Diagnosis Of Budd-Chiari Syndrome Research Articles

A Clinical Case of Timely Diagnosis and Successful Treatment of Budd-Chiari Syndrome With Fulminant Cytolysis in the Setting of a First-time Diagnosed Myeloproliferative Disease.

The article presents a clinical case of peculiarities of clinical manifestations, diagnostic and therapeutic approaches of undiagnosed chronic myeloproliferative disease, on the background of which Budd-Chiari syndrome (BCS) developed. The results of clinical course, examination, and treatment of a patient with BCS as a manifestation of the hidden course of primary myelofibrosis with the presence of somatic mutation (V617F) in Janus-tyrosine kinase-2 (JAK2) gene in myeloid cells are presented. Standard clinical and laboratory examinations, and cytomorphologic and histologic examination of bone marrow were used. The diagnosis of BCS was confirmed by ultrasound (US) Doppler examination of the portal system vessels. Symptomatic therapy resulted in insignificant positive results. The analysis of this clinical case showed that the development of BCS was due to a chronic myeloproliferative disease that was not diagnosed before the development of thrombosis. Hepatic vein thrombosis was accompanied by the development of fulminant cytolytic syndrome. Along with symptomatic therapy, patient K., female, 32 years old, underwent transjugular intrahepatic portosystemic shunting 1 month after the first symptoms of BCS appeared, which contributed to a significant clinical effect. Seven years after the installation of 4 transjugular intrahepatic portosystemic shunts, the patient's condition remains satisfactory. The uniqueness of this clinical case lies in the presence of 2 serious diseases at the same time: myeloproliferative pathology (primary myelofibrosis) JAK2-positive variant and BCS. Timely diagnosis of both hematological diseases and their complication in the form of hepatic vein thrombosis with fulminant cytolytic syndrome allowed timely prescription of adequate treatment with a good clinical response.

Budd-chiari syndrome: case report

Budd-Chiari Syndrome (BCS) is a rare pathological condition characterized by an obstruction of the hepatic venous outflow. It can be classified as primary, due to an intra-luminal venous lesion, or secondary, resulting from extrinsic compression or invasion of the venous system. This study aims to report a case of Budd-Chiari Syndrome, emphasizing the importance of diagnosis and treatment in preventing progression to chronic liver disease. We report the case of a 47-year-old patient who presented to the emergency department with severe pain in the right hypochondriac region and abdominal distension. A total abdominal CT scan was performed and revealed thrombosis of the suprahepatic veins, confirming the diagnosis of BCS. The patient developed portal hypertension and signs of chronic liver disease. Treatment was administered with Spironolactone and Marevan. After six months of Marevan use, the patient experienced worsening of the esophageal varices and hematemesis, requiring esophageal variceal band ligation. Liver transplantation was not recommended as the patient's MELD score was 14. Therefore, an accurate diagnosis of BCS is crucial for appropriate therapeutic intervention in order to prevent the progression to chronic liver disease, with permanent damage to liver functionality and the patient’s quality of life.

Fibrosis Progression in Patients with Budd–Chiari Syndrome and Transjugular Intrahepatic Portosystemic Shunt (TIPS): A Long-Term Study Using Transient Elastography

Hepatic vein outflow obstruction causes congestion of the liver, leading to necrosis, fibrosis, and portal hypertension (PH). A transjugular intrahepatic portosystemic shunt (TIPS) reduces congestion and PH by providing artificial outflow. The aim of the study was to investigate fibrosis progression in patients with Budd–Chiari syndrome (BCS) and TIPS using transient elastography (TE). From 2010 to 2022, 25 patients received 80 TEs using FibroScan®, Echosens, Paris, France (3.2 ± 2.1 per patient). TIPS function was assessed via Doppler ultrasound or radiological intervention. At the time of TE examination, 21 patients had patent shunts. Four patients had occluded shunts but normal pressure gradients during the intervention. The first TE measurement performed 9.8 ± 6.8 years after the BCS diagnosis showed stiffness values of 24.6 ± 11.5 kPa. A second or last measurement performed 7.0 ± 2.9 years after the first measurement showed similar stiffness values of 24.1 ± 15.7 kPa (p = 0.943). Except for three patients, the liver stiffness was always >12 kPa, indicating advanced fibrosis. Stiffness values obtained <5 years (n = 8, 23.8 ± 9.2 kPa) or >5 years after the BCS diagnosis (24.9 ± 12.7 kPa) did not differ (p = 0.907). In addition, stiffness was not related to the interval between BCS and TIPS implantation (p = 0.999). One patient received liver transplantation, and two patients died from non-hepatic causes. Most patients developed mild to moderate cirrhosis, possibly during the early phase of the disease. Timing of TIPS did not influence fibrosis progression. This and the release of portal hypertension may argue in favor of a generous TIPS implantation practice in patients with BCS.

