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

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. DISCUSSION: 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.

Clinical outcomes of large diameter balloon angioplasty for Budd-Chiari syndrome with inferior vena cava involvement.

OBJECTIVE: We compared the long-term outcomes of small and large diameter balloon angioplasty for Budd-Chiari syndrome (BCS) with inferior vena cava (IVC) involvement in a retrospective cohort study. METHODS: Of 119 patients with BCS and IVC involvement, 23 had undergone small diameter balloon angioplasty (diameter, 14-20 mm; group A) and 96 had undergone large diameter balloon angioplasty (diameter, 24-30 mm; group B). The patients were considered cured clinically if the IVC was patent with no symptom or signs evident. RESULTS: From January 2010 to December 2016, 119 BCS patients with IVC involvement had undergone balloon angioplasty, with angioplasty successful in all 119 patients. One patient died of pulmonary embolism after dilation in group A. Abdominal pain was the most common complication during dilation and was experienced by significantly more patients in group B (77.1%) than in group A (47.8%; P = .009). Of the 119 patients, 91 (76.5%) were considered cured, with significantly more patients in group B (82.3%) than in group A (52.2%; P = .005). The mean follow-up period was 41.3 ± 2.2 months. In group B, the 1-, 3-, and 5-year primary and secondary patency rates were 82.2% ± 4.2%, 69.8% ± 5.4%, and 54.0% ± 7.0% and 97.5% ± 1.7%, 92.5% ± 3.3%, and 90.2% ± 3.9%, respectively. The patency rates at all follow-up intervals were lower in group A than in group B. The clinical response rate in group B was also significantly greater than that in group A (96.9% vs 78.3%; P = .007). The 1-, 3-, and 5-year survival rates were 100%, 88.7% ± 7.6%, and 88.7% ± 7.6% and 96.5% ± 2.0%, 92.5% ± 3.0%, and 86.0% ± 5.4% in groups A and B, respectively. CONCLUSIONS: Large diameter balloon angioplasty is safe and effective for BCS patients with IVC involvement, yielding better patency rates and long-term outcomes compared with small diameter balloon angioplasty.

Long-Term Outcomes of Endovascular Interventions in More than 500 patients with Budd-Chiari Syndrome.

PURPOSE: To assess and compare the long-term outcomes of various endovascular interventions in patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: In this single-center retrospective study, 510 consecutive patients with BCS who had undergone a total of 618 endovascular procedures from January 2001 to December 2019 were included. Details of the type of endovascular intervention, technical success, clinical success, patency rate, complications, and survival outcomes were analyzed. RESULTS: The overall technical success rate was 96% (593 of 618 procedures; 500 in treatment-naïve patients and 93 repeat interventions for recurrent disease). Endovascular procedures included recanalization procedures (angioplasty and stent placement) in 355 patients (71%) and transjugular intrahepatic portosystemic shunt (TIPS) creation in 145 (29%). Major postprocedure complications occurred in 14 patients (2.8%). Vascular/stent restenosis occurred in 95 patients (19%), and successful repeat intervention was performed in 82 of those 95 (86.3%). An additional 11 of these 82 (13.4%) underwent a third intervention for restenosis. In the recanalization and TIPS groups, the 1- and 5-y cumulative patency rates were 87% and 74% and 95% and 68%, respectively. The 1- and 5-y survival rates were 96% and 89% and 90% and 76%, respectively. CONCLUSIONS: Endovascular interventions for BCS are feasible and safe in the majority of patients, with excellent short- and long-term patency and survival rates.

Living Donor Liver Transplant in Patients With Budd-Chiari Syndrome: A Single-Center Experience at Our University Hospital.

