Budd-Chiari Syndrome Patients Research Articles

Feasibility and long-term outcomes of hepatic vein recanalization in Budd-Chiari syndrome through intrahepatic collateral pathways.

To investigate the feasibility and long-term outcomes of hepatic vein (HV) recanalization using intrahepatic collateral pathways in patients with Budd-Chiari syndrome (BCS) with HV obstruction. Clinical data of 29 BCS patients with HV obstruction and intrahepatic collateral pathways were reviewed. All patients underwent HV recanalization through the intrahepatic collaterals. Follow-up was performed at 1, 3, 6, and 12 months after treatment and annually thereafter. Cumulative patency and survival rates were assessed using Kaplan-Meier curves. The independent predictors of patency were determined using a Cox regression model. HV recanalization was successful in 28 of the 29 patients (96.6%), with no complications. Of the 28 cases, simultaneous recanalization of the accessory HV and right HV was achieved in 11 patients, accessory HV and middle HV in six, accessory HV and left HV in three, right HV and middle HV in five, and left HV and middle HV in three. Twenty-eight patients were followed from 4 to 87 (mean, 53.6 ± 26.7) months after treatment, and six patients developed reocclusion. The overall cumulative 1-, 3-, 5-, and 7-year primary HV patency rates were 96.3, 82.9, 74.6, and 59.7%, respectively. The cumulative 1-, 3-, 5-, and 7-year survival rates were 100, 95.8, 95.8, and 86.3%, respectively. Interventional treatment of HV obstruction in BCS patients through intrahepatic collateral approaches is well tolerated and feasible and can result in excellent long-term patency and survival rates.

Prevalence of Antiphospholipid Syndrome in Primary Budd Chiari patients and their intervention outcome

Abstract Background Budd-Chiari syndrome (BCS) is a rare but potentially life threatening hepatic disorder that results from obstruction of the hepatic venous outflow tract. Obstruction can occur at any level from the hepatic venules to the right atrium. According to the etiology, BCS can be classified as primary (due to intrinsic intraluminal thrombosis or webs) or secondary (due to intraluminal invasion by a parasite or malignant tumor or extraluminal compression by an abscess, cyst or solid tumor) the clinical presentation of BCS has a wide spectrum and ranges from asymptomatic cases to fulminant hepatic failure The classic triad of abdominal pain, ascites, and hepatomegaly is nonspecific. Aim of the Work To assess the prevelance of Antiphospholipid syndrome (APS) in primary Budd Chiari syndrome (BCS) patients and their management outcome. Patients and Methods Patients presented to BCS outpatient clinic, Tropical medicine department, Ain Shams University Hospitals were enrolled in the study from 2005 to 2020. Some patients with APS have no evidence of any definable associated disease, while, in other patients, APS occurs in association with SLE or another rheumatic or autoimmune disorder. Traditionally, these have been referred to as primary or secondary APS, respectively. Results Regarding laboratory data, Lupus Anticoagulant, ACL IgG antibodies and ACL IgM antibodies were positive in 60 (83.3%), 49 (68.1%) and 27 (37.5%) patients respectively. For 42 patients (58.3%), intervention was performed. TIPSS was performed on 33 (45.8%) patients, while Angioplasty with Stent was performed on 9 (12.5%) patients. There was no intervention in 30 (41.7%) of the patients. In the studied patients, rate of mortality of the total 72 patients was 48.6%. The mean cause of death was Hepatorenal syndrome (31.4%). Conclusion BCS, although infrequent, is not a rare complication in patients with APS and may be the first clinical manifestation of this syndrome. However, no predisposing factor to BCS has been identified in patients with APS. This supports the need for systematic screening for LA and aCL in all cases of hepatic vein thrombosis. In these patients, TIPS placement may be an effective treatment for portal hypertension, but in any case, long-term high intensity anticoagulation treatment is essential in order to prevent recurrent thrombosis. Lupus anticoagulant is much more common than anticardiolipin antibodies (83.3%, 68.1% respectively) among BCS patients due to antiphospholipid syndrome.

Clinical profile and outcomes of hepatocellular carcinoma in primary Budd-Chiari syndrome.