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.

Budd-Chiari Syndrome Imaging Diagnosis: State of the Art and Future Perspectives.

Budd-Chiari syndrome (BCS) is a rare hepatic vascular disorder defined by the presence of partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Several conditions can lead to BCS, from hypercoagulable states to malignancies. Primary BCS is the most common subtype, and usually bartends hypercoagulability states, while secondary BCS involves tumor invasion or extrinsic compression. A combination of clinical and imaging features leads to the diagnosis of BCS, including (1) direct signs: occlusion or compression of the hepatic veins and/or inferior vena cava, and the presence of venous collaterals; (2) indirect signs: morphological hepatic changes with caudate lobe enlargement; inhomogeneous enhancement, and hypervascular nodules. From a clinicopathological point of view, two forms of BCS can be distinguished: acute and subacute/chronic BCS, although asymptomatic and fulminant forms are also possible. Acute presentations are rare, and symptoms include hepatomegaly, ascites, and hepatic insufficiency. Subacute/chronic forms are the most common presentation, with dysmorphic liver and variable degrees of fibrosis deposition. Patients with chronic BCS can develop benign regenerative nodules (large regenerative nodules or FNH [Focal Nodular Hyperplasia]-like lesions), but are also at a higher risk of hepatocellular carcinoma (HCC). The radiologist role is therefore fundamental in both diagnosis and surveillance of BCS. The aim of this review is to present all clinical and imaging signs that can help to reach the diagnosis of BCS, with their clinical significance, providing tips and tricks for the cross-sectional diagnosis of this condition.

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.

S2943 Budd-Chiari Syndrome Diagnosis Achieved With High Clinical Suspicion Raised by EUS-Guided Portal Pressures and Liver Biopsy