OBJECTIVES: Budd-Chiari syndrome is an infrequent, but potentially fatal, hepatic condition with the clinical manifestation of obstructed venous drainage. This may lead to progressive hepatic congestion, portal hypertension, and, ultimately, liver failure. If medical, interventional, and surgical approaches are not effective, liver transplant offers a rescue modality. The primary objective of this study was to report the perioperative and, above all, the vascular challenges associated with living donor liver transplant in patients with Budd-Chiari syndrome. MATERIALS AND METHODS: We retrospectively reviewed demographic and clinical characteristics of 6 patients with Budd-Chiari syndrome who underwent living donor liver transplant at our transplant center from April 2004 to July 2020. We also evaluated all data regarding perioperative course, surgical outcome, and the postoperative follow-up period. RESULTS: All patients displayed advanced liver disease with a Child-Pugh score C. The mean calculated Model for End-Stage Liver Disease score was 32. The causes of Budd-Chiari syndrome were factor V Leiden thrombophilia in 1 patient, myeloproliferative disorder in 3 patients, antiphospholipid antibody syndrome in 1 patient, and a protein C deficiency in 1 patient. The mean age of patients was 40 years. One of the 6 patients was female. All patients had living donor liver transplant from immediate kin according to Jordanian allocation rules. The mean graft-to-recipient weight ratio was 0.9, and the median follow-up period was 89 months. Cumulative 1-, 3-, and 5-year-survival rates were 84%, 67%, and 67%, respectively. CONCLUSIONS: Good survival rates are achievable with living donor liver transplant for patients with advanced Budd-Chiari syndrome, particularly by means of posterior cavoplasty for enlargement of the cava orifice. Therefore, in countries with insufficient deceased donor programs, such as Jordan, living donor liver transplant may be a lifesaving therapeutic possibility.

Prognostic model for identifying candidates for hepatectomy among patients with hepatocellular carcinoma and hepatic vein invasion.

BACKGROUND: Hepatic vein tumour thrombus (HVTT) is a major determinant of survival outcomes for patients with hepatocellular carcinoma (HCC). An Eastern Hepatobiliary Surgery Hospital (EHBH)-HVTT model was established to predict the prognosis of patients with HCC and HVTT after liver resection, in order to identify optimal candidates for liver resection. METHODS: Patients with HCC and HVTT from 15 hospitals in China were included. The EHBH-HVTT model with contour plot was developed using a non-linear model in the training cohort, and subsequently validated in internal and external cohorts. RESULTS: Of 850 patients who met the inclusion criteria, there were 292 patients who had liver resection and 198 who did not in the training cohort, and 124 and 236 in the internal and external validation cohorts respectively. Contour plots for the EHBH-HVTT model were established to predict overall survival (OS) rates of patients visually, based on tumour diameter, number of tumours and portal vein tumour thrombus. This differentiated patients into low- and high-risk groups with distinct long-term prognoses in the liver resection cohort (median OS 34·7 versus 12·0 months; P < 0·001), internal validation cohort (32·8 versus 10·4 months; P = 0·002) and external validation cohort (15·2 versus 6·5 months; P = 0·006). On subgroup analysis, the model showed the same efficacy in differentiating patients with HVTT in peripheral and major hepatic veins, the inferior vena cava, or in patients with coexisting portal vein tumour thrombus. CONCLUSION: The EHBH-HVTT model was accurate in predicting prognosis in patients with HCC and HVTT after liver resection. It identified optimal candidates for liver resection among patients with HCC and HVTT, including tumour thrombus in the inferior vena cava, or coexisting portal vein tumour thrombus.