There is scant literature on hepatocellular carcinoma (HCC) in patients with Budd-Chiari syndrome (BCS). To assess the magnitude, clinical characteristics, feasibility, and outcomes of treatment in BCS-HCC. A total of 904 BCS patients from New Delhi, India and 1140 from Mumbai, India were included. The prevalence and incidence of HCC were determined, and among patients with BCS-HCC, the viability and outcomes of interventional therapy were evaluated. In the New Delhi cohort of 35 BCS-HCC patients, 18 had HCC at index presentation (prevalence 1.99%), and 17 developed HCC over a follow-up of 4601 person-years, [incidence 0.36 (0.22-0.57) per 100 person-years]. BCS-HCC patients were older when compared to patients with BCS alone (P = 0.001) and had a higher proportion of inferior vena cava block, cirrhosis, and long-segment vascular obstruction. The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up (13029 ng/mL vs 500 ng/mL, P = 0.01). Of the 35 BCS-HCC, 26 (74.3%) underwent radiological interventions for BCS, and 22 (62.8%) patients underwent treatment for HCC [transarterial chemoembolization in 18 (81.8%), oral tyrosine kinase inhibitor in 3 (13.6%), and transarterial radioembolization in 1 (4.5%)]. The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years vs 3.1 mo (P = 0.0001). In contrast to the New Delhi cohort, the Mumbai cohort of BCS-HCC patients were predominantly males, presented with a more advanced HCC [Barcelona Clinic Liver Cancer C and D], and 2 patients underwent liver transplantation. HCC is not uncommon in patients with BCS. Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.

TEN-YEAR OUTCOMES OF TIPS FOR BUDD-CHIARI SYNDROME: SYSTEMATIC REVIEW AND META-ANALYSIS.

Budd-Chiari syndrome (BCS) results from the obstruction of the hepatic venous flow, usually at the level of the hepatic vein or inferior vena cava. When left untreated, it can progress with several complications, including liver cirrhosis. Transjugular intrahepatic portosystemic shunt (TIPS) appears to be effective in a subgroup of BCS patients. To perform a systematic review and meta-analysis of TIPS effectiveness in BCS treatment, considering the survival rate, reduction in portosystemic pressure, need for liver transplantation, technical failure, and shunt dysfunction for up to 10 years of follow-up. We evaluated 17 studies published in PubMed, Science Direct, Web of Science, and SCOPUS databases, which used TIPS as a treatment for BCS, comprising 618 subjects between 18 and 78 years old. We assessed the bias risk by the NOS, NHI, and JBI scales for cohort stu-dies, before-after studies, and case series, respectively. We conducted the meta-analyses by extracting the number of events and the total patients evaluated to perform the proportion meta-analyses using the R software ("meta" package - version 4.9-6). The pooled results (95%CI) showed a 19% (25.9-12.5%) rate of portosystemic pressure reduction, 6% (1-12%) rate for the need for liver transplants despite the use of TIPS, 2% (1-6%) technical failure rate, 30% (18-46%) shunt dysfunction rate, and 88% (81-93%) for the mean frequency of patients alive between 1 and 10 years after the procedure. We stratified survival rate and found an 86% (74-93%) prevalence of living subjects during less than five years, 92% (83-97%) at five years, and a 77% frequency (68-83%) of patients alive ten years after the TIPS placement. TIPS is an effective treatment for BCS, providing a high 10-year frequency of living patients and a significant decrease in portosystemic pressure. The need for liver transplants after TIPS and the technical failure rate is low.

Exploring the utility of EUS in Budd-Chiari syndrome: a multicenter multiobserver prospective cohort study