Introduction: Budd Chiari syndrome (BCS) is a rare syndrome due to hepatic venous obstruction in the absence of cardiac cause, mostly secondary to thrombosis. As high as 80%of patients present with ascites. The diagnosis should be suspected in patient with acute or chronic liver disease without identified cause. Venous obstruction can be seen on Doppler ultrasound, CT, or MRI of the hepatic veins and inferior vena cava. In some cases, hepatic venogram is needed for its diagnostic and therapeutic advantages. We report a 50-year-old woman with BCS who underwent successful wire recanalization, angioplasty and thrombolysis of the right hepatic vein. Case Description/Methods: 50-year-old woman with no significant past medical history presented with abdominal distension and bilateral lower extremity swelling of two weeks duration. On exam, abdomen was distended with positive signs of fluid collection with +3 bilateral lower extremity edema. Labs revealed total bilirubin 3.3 direct bilirubin 1.4, ALP 477, ALT 38, AST 96, PT 14.4, INR 1.35. Ultrasound showed Heterogeneous appearance of liver and large volume ascites. Doppler study showed normal hepatic and portal venous flow. Abdominal MRI showed hepatic steatosis and caudate lobe hypertrophy. Large volume paracentesis was done with analysis showing SAAG of 3.1, WBC 364, Neutrophils 8. EGD revealed large esophageal varices. Viral hepatitis panel, ceruloplasmin, alpha-1antitripsin, ASMA, AMA antibodies were negative. EUS guided liver biopsy and portal pressure measurement was done showing venous pressure gradient of 13 consistent with portal hypertension. Liver biopsy showed grade 2 fibrosis and zone 3 congestion. Hepatic venogram showed chronically occluded right hepatic vein on which successful wire recanalization was done. Pt was worked up for hypercoagulable state and was found to have JAK 2 mutation and Protein C deficiency. Apixaban, Beta blocker and diuretics were started. Patient showed significant improvement with ascites and MELD score. Discussion: First line investigation for diagnosis of BCS is doppler ultrasound. Doppler US and MRI venography didn’t reveal the hepatic venous thrombosis in our patient. However, high index of suspicion, finding of elevated IVC pressure on EUS, and caudate lobe hypertrophy found on imaging as well as congestion liver biopsy findings led us to further investigate with hepatic venogram which confirmed the diagnosis and provided therapeutic advantages. Table 1. - Prothrombotic risk factors for BCS ACG Clinical Guideline A. Acquired thrombophilia B. Inherited thrombophilia C. Systemic factors D. Hormonal factors Myeloproliferative disorderPolycythemia veraEssential thrombocytosisIdiopathic myelofibrosisJAK 2 mutationPNH Hyperhomocysteinemia Factor V LeidenProthrombin gene G20210A mutationMTHFR C677T mutationThalassemiaPC deficiencyProtein C deficiencyAntithrombin deficiency SarcoidosisVasculitisBehcet’s diseaseConnective tissue diseaseInflammatory bowel diseases OCP usePregnancy Disorders of the Hepatic and Mesenteric Circulation Simonetto, Douglas A.; Singal, Ashwani K.; Garcia-Tsao, Guadalupe; Caldwell, Stephen H.; Ahn, Joseph; Kamath, Patrick S. Official journal of the American College of Gastroenterology | ACG115(1):18-40, January 2020. doi: 10.14309/ajg.0000000000000486

Long-Term Improvement in Liver Function Following Transjugular Intrahepatic Portosystemic Shunt in Patients With Budd–Chiari Syndrome

Transjugular intrahepatic portosystemic shunt (TIPS) relieves hepatic venous obstruction in Budd-Chiari syndrome (BCS), but the effect on liver function is unclear, particularly outside the immediate post-treatment period. This study aims to evaluate the long-term impact of TIPS on liver function and outcomes in BCS patients. Twenty patients with BCS who underwent TIPS from 1999 to 2018 were included. Demographic data and clinical data at the time of TIPS procedure, 6 months, 12 months, 2 years, 5 years, and 10 years post-TIPS were collected. There were 13 (13/20, 65%) women and 7 (7/20, 35%) men with a mean age at the time of TIPS of 42.6±12.8 years. The median time from BCS diagnosis to TIPS was 41 (IQR: 4-165) days. The number of patients with severe ascites decreased significantly from 10/17 (58.8%) at the time of TIPS, to 1/16 (7.7%), 1/13 (7.7%), 2/16 (12.5%), 1/14 (7.1%), and 0/8 (0%) at 6 months, 12 months, 2 years, 5 years and 10 years post-TIPS, respectively.4/20 (20%) patients developed new hepatic encephalopathy post-TIPS procedure. Child-Pugh score significantly decreased from a score of 9.4±1.8 pre-TIPS to 7.6±1.8at 6 months, 7.4±1.5at 12 months, 7.3±1.6at 2 years, 6.8±1.5at 5 years, and 6.4±0.7at 10 years post-TIPS. Fifteen (15/20, 75%) patients required TIPS revision including 4 (4/15, 26.7%) within 30 days, 2 (2/15, 13.3% within 1 month to 1 year, and 9 (9/15, 60%) at more than 1 year. Eight (8/20, 40%) patients underwent liver transplantation (LT) at median time of 7.3 (IQR 3.2-12.9) years after TIPS. TIPS placement for BCS results in sustained resolution of symptoms and improved liver function. Despite the frequent need for revisions, the long-term durability of TIPS can forgo the need for LT in the majority of patients.