ANTECEDENTES: La trombosis tumoral de la vena hepática (hepatic vein tumour thrombus, HVTT) es un determinante importante de los resultados de supervivencia en pacientes con carcinoma hepatocelular (hepatocellular carcinoma, HCC). Se desarrolló el modelo llamado Eastern Hepatobiliary Surgery Hospital (EHBH)-HVTT para predecir el pronóstico de los pacientes con HCC y HVTT después de la resección hepática (liver resection, LR), con el fin de identificar los candidatos óptimos para LR entre estos pacientes. MÉTODOS: Se incluyeron pacientes con HCC y HVTT de 15 hospitales en China. El modelo EHBH-HVTT con gráfico de contorno se desarrolló utilizando un modelo no lineal en la cohorte de entrenamiento, siendo posteriormente validado en cohortes internas y externas. RESULTADOS: De 850 pacientes que cumplieron con los criterios de inclusión, hubo 292 pacientes en el grupo LR y 198 pacientes en el grupo no LR en la cohorte de entrenamiento, y 124 y 236 en las cohortes de validación interna y externa. Los gráficos de contorno del modelo EHBH-HVTT se establecieron para predecir visualmente las tasas de supervivencia global (overall survival, OS) de los pacientes, en función del diámetro del tumor, número de tumores y del trombo tumoral de la vena porta (portal vein tumour thrombus, PVTT). Esto diferenciaba a los pacientes en los grupos de alto y bajo riesgo, con distinto pronóstico a largo plazo en las 3 cohortes (34,7 versus 12,0 meses, 32,8 versus 10,4 meses y 15,2 versus 6,5 meses, P < 0,001). En el análisis de subgrupos, el modelo mostró la misma eficacia en la diferenciación de pacientes con HVTT, con trombo tumoral en la vena cava inferior (inferior vena cava tumour thrombus, IVCTT) o en pacientes con PVTT coexistente. CONCLUSIÓN: El modelo EHBH-HVTT fue preciso para la predicción del pronóstico en pacientes con HCC y HVTT después de la LR. Identificó candidatos óptimos para LR en pacientes con HCC y HVTT, incluyendo IVCTT o PVTT coexistente.

Acute-on-Chronic Liver Failure in Budd-Chiari Syndrome: Profile and Predictors of Outcome.

BACKGROUND AND AIM: There is a paucity of data on the clinical presentations and outcome of Budd-Chiari syndrome (BCS) patients presenting as acute-on-chronic liver failure (BCS-ACLF). We aimed to describe the profile and outcomes of endovascular interventions in patients with BCS-ACLF. METHODS: All BCS-ACLF patients presenting between October 2007 and April 2019 satisfying the Asian Pacific Association for the Study of the Liver (APASL) definition were studied. We compared 30- , 90- and, 180-day survival among BCS-ACLF patients who underwent endovascular intervention with those who did not, and with a historical cohort of Child-C BCS patients without ACLF who underwent endovascular intervention. RESULTS: Twenty-eight (5%) of 553 BCS patients presented as ACLF as per APASL definition. The majority (60.7%) were males, and mean age was 29.6 ± 11.2 years. The most common site of the block was isolated involvement of hepatic veins-HV (68%), followed by combined inferior vena cava (IVC) and HV block (25%) and isolated IVC block (7%). The acute precipitants were stent thrombosis (17.9%), acute HV thrombosis (10.7%), acute viral hepatitis (7.1%), and antituberculosis drug with hepatitis B virus reactivation (3.6%). In 60.7% patients, no acute precipitant could be identified. The 30- , 90- , and 180-day survival in BCS-ACLF post-endovascular intervention (n = 15), BCS-ACLF without endovascular intervention (n = 13), and Child-C BCS without ACLF who underwent endovascular intervention (n = 25) were (93%, 87%, and 87%), (46%, 28%, and 0%) and (96%, 92%, and 88%), respectively (log-rank test, p value < 0.001). On multivariate Cox proportional analysis, endovascular intervention and the presence of hepatic encephalopathy were independent predictors of mortality. CONCLUSION: Budd-Chiari syndrome can present as acute-on-chronic liver failure. Endovascular intervention is associated with an improved outcome.

Single-Center Retrospective Study of Clinical and Laboratory Features That Predict Survival of Patients With Budd-Chiari Syndrome After Liver Transplant.