Background and aimsThe diagnosis of Budd-Chiari syndrome (BCS) is essentially radiological and is needed to plan appropriate therapy. We therefore conducted this proof of concept study to assess the utility of EUS in assessing anatomy of BCS. MethodsA prospective multi-centre observational study including 50 consecutive patients with a diagnosis of BCS were enrolled. All patients underwent a detailed EUS examination by three independent endosonographers, blinded to the anatomical details of BCS and others’ findings. This was compared to each other and to conventional angiography (where available) or Magnetic resonance venography (MRV). Outcomes assessed include inter observer agreement, sensitivity, specificity, positive predictive value, negative predictive value and overall accuracy in diagnosing pathological lesions of BCS. ResultsFifty BCS patients (Mean age 34 years ; range 13-65 years) underwent EUS. Results showed good agreement among endosonographers for diagnosing right hepatic vein (RHV) (kappa=0.716) and left hepatic vein (LHV) lesions (kappa=0.722), moderate agreement for middle hepatic vein (MHV) lesions (kappa=0.660) and very good agreement for inferior vena cava (IVC) lesions (kappa=0.823). EUS demonstrated high sensitivity, positive predictive value, low inter-observer variability, and overall diagnostic accuracy for BCS lesions. ConclusionsIn conclusion, EUS is a safe and accurate diagnostic tool for BCS. It can provide accurate mapping of hepatic veins, intrahepatic collaterals, and the IVC.

Factor V Leiden (FVL), Prothrombin G20210A and MTHFR C677T Mutations among Patients of Budd-Chiari Syndrome

Abstract Background Many studies ask whether screening Factor V Leiden (FVL), Prothrombin G20210A and Methyl Tetra Hydro Folate Reductase (MTHFR) C677T Mutations should be routinely performed in patients with Budd–Chiari syndrome (BCS). However, the prevalence of these mutations in such patients is substantially variable. This is the first study in clinical pathology department in Ain Shams University to evaluate the importance of this mutational screening in patients with Budd-Chiari syndrome to detect occult genetic susceptibility to thrombophilia. Aim of the Work This study aims to detect Factor V Leiden (FVL), Prothrombin G20210A and MTHFR C677T Mutations among Patients with Budd-Chiari Syndrome. Patients and Methods This study was a cross sectional study conducted on 35 newly of Budd Chiari syndrome patients who were attending tropical department at Ain Shams University Hospitals during the period from April 2020 to January 2021. Informed consent was obtained from all patients in the study. Results results of our study revealed that JAK2 mutation it was detected in 17.1% of patients (6 patients), While PGM was detected in 2.9% of our patients. Furthermore Factor V Leiden Mutation was detected in 65.7%of patients and MTHFR mutation was detected in 48.6% of patients. The present study found no significant statistical difference between patients with Factor V Leiden Mutation & patients with normal Factor V Leiden regarding CBC parameters, levels of ANA, ACL IgM, ACL IgG, PC Deficiency, PS Deficiency and AT III Deficiency. In addition, our study revealed that there was no effect of MTHFR Mutation & JAK2 Mutation on levels of ANA, ACL IgM, ACL IgG, PC Deficiency, PS Deficiency and AT III Deficiency. Also there was no effect for MTHFR Mutation & JAK2 Mutation on outcome of patients, survival and response to treatment. Conclusion BCS is associated usually with genetic thrombophilia. Most commonly Factor V Leiden Mutation in 65.7%, followed by MTHFR mutation in 48.6% of our patients. Although, only Factor V Leiden Mutation has an effect on patients Outcome, Survival and Response to treatment through its effect on CBC parameters, levels of ANA, ACL IgM, ACL IgG, PC Deficiency, PS Deficiency and AT III Deficiency. Otherwise, JAK2 Mutation have significant effect on hemopoiesis.

Extracellular vesicles-transmitted long non-coding RNA MTUS2-5 promotes proliferation and vascularization of human vascular endothelial cells in patients with Budd-Chiari syndrome.

The high rates of misdiagnosis and untreated mortality with regard to Budd-Chiari syndrome (BCS) indicated the need to screen effective biomarkers. The aim of this study was to explore the function of extracellular vesicles (EVs) in patients with BCS as well as associated mechanisms. First, differentially expressed long non-coding RNAs (lncRNAs) from EVs separated from serum between BCS and healthy controls were screened using microarray analysis. Second, the proliferation, migration and tube formation of human vascular endothelial cells (HUVECs) were detected after EVs treatment, along with vascular endothelial growth factor (VEGF) levels and inflammatory factors from the cell supernatant. Last, the overexpressed lncRNA was transfected into the cells to further explore the mechanisms involved. Extracellular vesicles of BCS patients have significantly higher levels of lncRNA MTUS2-5 than healthy controls. Apparently, treatment with EVs from BCS or the ones transfected with plasmids that overexpress lncRNA MTUS2-5 enhances proliferation, migration and angiogenesis capacity. The results were considerably better than those obtained from treatment with EVs from healthy controls or transfection with the normal control plasmid, which also elevated the level of VEGF and inflammatory factors. Furthermore, FOS and PTGS2 were potentially regulated by the lncRNA MTUS2-5 transmitted by EVs. The lncRNA MTUS2-5 in EVs plays an important role in angiogenesis in the Budd-Chiari syndrome.