Hepatic Venous Stenting in a case of Budd-Chiari Syndrome: A Case report

Budd-Chiari syndrome (BCS) results from hepatic venous outflow obstruction at the level of the hepatic veins or inferior vena cava.There is increasing number of successful reports of BCS therapy have involved endovascular techniques, including angioplasty and stent placement. Case Presentation : 22 Years old female patient presented to IR OPD with complaints of chronic abdominal pain and distension and decreased energy. The patient had no history of jaundice, bruising, or acholic stools. ChildPugh Score was 6 point and Child Class A. On Ultrasonography and CT scan, diagnosis of Budd chiari syndrome due to narrowing of IVC in its hepatic portion with occlusion of hepatic veins ostia was kept.Endovascular management was done. Conclusion : BCS denotes a heterogeneous group of diseases characterized by hepatic venous outflow obstruction at the level of hepatic veins or inferior vena cava resulting in portal hypertension.CT and MR angiography provide noninvasive anatomical definition of the hepatic artery that may be important for operative or interventional planning.

Three-fold Increased Risk of Death in Budd-Chiari Syndrome Compared to Matched Controls: A Population-based Cohort Study

Patients with Budd-Chiari syndrome (BCS) have an elevated risk of overall and liver-specific mortality, but this has not been quantified on a population level nor compared against a matched general population cohort. We identified all patients in Sweden with a recorded diagnosis of BCS in the Swedish National Patient Register between 1987 and 2016. Patients with BCS were matched for age, sex, and municipality at baseline with up to 10 reference individuals from the general population. Data on cause-specific mortality were obtained from the Causes of Death Register. A Cox regression model was performed to investigate rates of all-cause and cause-specific mortality. A total of 478 patients with BCS were matched with 4603 reference individuals. Of the patients with BCS, 43% were men, the median age was 58 years, 39% had a recorded diagnosis of a precipitating risk factor, and 13% had underlying liver disease. During a follow-up of up to 29years, 243 (51%) of the patients with BCS died compared with 1346 (29%) of the reference individuals. Overall mortality was 70 per 1000 person-years in patients with BCS compared with 28 per 1000 person-years in reference individuals, translating into an adjusted hazard ratio (aHR) of 3.1 (95% confidence interval [CI], 2.6-3.6). Although liver-related mortality was particularly high (aHR, 47.6; 95% CI, 16.5-137.4), liver disease accounted for only 10% of deaths in BCS. The most common cause of death was cardiovascular disease (aHR, 2.2; 95% CI, 1.7-2.9). Patients with BCS in Sweden had a 3-fold higher risk of death compared with general population reference individuals. Although mortality from liver diseases was high in relative terms, most patients died from cardiovascular causes.

Single-center study: evaluation of sonography in Budd-Chiari syndrome.

Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction. The study aimed to evaluate the diagnostic utility of ultrasound in confirming the diagnosis of BCS and to provide an overview of the clinical picture. In this retrospective single-center study, patients with an initial diagnosis of BCS were included. The files were analyzed concerning the ultrasound images and compared to computed tomography (CT) and magnetic resonance imaging (MRI). Main clinical signs of BCS were collected. Data of 25 patients were analyzed. Doppler sonography showed the highest sensitivity (78.9%) with the highest specificity 97.4 (%) in confirming the correct diagnosis of BCS. Main imaging signs were obstruction in the hepatic veins (68.0%, 17/25 thrombotic), collaterals (91.7%, 11/12 intrahepatic), inhomogeneous liver parenchyma (7/21), and a hypertrophied lobus caudatus (18/21) (p < 0.01). All imaging signs could be detected with sonography. Hypertrophied lobus caudatus was seen exclusively in BCS. Furthermore, portal hypertension (9/25), liver cirrhosis (9/25), and ascites (19/25) can be diagnosed as non-specific signs of BCS (p < 0.01).The main clinical findings were elevated γ-GT levels in the laboratory (92.0%, 23/25, p < 0.01) and esophageal varices in endoscopy (12/25 p < 0.01). An association with myeloproliferative neoplasia (MPN) was frequently seen (10/25) (p < 0.01). The present study demonstrates that sonography is an appropriate tool for the diagnosis of BCS and should be used as the first imaging procedure.