OBJECTIVES: Budd-Chiari syndrome is a rare but critical condition that can progress to liver failure and death. For severe cases, orthotopic liver transplant remains the only curative option. The present study aimed to identify predictive parameters to assess outcomes of liver transplant. MATERIALS AND METHODS: Medical records of 33 individuals with Budd-Chiari syndrome who received orthotopic liver transplant were retrospectively assessed. Twenty-seven eligible patients were identified and grouped by outcome (survived/deceased) after transplant for Budd-Chiari syndrome. Demographic, clinical, and serum parameters taken at the time of Budd-Chiari syndrome diagnosis were evaluated for prognostic value. RESULTS: Differences between patients who survived and those who died were found for nausea/vomiting (P < .01) and splenomegaly (P < .01), which were both more common in patients who died after transplant. In addition, patients in the deceased group exhibited significantly lower serum cholinesterase levels (P < .01) and higher alkaline phosphatase levels (P < .01). Scoring systems to assess liver status or Budd-Chiari syndrome severity (Model for End-Stage Liver Disease and Child-Pugh scores, Rotterdam score, and the transjugular intrahepatic portosystemic shunting prognostic index) did not differ between groups. CONCLUSIONS: Nausea/vomiting, splenomegaly, low serum cholinesterase, and high alkaline phosphatase were associated with adverse outcomes after orthotopic liver transplant for Budd-Chiari syndrome. These factors may be surrogate markers for a severely impaired health status at time of diagnosis and should be evaluated prospectively in larger cohorts.

Long-term outcomes of individualized treatment strategy in treatment of type I Budd-Chiari syndrome in 456 patients.

AIM: To evaluate individualized treatment strategy (ITS) and long-term outcomes of endovascular treatment of Budd-Chiari syndrome (BCS) with obstructed inferior vena cava (IVC) based on different degrees of hepatic vein (HV) involvement. METHODS: From January 2006 to June 2017, 456 consecutive patients with BCS with obstructed IVC underwent endovascular treatment with ITS. All patients received IVC recanalization. Then, 426 patients with at least one patent HV received no additional treatment. Twenty-fivepatients with membranous or segmental occlusion of HVs underwent HV recanalization and for the remaining five patients with diffuse HVs occlusion, a transjugular intrahepatic portosystemic shunt (TIPS) was performed. RESULTS: The endovascular treatment was technically successful in 455 of the 456 patients (99.8%). The complication rate was 5.0% (23/456), with major complications in 13 patients (2.8%) and minor complications in 10 patients (2.2%). Median follow-up time was 60.5 months (range, 4-120 months). The cumulative 1-, 2-, 5- and 10-year primary vessel patency rates were 93.6%, 89.9%, 80.5% and 74.3% respectively and the cumulative 1-, 2-, 5-, 10- year secondary patency rates were 99.8%, 99.8%, 98.2% and 97.2% respectively. The cumulative 1-, 2-, 5- and 10-year survival rates were 98.4%, 95.8%, 91.2% and 76.5% respectively. Illness duration and decreased serum albumin were independent predictors of survival. CONCLUSION: The ITS for Asian BCS with obstructed IVC and varying degrees of HV involvement appears to be effective and with good long-term outcomes.

Radical surgical treatment of Budd-Chiari syndrome through entire exposure of hepatic inferior vena cava.