Focal Liver Lesions in Budd-Chiari Syndrome: Spectrum of Imaging Findings.

Budd-Chiari syndrome (BCS) is a rare clinical entity characterized by hepatic venous outflow obstruction, resulting in liver congestion and subsequent chronic parenchymal damage. This condition often leads to the development of focal liver lesions, including benign focal nodular hyperplasia-like regenerative nodules, hepatocellular carcinoma, and perfusion-related pseudo-lesions. Computed tomography, ultrasound, and magnetic resonance are the commonly employed imaging modalities for the follow-up of BCS patients and for the detection and characterization of new-onset lesions. The accurate differentiation between benign and malignant nodules is crucial for optimal patient management and treatment planning. However, it can be challenging due to the variable and overlapping characteristics observed. This review aims to provide a comprehensive overview of the imaging features and differential diagnosis of focal liver lesions in BCS, emphasizing the key findings and discussing the challenges associated with their interpretation, with the purpose of facilitating the subsequent clinical decision-making.

Identification of potential genetic Loci and polygenic risk model for Budd-Chiari syndrome in Chinese population

Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction, posing life-threatening risks in severe cases. Reported risk factors include inherited and acquired hypercoagulable states or other predisposing factors. However, many patients have no identifiable etiology, and causes of BCS differ between the West and East. This study recruited 500 BCS patients and 696 normal individuals for whole-exome sequencing and developed a polygenic risk scoring (PRS) model using PLINK, LASSOSUM, BLUP, and BayesA methods. Risk factors for venous thromboembolism and vascular malformations were also assessed for BCS risk prediction. Ultimately, we discovered potential BCS risk mutations, such as rs1042331, and the optimal BayesA-generated PRS model presented an AUC >0.9 in the external replication cohort. This model provides particular insights into genetic risk differences between China and the West and suggests shared genetic risks among BCS, venous thromboembolism, and vascular malformations, offering different perspectives on BCS pathogenesis.

Comparison of Balloon-Occluded Thrombolysis with Catheter-Directed Thrombolysis in Patients of Budd-Chiari Syndrome with Occluded Direct Intrahepatic Portosystemic Shunt.

Objectives Direct intrahepatic portosystemic shunt (DIPS) stent placement is an effective treatment for patients with Budd-Chiari syndrome (BCS); however, thrombotic occlusion of DIPS stent remains a cause of concern. The purpose of this study is to describe a novel technique of balloon-occluded-thrombolysis (BOT) for occluded DIPS stent, and compare it with the conventional catheter-directed-thrombolysis (CDT). Methods In this retrospective study, the hospital database was searched for BCS patients who underwent DIPS revision for thrombotic stent occlusion between January 2015 and February 2021. Patients were divided into CDT group and BOT group. The groups were compared for technical success, total dose of thrombolytic agent administered, duration of hospital stay, and primary assisted stent patency rates at 1- and 6-month follow-up. Results CDT was performed in 12 patients, whereas 21 patients underwent BOT. Complete recanalization was achieved in 66.7% (8 of 12) patients of CDT group as compared to 81% (17 of 21) patients of BOT group (nonsignificant difference, p = 0.420). BOT group had a short hospital stay (1.8 ± 0.7 vs. 3.5 ± 1.0 days) and required less dose of thrombolytic agent ([2.2 ± 0.4]x10 5 IU versus [8.3 ± 2.9]x10 5 IU of urokinase) as compared to the CDT group and both differences were statistically significant ( p < 0.001). Further, 6-month patency rate was higher in BOT group as compared to CDT group ( p = 0.024). Conclusion The novel BOT technique of DIPS revision allows longer contact time of thrombolytic agent with the thrombi within the occluded stent. This helps in achieving fast recanalization of thrombosed DIPS stent with a significantly less dose of thrombolytic agent required, thus reducing the risk of systemic complications associated with thrombolytic administration.