BUDD-CHIARI SYNDROME IN PATIENTS WITH ULCERATIVE COLITIS: A SYSTEMATIC LITERATURE REVIEW

Objective Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction that can lead to hepatic congestion, portal hypertension, ascites, esophageal varices, and liver failure. This syndrome is deeply associated with acquired or inherited hypercoagulation states; such as myeloproliferative disorders, factor V Leiden, deficiency of protein C, pregnancy, and systemic inflammation. Ulcerative colitis (UC) is an inflammatory bowel disease that leads to chronic systemic inflammation. Therefore, the aim of this study was to conduct a systematic literature review to analyze the association between Budd-Chiari syndrome and ulcerative colitis. Materials and methods A systematic literature review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Papers were selected searching PubMed/Medline, SciELO, and LILACS databases in August 2021 using the search terms [Budd-Chiari syndrome] AND [ulcerative colitis]. The inclusion criterion was limited to observational studies, case reports, or case series that reported an association between Budd-Chiari syndrome and ulcerative colitis. Language was restricted to English, Spanish or Portuguese. Results Among the 33 papers initially identified, 17 were eligible for this review after full texts were read. The selected studies were all case reports or case series. Budd-Chiari syndrome has been described as a rare extraintestinal complication of ulcerative colitis. Regarding gender, 12 patients were female and 5 were male. Seven articles reported cases of BCS in pediatric patients with UC. Three articles reported the diagnosis of BCS before the clinical manifestations and diagnosis of UC. Two cases also described the occurrence of severe thrombotic storm (development of multiple thrombi in different sites in quick succession) in patients with UC and no other coagulopathy. Discussion The studies suggest that patients with ulcerative colitis have a higher risk of Budd-Chiari syndrome and thromboembolic events. This risk can be eight times higher during a flare-up, which can be explained by the increased systemic levels of cytokines (e.g. IL-1, 1L-6, and TNF-alpha) that can modulate the coagulation cascade leading to a hypercoagulation state. In addition, the increased intestinal epithelial permeability can promote bacterial translocation and systemic endotoxemia, which further accelerates the coagulation cascade activation. Still, studies have shown that patients with UC have higher rates of thrombocytosis, fibrinogen levels, and low antithrombin levels. Conclusion Budd-Chiari syndrome can be a rare extraintestinal complication of ulcerative colitis in pediatric and adult patients. This complication can occur even before the diagnosis of inflammatory bowel disease.

Abstract No. 28 Sustained improvement in hepatic function following transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome

While transjugular intrahepatic portosystemic shunt (TIPS) has been shown to relieve hepatic venous obstruction and is a viable alternative to liver transplantation (LT) in Budd-Chiari syndrome (BCS), the effect of TIPS on liver function in patients with BCS is unclear, particularly outside the immediate post-treatment period. 20 patients with BCS who underwent TIPS from 1999-2018 were included. Demographic data and clinical data at the time of TIPS procedure, 6-month, and 12-month post TIPS were collected. There were 13 (65%) women and 7 (35%) men with a mean age of 42.6 ± 12.8 years. 8 (40%) patients had a JAK-2 mutation. Time from BCS diagnosis to TIPS procedure was 10.3 ± 23.3 months. Median follow-up time was 8.4 years. The ascitic burden significantly decreased from pre-TIPS (moderate ascites 10/17 (58.8%)) at 6 (moderate ascites 1/16 (6.3%), P = 0.0001) and 12 (1/13 (7.7%, P = 0.0007)) months follow-up, respectively. Hepatic encephalopathy was present in 5/17 (29.4%) pre-TIPS, 3/16 (18.8%) 6-month post TIPS, and 4/13 (30.8%) 12-months post-TIPS. Serum albumin significantly improved from pre-TIPS (3.0 ± 0.6) through 6 (3.5 ± 0.7 (P = 0.03)) and 12 (3.7 ± 0.6 (P< 0.01)) month follow up. Similarly, total protein significantly improved at 6-months after TIPS (pre-TIPS 6.4 ± 1.4, 6-month 7.3 ± 1 (P = 0.03)). Child-Pugh Score significantly decreased with a score of 9.4 ± 1.8 pre-TIPS, as compared to 7.6 ± 1.8 at 6-months (P = 0.007), and 7.4 ± 1.5 (P = 0.05) at 12-months post TIPS. 15 (75%) patients required TIPS revision with 4/15 (26.7%) within 30 days; 2/15 (13.3%) between 1 month and 1 year, and 9/15 (69.2%) after 1 year. 8 (40%) patients underwent LT at mean 7.68 ± 6 years after TIPS. 3 (15%) patients died at 1.9, 9.8, and 16.9 years following TIPS. In patients with BCS, TIPS significantly resolves symptoms and improves liver synthetic function at 1-year. Despite the frequent need for revision, TIPS for BCS also has good long-term durability and is able to prolong time to LT in a large proportion of patients.