OBJECTIVE: Therapies for Budd-Chiari syndrome (BCS) can be divided into three main categories: medical, endovascular, and surgical. Surgery is applicable to the disease when other therapeutic options have failed. We introduce a surgical method of recanalization through exposure of the entire hepatic inferior vena cava (IVC) and hepatic vein (HV) outflow tract for BCS and investigate the long-term outcomes. METHODS: From July 2002 to December 2015 in our center, 83 consecutive symptomatic BCS patients with failure of endovascular therapy were treated by radical surgical recanalization. IVC recanalization was the first goal for all patients, and recanalization of at least one HV was the second goal for selected patients at the same surgical operation. Patients were followed up, and data on technical and clinical success, survival, and patency of target vessels were analyzed. RESULTS: Technical success of surgical recanalization was achieved in 80 patients (96.4%), with relief of clinical symptoms and improvement of liver function. During a mean follow-up of 84 ± 25.9 months, the cumulative 1-, 3-, and 5-year primary patency rates of the HV were 96.7%, 90.0%, and 83.3%, respectively. The cumulative 1-, 3-, and 5-year primary patency of the IVC was 86.7%, 71.7%, and 68.3%, respectively. No factor demonstrated significant association with recurrence of obstruction. During follow-up, 10 patients died, 8 of end-stage hepatic disease and 2 of unknown causes. The cumulative 1-, 3-, and 5-year all-cause survival rates were 91%, 90%, and 87%, respectively. Female sex, encephalopathy, severe ascites, and hypersplenism had an impact on survival in univariate analysis. With Cox regression, encephalopathy was the only independent determining factor for surgical survival. CONCLUSIONS: Surgical recanalization through exposure of the entire hepatic IVC for BCS is suitable for most primary BCS patients after failure of endovascular therapies.

Percutaneous thermal ablation combined with TACE versus TACE monotherapy in the treatment for liver cancer with hepatic vein tumor thrombus: A retrospective study.

PURPOSE: To investigate the efficacy of percutaneous thermal ablation combined with transarterial chemoembolization (TACE) versus TACE monotherapy in treating primary liver cancer with hepatic vein tumor thrombus (HVTT), and to identify potential factors of overall survival after combination therapy. MATERIALS AND METHODS: Patients with primary liver cancer and HVTT from 2011 to 2016 at our institute were retrospectively identified. They were divided into two groups (group A and group B). Patients in group A underwent TACE with subsequent percutaneous thermal ablation, while patients in group B who were unsuitable for ablation received TACE monotherapy. Characteristics and survival data of the two groups were analyzed and compared. Relevant factors for overall survival (OS) of group A were explored by univariate analysis. RESULTS: Twenty-six patients were included and analyzed. The median OS for group A (n = 13) was 18 months, while the 1-, 2- and 3-year survival rates were 58.6%, 46.9% and 46.9%, respectively. The median OS for group B (n = 13) was 6.5 months and the 1-year survival rate was 10.9%. The survival of group A was significantly better than group B (P = 0.02). The following factors were related with overall survival of group A: ablation technique, complete response of tumor and HVTT, Child-pugh grade, pre-operative extrahepatic metastases and lymph node metastases. In group A, patients who achieved complete response had the longest average survival time (42.1 months). CONCLUSION: For patients with primary liver cancer and HVTT, percutaneous thermal ablation and TACE present better efficacy than TACE monotherapy. Long-term survival could be achieved in selected patients.

Budd-Chiari syndrome in China: A 30-year retrospective study on survival from a single center.

AIM: To investigate 30-year treatment outcomes associated with Budd-Chiari syndrome (BCS) at a tertiary hospital in China. METHODS: A total of 256 patients diagnosed with primary BCS at our tertiary hospital between November 1983 and September 2013 were followed and retrospectively studied. Cumulative survival rates and cumulative mortality rates of major causes were calculated by Kaplan-Meier analysis, and the independent predictors of survival were identified using a Cox regression model. RESULTS: Thirty-four patients were untreated; however, 222 patients were treated by medicine, surgery, or interventional radiology. Forty-four patients were lost to follow-up; however, 212 patients were followed, 67 of whom died. The symptom remission rates of treated and untreated patients were 81.1% (107/132) and 46.2% (6/13), respectively (P = 0.009). The cumulative 1-, 5-, 10-, 20-, and 30-year survival rates of the treated patients were 93.5%, 81.6%, 75.2%, 64.7%, and 58.2%, respectively; however, the 1-, 5-, 10-, 20-, and 30-year survival rates of the untreated patients were 70.8%, 70.8%, 53.1%, 0%, and unavailable, respectively (P = 0.007). Independent predictors of survival for treated patients were gastroesophageal variceal bleeding (HR = 3.043, 95%CI: 1.363-6.791, P = 0.007) and restenosis (HR = 4.610, 95%CI: 1.916-11.091, P = 0.001). The cumulative 1-, 5-, 10-, 20-, and 30-year mortality rates for hepatocellular carcinoma were 0%, 2.6%, 3.5%, 8%, and 17.4%, respectively. CONCLUSION: Long-term survival is satisfactory for treated Chinese patients with BCS. Hepatocellular carcinoma is a chronic complication and should be monitored with long-term follow-up.

Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd-Chiari syndrome in children.

BACKGROUND AND AIM: Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children. METHODS: Chronic BCS children were analysed in four subgroups: (i) SI: successful intervention (primary or secondary stent patency) (ii) PO: poor outcome (refractory stent block or requirement of liver transplantation), (iii) NU: naïve unintervened (awaiting RI) and (iv) DBI: died before intervention. PSS analysed included Paediatric end-stage liver disease (PELD), Rotterdam, BCS-Transjuglar intrahepatic Portosystemic shunt (BCS-TIPS) index, Zeitoun, Child-Pugh and Model for end-stage liver disease. RESULTS: Of 113 BCS children, 48 children underwent 53 successful primary RI. Actuarial probability of vascular patency was 87% at 1 year and 82% at 5 years follow-up. Four groups (SI: n = 40, PO: n = 7, NU: n = 13, DBI: n = 6) were analysed. Univariate analysis showed pre-intervention PELD score [PO: 11 (-1-23) vs SI: 2 (-8-25), P = .009] with a cut-off of 4 (AUC: 0.809, 86% sensitivity, 75% specificity) determined PO following intervention. In unintervened group (NU vs DBI), multivariate analysis demonstrated that Zeitoun score predicted death independently (OR 15.4, 95% CI: 1.17-203.56, P = .04) with a cut-off of 4.3 (AUC: 0.923, 83% sensitivity and 77% specificity). CONCLUSIONS: Children with BCS have a favourable long-term outcome. Among those undergoing RI, pre-intervention PELD score determines the outcome. Survival is determined by Zeitoun score in those unintervened.

Experience of Liver Transplant in Patients Diagnosed with Budd-Chiari Syndrome.

OBJECTIVES: Budd-Chiari syndrome can lead to fulminant hepatic failure and cirrhosis. The treatment depends on the severity of disease. Liver transplant is a successful treatment option for those with advanced-stage disease. MATERIALS AND METHODS: In this retrospective study, we analyzed all liver transplants conducted for Budd-Chiari syndrome at the organ transplant unit of Shiraz University of Medical Sciences, Iran, from 1993 to January 2016. Overall, 3201 liver transplant procedures were performed. Among these, 68 presented with Budd-Chiari syndrome. RESULTS: The median age was 31 years among 27 male and 41 female patients. Five patients received pretransplant interventions, with 2 treated with inferior vena cava stenting and 3 having transjugular intrahepatic portosystemic shunts. Sixty-five patients with Budd-Chiari syndrome received deceased-donor grafts and 3 received living-donor grafts. Among the Budd-Chiari transplant patients, 6 patients died. Five deaths occurred in the early posttransplant period, and 1 patient retransplanted after 2 years for recurrence of disease died due to graft failure. The five-year survival rate was 89% among patients with Budd-Chiari syndrome. CONCLUSIONS: Liver transplant along with posttransplant anticoagulation therapy can improve the survival of patients with advanced-stage Budd-Chiari syndrome.

Long-term outcomes of transjugular intrahepatic portosystemic shunt in Indian patients with Budd-Chiari syndrome.