Prevalence of Portal Vein Thrombosis in Budd-Chiari Syndrome Patients

Background and Aim: Thrombosis of the portal vein or obstruction of the hepatic venous outflow tract is the cause of portal vein thrombosis (PVT) and Budd-Chiari syndrome (BCS). Patients with PVT may experience a shorter survival rate compared to those without. The present study aimed to determine the portal vein thrombosis occurrence in Budd-Chiari syndrome patients. Patients and Methods: This cross-sectional study was conducted on 46 Budd-Chiari syndrome patients in Gastroenterology department of DHQ Teaching Hospital and Mufti Mahmood Memorial Hospital, Dera Ismail Khan from November 2021 to October 2022. Written informed consent was taken from each individual. Doppler ultrasonography (colored-pulsed) was used to confirm the PVT and BCS cases. Clinical data, laboratory findings, and radiological data were recorded. SPSS version 26 was used for data analysis. Results: The overall mean age of the BCS patients was 28.34±4.6 years. The prevalence of portal vein thrombosis in BCS patients was 12.8% (n=6). Of the total cases, the incidence of chronic and acute BCS presentation was 42 (91.3%) and 4 (8.7%) respectively. The most prevalent symptoms of BCS were abdominal pain and abdominal enlargement in 38 (82.6%) and 41 (89.1%) respectively. Hepatomegaly and ascites were most prevalent clinical signs found in 36 (78.3%) and 37 (80.4%) respectively. The incidence of esophageal varices, gastric extension, and fundal varices were 32 (69.6%), 4 (8.7%), and 2 (4.3%) respectively. About 21 (45.7%) cases had Portal hypertensive gastropathy (PHG). Conclusion: A higher incidence of PVT was observed in the present study than previously reported. Sociodemographic data and underlying etiology of BCS were not significantly different between patients with and without PVT. Those with PVT were more likely to experience hepatic tenderness, increased white blood cells, and increased direct bilirubin. PVT and other patients had similar Doppler ultrasound findings. Keywords: Portal vein thrombosis, Prevalence, Budd-Chiari syndrome

Liver Stiffness in Budd-Chiari Syndrome Patients Following Endovascular Interventions And Its Role As A Non-Invasive Predictor In Assessing The Effectiveness Of Intervention Over A Period Of 6 Months.

Liver Stiffness in Budd-Chiari Syndrome Patients Following Endovascular Interventions And Its Role As A Non-Invasive Predictor In Assessing The Effectiveness Of Intervention Over A Period Of 6 Months.

Accessory Hepatic Vein Stenting in the Management of Budd-Chiari Syndrome: A Case Report

The Budd-Chiari Syndrome (BCS) is associated with hepatic venous outflow obstruction. A 22-year-old female patient presented with abdominal pain and backache for 12 days, along with generalised weakness. Physical examination revealed pitting pedal oedema and abdominal distension. An abdominal ultrasound with Doppler Ultrasonography (USG) was performed, revealing liver parenchymal disease with gross ascites and occlusion of all three Hepatic Veins (HV), suggestive of BCS. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) of the liver, as well as a right hepatic venogram using Digital Subtraction Angiography (DSA), showed chronic total occlusion at the ostium. An Accessory Hepatic Vein (AHV) was identified, joining the Right Hepatic Vein (RHV) to the Inferior Vena Cava (IVC). The accessory RHV exhibited high-grade stenosis (approximately 90-95%) at its junction with the IVC. Recanalisation of the AHV (balloon dilatation/stent insertion) was performed. Post-procedure accessory hepatic venogram showed a patent stented segment with a significant reduction in adjacent venous collaterals. No procedure-related complications were observed. The present case highlights the importance of AHV stenting in BCS patients, as it helps maintain normal physiology, in contrast to Direct Intrahepatic Porto-systemic Shunt (DIPS), which alters normal physiology by allowing portal venous blood to mix directly into the systemic circulation, bypassing the liver parenchyma.