A Scoring Model to Predict In-Hospital Mortality in Patients With Budd-Chiari Syndrome.

A model that can predict short-term mortality in patients with the Budd-Chiari syndrome (BCS) with a high degree of accuracy is currently lacking. The primary objective of our study was to develop an easy-to-use in-hospital mortality prediction model in patients with BCS using easily available clinical variables. Data were extracted from the National Inpatient Sample to identify all adult patients with a listed diagnosis of BCS from 2008 to 2017 using ICD-9 or ICD-10 codes. After identifying independent risk factors of in-hospital mortality, we developed a prediction model using logistic regression analysis. The model was built and validated in a training and a validation data set, respectively. Using the model, we risk stratified patients into low-, intermediate-, and high-risk groups. Between 2008 and 2017, we identified a total of 5,306 (weighted sample size 26,110) discharge diagnosis of patients with BCS, with an overall in-hospital mortality of 7.14%. The independent risk factors that predicted mortality were age of 50 years or older, ascites, sepsis, acute respiratory failure, acute liver failure, hepatorenal syndrome, and cancers. The mortality prediction model that incorporated these risk factors had an area under the receiver operating characteristic curve of 0.87 (95% CI 0.85-0.95) for the training data and 0.89 (95% CI 0.86-0.92) for the validation data. Patients with low-, intermediate-, and high-risk scores had a predicted in-patient mortality of 4%, 30%, and 66%, respectively. Using a national administrative database, we developed a reliable in-patient mortality prediction model with an excellent accuracy. The model was able to risk stratify patients into low-, intermediate-, and high-risk groups.

Evaluating the Short-Term Clinical Efficacy of Magnetic Resonance Elastography in Patients with Budd-Chiari Syndrome

To investigate the clinical relevance of liver stiffness (LS) in evaluating liver function properties in patients with Budd-Chiari syndrome (BCS) with different severities and LS variation before and after endovascular intervention. Between December 2016 and March 2019, patients with a diagnosis of BCS were considered for enrollment consecutively in our study. Liver function of these patients was classified according to Child-Pugh grading standard before treatment. Liver function parameters were recorded, including albumin, alanine aminotransferase, aspartate aminotransferase, prothrombin time, and total bilirubin. LS was measured with MR elastography (MRE) before and after treatment. Pearson correlation analysis was performed to measure the correlation between LS and liver function-related parameters. Univariate analysis of variance test was used to compare LS and clinical quantitative variables of patients in three different Child-Pugh categories. Paired t test with a significant threshold of p = 0.05 was used to compare LS and pressure gradient of these patients before and after treatment. Correlation analysis between changes in LS and that in pressure gradient was performed by linear regression. A total of 43 patients (23 males and 20 females) were finally enrolled in this study. The mean LS in the three groups was 5.67 ± 1.15 kPa (Child-Pugh A), 6.31 ± 1.13 kPa (Child-Pugh B), and 8.27 ± 2.22 kPa (Child-Pugh C), respectively. LS showed significant difference for patients with different Child-Pugh grades (F = 9.536, p < 0.001). Prothrombin time and total bilirubin were positively correlated with LS (p < 0.05). After treatment, mean LS in three groups was 4.83 ± 1.06 kPa, 5.12 ± 0.93, and 7.37 ± 1.96, respectively. LS decreased significantly in all three Child-Pugh grades (p < 0.001 from Child-Pugh A, p < 0.001 from Child-Pugh B, p = 0.009 from Child-Pugh C). The mean pressure gradient before intervention was 17.6 ± 4.9 mm Hg, and 8.7 ± 7.2 mm Hg after the treatment (p < 0.001). The changes in LS were correlated to that in pressure gradients (r = 0.439; r2 = 0.193; p = 0.015). MR elastography for LS measurement has been demonstrated to act as an effective tool to evaluate liver function, and to monitor the BCS patients in follow-up treatments.

