varies depending on the presence of collateral veins. The authors report a rare case of infective endocarditis and chronic primary BCS in a 50-year-old man who underwent open cardiac surgery. Due to the presence of dilated collateral veins flowing directly into the inferior vena cava, cardiopulmonary bypass was established by arterial cannulation of the ascending aorta, with venous cannulation of the upper portion of the superior vena cava, as well as the dilated collateral vein. Mitral valve replacement and tricuspid valvuloplasty were performed uneventfully, and the patient then was admitted to the intensive care unit. Patients with primary BCS need to be evaluated rigorously preoperatively and intraoperatively for collateral flow to establish cardiopulmonary bypass.

The outcomes of interventional treatment for Budd-Chiari Syndrome complicated by inferior vena cava thrombosis: Systematic review and meta-analysis.

PURPOSE: The aim of this study was to perform a systematic review and meta-analysis to assess the safety and efficacy of interventional treatment for Budd-Chiari syndrome (BCS) complicated by Inferior Vena Cava thrombosis (IVCT) patients. METHODS: We evaluated the published studies on interventional treatment for BCS complicated by IVCT. Meta-analysis was used to calculate the combined effect size and their 95% confidence intervals (CI) based on random effect. The publication bias was assessed by Begg's test. RESULTS: Sixteen studies on interventional treatment for BCS complicated by IVCT patient were selected for meta-analysis, a total of 767 BCS complicated by IVCT patients were included. The combined effect size (95% CI) were 99% (98-100%) for the total successful rate of IVC recanalization, 15% (11-21%) for the rate of IVC restenosis after initial operation, 92.0% (86-97%) for the rate of clinical improvement, 76% (68-84%) for the rate of thrombus clearance and 0.00% (0-1%) for the incidence of pulmonary embolism (PE). Through subgroup meta-analysis about the rate of thrombus clearance, we got the pooled results (95% CI) of individualized treatment strategy (ITS) group and non-individualized treatment strategy (non-ITS) group, were 81% (71-92%) and 73% (63-83%), respectively. CONCLUSIONS: The interventional treatment for BCS complicated by IVCT patients is safe and effective with low incidence of PE, high thrombus clearance rate, high technically successful rate, good patency, and high clinical improvement rate. Moreover, subgroup analysis indicated that management based on the type and extent of the thrombus is proposed.

Orthotopic Liver Transplantation for Budd-Chiari Syndrome: Observations from a 30-Year Liver Transplant Program.

Background and objectives Budd-Chiari syndrome (BCS) refers to a complete thrombotic obstruction of the venous hepatic outflow tract due to various etiologies and constitutes a rare indication for ortothopic liver transplantation (LT). Few studies investigated long-term outcomes after LT for BCS. The aim of this study was to examine potential risk factors for late mortality and to evaluate long-term outcomes after LT for BCS. Materials and methods: 46 patients received an LT for BCS between 1989 and 2019 at the transplant center of the Charité-Universitätsmedizin Berlin. We analyzed potential effects of disease etiology, vascular events, rejection, and immunosuppression on long-term survival after transplantation using Kaplan-Meier curves and Cox logistic regression. Results: Of the 46 patients, 70% were female and 30% were male. Median age at the time of transplantation was 36 years. A total of 41 vascular events, including 26 thrombotic and 17 hemorrhagic incidents, occurred. The 1 year, the 5 year, the 10 year, and the 20 year survival rates were 87%, 83%, 76%, and 60%, respectively. By comparison, survival rates of the liver transplant cohort across all other indications at our center were slightly inferior with 85%, 75%, 65%, and 46%, respectively. In the study population, patients with myeloproliferative disorders showed worse outcomes compared to patients with other causes of BCS. Conclusion: Liver transplantation for BCS showed excellent results, even superior to those for other indications. Vascular events (i.e., thrombotic or hemorrhagic complications) did not have any prognostic value for overall mortality. Patients with myeloproliferative disorders seem to have a disadvantage in survival.

[Analysis of curative efficacy of transjugular intrahepatic portosystemic shunt using viabahn stent in the treatment of Budd-Chiari syndrome with extensive hepatic vein occlusion].

Objective: To investigate the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) using viabahn stent in patients with Budd-Chiari syndrome (BCS) with extensive hepatic vein occlusion. Methods: Technical success rates, portal pressure gradient (PPG), complications and shunt patency rate of 25 cases with BCS with extensive hepatic vein occlusion treated by TIPS using viabahn stent were retrospectively analyzed. Color Doppler ultrasound was used for postoperative follow-up. Patency rates were evaluated by Kaplan-Meier curve. Results: The technical success rate of TIPS was 100%. Mean PPG decreased from (22.7 ± 6.2) mmHg preoperatively to (9.7 ± 2.6) mmHg postoperatively (t = 9.58, P < 0.05). Hepatic encephalopathy incidence was 8.0% (2/25), and there were no complications such as intra-abdominal hemorrhage. Twenty-five cases were followed up for 1 to 39 ( mean 19.0 ± 11.9) months, and one patient died of liver failure three months after interventional treatment. The primary patency rates at 1, 2, and 3 years after interventional treatment were 91.3%, 91.3%, and 78.2%, respectively, while the repatency rates were 100%, 92.9%, and 92.9%, respectively. Conclusion: TIPS using viabahn stent can bring better short-term and mid-term curative efficacy in treatment of BCS with extensive hepatic vein occlusion.