Budd-Chiari syndrome and its associated hepatocellular carcinoma: Clinical risk factors and potential immunotherapeutic benefit analysis.

Hepatocellular carcinoma (HCC) is a well-described complication of Budd-Chiari syndrome (BCS). However, the risk factors of BCS in developing HCC and clinical characteristics and imaging features of BCS-associated HCC is still to be determined. Data from 113 consecutive patients with primary BCS in Qilu hospital were retrospectively studied. The clinical features of 12 HCC patients associated with BCS were also analyzed. Chi-square analysis was performed to analyze the differences in clinical characteristics. The treatment regime and CT imaging features of BCS-associated HCC were also illustrated. 113 consecutive patients admitted to our hospital between January 2009 and June 2016 with a primary diagnosis of BCS were enrolled. 10.6% (12/113) was diagnosed with HCC. The BCS patients were mainly male gender with an average age of 49.2 years. Symptom duration longer than one year exhibited decreased serum ALT and AST and increased ascites ratio. BCS-associated HCC patients were presented with IVC block and stricture of the hepatic venous outflow tract. Patients with HCC were older and showed elevated serum AST and total bilirubin. Most nodules of HCC located in the right posterior lobe with heterogeneous enhancement during the arterial phase and washout during the delayed phase. The results indicate that BCS patients with IVC block and stricture of hepatic venous outflow tract seem to be associated with HCC. BCS associated HCC nodules exhibited irregular and heterogeneous enhancement in the arterial phase and washout on the delayed phase.

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.

Liver Transplantation for Budd-Chiari Syndrome in the MELD Era.

We extracted data from the United Network for Organ Sharing database on all adult (≥18 y old) waitlisted candidates and recipients of LT with BCS in the United States between 2002 and 2019. Multivariable Cox regression was used to determine predictors of mortality and hazard ratios (HRs). A total of 647 BCS patients were waitlisted between 2002 and 2019. BCS was an indication for LT in 378 (0.2%) of all adult LT recipients during the study period. Of BCS patients who received LT, approximately three-fourths (72.3%) were alive for up to 10 y. We found no significant difference in LT outcomes in BCS patients with or without TIPS. Longer length of hospital stay following LT (HR, 1.32; 95% confidence interval [CI], 1.19-1.47), Black/African American race (HR, 2.24; 95% CI, 1.38-3.64), diabetes (HR, 3.17; 95% CI, 1.62-6.21), donor risk index (HR, 1.44; 95% CI, 1.05-1.99), and lower albumin levels at the time of transplantation (HR, 0.66; 95% CI, 0.50-0.88) were negatively associated with survival after LT. Interestingly, neither the Model for End-stage Liver Disease nor prior TIPS showed a significant association with survival after LT. These findings demonstrate good comparable survival among TIPS versus no TIPS in LT recipients with BCS. The decision for TIPS versus LT should be individualized on a case-by-case basis.

S3232 JAK2 Positive Budd-Chiari Syndrome and Risk in Liver Transplant

Introduction: Patients that present with Budd-Chiari Syndrome (BCS) may have underlying myeloproliferative neoplasms (MPN). Long-term prognosis is taken into consideration when evaluating for transplant. Here we present a complex patient with BCS secondary to MPN. Case Description/Methods: A 63-year-old male with new onset cirrhosis complicated by ascites and hepatic encephalopathy was hospitalized with three days of abdominal pain, distention, and diarrhea. He denied any new medications. He had no known personal or family history of liver or autoimmune diseases. He denied alcohol use. Initial laboratory evaluation included MELD-Na 15, undetectable acetaminophen, negative viral hepatitis serologies, and paracentesis was negative for infection with SAAG of 1.9. CT showed cirrhotic morphology, splenomegaly, and ascites. Ultrasound with doppler and CT venogram showed findings consistent with Budd-Chiari Syndrome (BCS). He was started on therapeutic heparin. The patient decompensated further with high MELD-Na and renal failure ultimately requiring renal replacement therapy and pressors. He was found to have a positive JAK2 V617F mutation. With leukocytosis and JAK2 mutation, bone marrow biopsy was obtained showing MPN consistent with myelofibrosis (MF). He was listed for transplant as MF was not deemed an absolute contraindication. Unfortunately, he decompensated further with variceal bleed and expired prior to transplant. Discussion: Considerations for our patient prior to transplant included the risk of leukemic transformation in setting of immunosuppression as well as increased thrombotic risk in the peri/post-operative setting. One study evaluated the prognosis of liver transplant with BCS and MPN and effects of immunosuppression. There was no difference in survival in BCS patients with or without MPN. Progression of MPN was not noted after transplant. The major concern was, however, was his thrombotic risk. One study identified portal vein thrombosis as an independent risk factor for graft loss in transplant recipients, independent of other factors. Listing the patient for transplant was controversial for these reasons. Hydroxyurea was initiated to mitigate these risks and bridge to transplant in this otherwise healthy, young, and high functioning patient. This case highlights the rapid decline of a complex patient with BCS secondary to myeloproliferative disease and the current data available to guide decision making in the transplant evaluation process.