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 (> 18years 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 5years 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.

Endovascular treatment of an obstructive membrane between inferior vena cava and right atrium in an unrecognized Budd-Chiari syndrome

BackgroundBudd-Chiari syndrome is defined as a hepatic venous outflow track obstruction of various etiology, which appears at different levels. The inferior vena cava outflow membrane is an unusual, but a potentially treatable cause. The percutaneous treatment has emerged as a very promising management mode for such patients. Follow-up results are favorable for balloon angioplasty and/or stenting, with minimal re-stenosis rates.Case presentationWe report a case of a young woman, earlier operated on congenital heart defect and with previous pulmonary embolic incident after childbirth, with no evidence of thrombophilia. She was admitted to our institution for a suspected right atrial tumor. After the diagnosis of Budd-Chiari syndrome caused by membranous inferior vena cava obstruction, a percutaneous treatment of a thick membrane was successfully performed, using an unusual technique.ConclusionBalloon angioplasty should be considered in cases of membranous obstruction of vena cava, where a focal obstruction is causing the symptoms. In our patient, the anatomy was not suitable for stenting, and balloon dilatation was successful just after the membrane was pulled apart with a big balloon in a “Rashkind-like” procedure.

S1038 The Clinical Characteristics Associated With Poor Prognosis in Hospitalized Patients With Budd-Chiari Syndrome: A Nationwide Analysis

INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare thrombotic complication of the hepatic vein that results in rapid ascites accumulation, hepatomegaly, and death. Given the seriousness of this complication, the risk factors associated with poor prognosis in patients with BCS need to be identified in order to better risk-stratify this population. METHODS: This was a nationwide analysis that used ICD 9/10 codes to select hospital cases with BCS diagnosis from the 2011 to 2017 National Inpatient Sample. The clinical characteristics of the deceased cohort were compared to the surviving cohort using univariate and multivariate analysis in order to identify the risk factors for mortality. RESULTS: Of the 3641 cases with BCS, 260 cases resulted in death (7.14%). The deceased cohort was older in age (54.8 vs 48.6y P < 0.01), but the gender ratio (female: 50.8 vs 54.9% P = 0.22) and racial distribution (P = 0.25) were no different from those of the controls. Compared to the control cohort, the deceased cohort had increased length of stay (13.8 vs 8.64d P < 0.01), and hospitalization costs ($253,618 vs $101,115, P < 0.01). In terms of hepatic events, the deceased cohort had higher rates of SBP (10.8 vs 1.80% P < 0.01, OR 6.57 95%CI 4.12–10.48), ascites (44.2 vs 26.9% P < 0.01, OR 2.15 95%CI 1.66–2.78), hepatic encephalopathy (19.6 vs 9.58% P < 0.01, OR 2.30 95%CI 1.66–3.19), hepatorenal syndrome (16.2 vs 2.34% P < 0.01, OR 8.05 95%CI 5.41–12.0), and portal vein thrombosis (17.7 vs 10.6% P < 0.01, OR 1.82 95%CI 1.30–2.54). However, no differences were found between cohorts in the incidence of variceal bleeding (3.08 vs 2.45% P = 0.68, OR 1.26 95%CI 0.60–2.64) or in the incidence of cirrhosis (29.6 vs 27.2% P = 0.44, OR 1.13 95%CI 0.85–1.48). The deceased cohort had higher incidences of acute kidney injury (AKI) (66.5 vs 19.1% P < 0.01, OR 8.42 95%CI 6.42–11.0) and sepsis (51.5 vs 10.8% P < 0.01, OR 8.7607 95%CI 6.71–11.43). Through a multivariate model, sepsis (P < 0.01, aOR 5.14 95%CI 3.78–6.98) and AKI (P < 0.01, aOR 4.22, 95%CI 3.09–5.78) were found to be strongly associated with increased mortality. CONCLUSION: Sepsis and AKI are independent risk factors of inpatient mortality in hospitalized patients with Budd-Chiari Syndrome. Therefore, alongside the standard medical-surgical management of BCS, these patients require early recognition and treatment of sepsis and AKI with antibiotics/renal-preserving therapies.Figure 1.: Multivariate model: sepsis and acute kidney injury are independent risk factors of mortality in hospitalized patients with Budd-Chiari Syndrome.

A Nationwide Analysis of Budd–Chiari Syndrome in the United States

The Budd-Chiari Syndrome (BCS) is a rare disorder characterized by hepatic venous outflow obstruction. The primary objectives of our study were to assess temporal trends in the prevalence of BCS among hospitalized patients in the United States using the National Inpatient Sample (NIS) database and to evaluate demographics, risk factors, and common presentation of BCS. Data were extracted from the NIS to identify patients >18 years of age using all listed diagnosis of BCS from 1998 to 2017 and analyzed. Between 1998 and 2017, we identified a total of 8435 hospitalizations related to BCS. Over the 19-year period, the hospitalization rate for BCS increased consistently from 4.96 per 1,000,000 US population in 1998 to 10.44 per 1,000,000 in 2017, with an annual percentage change increase of 4.41% (95% confidence interval [CI]: 4.23%-4.59%, P < 0.0001). The most common risk factor (7.75%) was myeloproliferative disorder (essential thrombocythemia, polycythemia vera, myelofibrosis, chronic myeloid leukemia) followed (7.32%) by a hypercoagulable state (primary thrombophilia, protein C deficiency, factor V Leiden mutation, antiphospholipid antibody syndrome or prothrombin gene mutation) and paroxysmal nocturnal hemoglobinuria (1.63%). Cirrhosis was present in 18.7%, Portal vein thrombosis in 7.9%, and inferior vena cava thrombosis in 6.4%. The most common manifestations of BCS were ascites (29.9%) or acute kidney injury (18.8%) followed by hepatic encephalopathy (9.6%) and acute liver failure (5.6%). This large population-based study from the United States showed increasing hospitalizations related to BCS. Common presentation was ascites and acute kidney injury.

Contrast-Enhanced Ultrasound is a Useful Adjunct to Doppler Ultrasound in the Initial Assessment of Patients Suspected of Budd Chiari Syndrome

BackgroundDoppler is the screening modality of choice for assessment of patients suspected of Budd Chiari syndrome (BCS). The aim of this study was to compare the diagnostic value of contrast enhanced ultrasound (CEUS) with Doppler in the initial evaluation of patients with BCS. MethodsThis was a retrospective study of patients with suspicion of BCS who underwent CEUS of the hepatic veins and inferior vena cava between July 2017 and April 2019. CEUS was performed using Sonovue. All patients underwent Doppler evaluation of the hepatic veins and inferior vena cava. The final diagnosis of BCS was based on transvenous or percutaneous digital subtraction venography. The diagnostic accuracy of CEUS was compared with Doppler. ResultsA total of 19 patients (median age, 30 years; 11 males) were evaluated with CEUS and Doppler. A final diagnosis of BCS was established on digital subtraction venography in 15 patients. CEUS was found to have a 100% sensitivity and 75% specificity. The sensitivity and specificity of Doppler was 100% and 25%. The diagnostic accuracies of CEUS and Doppler were 94.74% and 84.29%, respectively. ConclusionCEUS is a useful adjunct to the Doppler in the initial assessment of patients with BCS. However, further prospective studies must confirm our preliminary observations.