Thrombophilia in Budd-Chiari syndrome patient

A 27-years old female patient presented with a severe general condition of generalized edema and hypotension. Two months earlier computed tomography imaged thrombosis of the inferior vena cava and the three hepatic veins, or Budd-Chiari syndrome (BCS). Additional findings were thrombosis of the right common iliac vein and thrombosis of the renal veins bilaterally. Genetic testing proved congenital thrombophilia. Anticoagulation therapy did not affect thrombotic occlusions. In another cardiovascular center, an unsuccessful attempt for interventional treatment of the inferior vena cava with a jugular approach was made. The patient was admitted to our hospital for further evaluation and decision on treatment strategy. Laboratory and non-invasive imaging at admission rejected hepatic cirrhosis. An abdominal ultrasound scan demonstrated complete occlusion of the three hepatic veins and post hepatic portal hypertension. When thrombosis of all hepatic veins was detected transjugular intrahepatic portosystemic shunt (TIPS) was an option. An endovascular strategy for inferior vena cava was undertaken and complete revascularization was achieved with the right femoral approach as a bridge to TIPS shunt procedure, since the patient didn&amp;rsquo;t meet the criteria for liver transplantation. In diagnosing disease, the main contributors were the gastroenterologist, diagnostic imaging specialist, and hematologist, while the multidisciplinary team included also cardiologist, interventional cardiologist, and angiologist. In this case the multidisciplinary decisions played a major role in diagnosing and building an appropriate therapeutic strategy for systemic illnesses and conditions for which medical guidance does not yet have clear guidelines.

Evaluation of outcome from endovascular therapy for Budd-Chiari syndrome: a systematic review and meta-analysis

This study was performed to evaluate the outcome of endovascular intervention therapy for Budd-Chiari syndrome (BCS) and compare recanalization, transjugular intrahepatic portosystemic shunt (TIPS)/direct intrahepatic portosystemic shunt (DIPS), and combined procedure treatment. For the meta-analysis, 71 studies were identified by searching four databases. The individual studies’ samples were used to calculate a confidence interval (CI 95%), and data were pooled using a fixed-effect model and random effect model. The pooled measure and an equal-weighted average rate were calculated in all participant studies. Heterogeneity between the studies was assessed with I2, and T2 tests, and publication bias was estimated using Egger’s regression test. A total of 4,407 BCS patients had undergone an endovascular intervention procedure. The pooled results were 98.9% (95% CI 97.8‒98.9%) for a technical success operation, and 96.9% (95% CI 94.9‒98.9%) for a clinical success operation. The re-intervention rate after the initial intervention procedure was 18.9% (95% CI 14.7‒22.9%), and the survival rates at 1 and 5 years after the initial intervention procedure were 98.9% (95% CI 96.8‒98.9%) and 94.9% (95% CI 92.9‒96.9%), respectively. Patients receiving recanalization treatment (98%) had a better prognosis than those with a combined procedure (95.6%) and TIPS/DIPS treatment (94.5%). The systematic review and meta-analysis further solidify the role of endovascular intervention treatment in BCS as safe and effective. It maintains high technical and clinical success and long-term survival rates. The recanalization treatment had a better prognosis and outcome than the combined procedures and TIPS/DIPS treatment.

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

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. 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. 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). 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.