BACKGROUND/AIM: Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice in Budd-Chiari syndrome (BCS) based on current data. Our objective was to evaluate outcomes and assess prognostic factors in BCS patients undergoing TIPS. PATIENTS AND METHODS: In this retrospective analysis of a propectively maintained database, all consecutive BCS patients undergoing TIPS from September 2010 to February 2017 were included. Complete response after TIPS was defined as resolution of symptoms (ascites/pedal edema) with no requirement of diuretics at the end of 4 weeks. The Cox proportional hazard regression model was used to assess predictors of outcome and complications. RESULTS: Eighty patients with BCS who underwent TIPS were included; 40 (50%) were male. The mean age at onset of symptoms was 24.2±8.7 years. The median (range) follow-up was 660 (2-2400) days. The 1-, 3-, and 5-year rates for TIPS stent patency were 89, 81, and 81%, respectively. Cumulative encephalopathy-free rates were 91, 86, and 86%, respectively, and survival rates were 93, 89, and 84%, respectively. Eight (10.0%) patients died during follow-up, five within the first year (three of these five had incomplete response). On univariate analysis, serum bilirubin, response to intervention, serum creatinine, Child class, model for end-stage liver disease, and All India Institute of Medical Sciences-hepatic venous outflow tract obstruction score were significantly different between survivors and nonsurvivors. On multivariate analysis, response to therapy after TIPS (hazard ratio: 8.37; 95% confidence interval: 1.60-43.82) was independently associated with mortality. The 1-year survival was 97% in patients with complete response, compared with 59% in those with incomplete response (P<0.004). CONCLUSION: Incomplete symptom response after TIPS is associated with poor outcome and can be used for selection of patients for liver transplantation.

Early radiological intervention and haematology screening is associated with excellent outcomes in Budd-Chiari syndrome.

BACKGROUND: Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder, resulting from thrombosis of the hepatic veins. Various treatments, including pharmacological, radiological and surgical interventions, have been used. AIM: To describe retrospectively our institution's experience with management of patients with BCS. METHODS: A retrospective study of all cases of primary Budd-Chiari syndrome presenting to our institution between January 2000 and August 2012 was performed. Patients with secondary Budd-Chiari syndrome due to malignancy or local mass compression were excluded. RESULTS: Between 2000 and 2012, 27 patients with primary BCS presented with a median Rotterdam score of 1.16 (range: 0.07-2.11). A total of 24 patients (89%) had at least one risk factor, with the commonest being myeloproliferative neoplasm (MPN), detected in 17 of 24 (71%) of the tested patients, including four patients with normal blood counts at diagnosis. All patients were anticoagulated with warfarin or low-molecular-weight heparin (LMWH). A total of 25 (92.6%) patients also had primary radiological interventions, consisting of transjugular intrahepatic portosystemic shunt (TIPS) in 18 (67%) patients and/or angioplasty/stenting in 11 (40%). A total of 14 patients developed TIPS stenoses, requiring a median of 1.5 (range: 1-14) revisions. No patient developed TIPS failure requiring alternative therapy. Two patients were lost to follow-up. At a median follow up of 59 months (range: 2-248 months), the overall survival was 96% at 1 year and 81% at 5 years, much greater than predicted by the Rotterdam score. No patients required liver transplantation. CONCLUSION: There is a high incidence of MPN in patients with primary BCS, including patients with normal peripheral blood counts at the time of diagnosis. Our approach of anticoagulation, aggressive and early radiological intervention aimed at rapid decompression of the congested liver resulted in excellent medium-term outcomes.

Clinical Characteristics and Outcome of Budd-Chiari Syndrome at a Tertiary Care Hospital in Pakistan.

OBJECTIVE: To determine the clinical characteristics of Budd-Chiari syndrome (BCS), its causes and outcome at a tertiary care hospital. STUDY DESIGN: An observational study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital,Karachi, from 2004 to 2014. METHODOLOGY: Aretrospective analysis of data was conducted. Apredesigned questionnaire was filled from medical records of patients with BCS. Clinical features, etiology, management and outcome was noted from 2004 to 2014. Descriptive statistics were determined. RESULTS: Forty-five patients' charts were reviewed; 26 (57.8%) were male patients. The median (IQR) age at diagnosis was 26.0 (20.5 to 34.5) years. Primary BCS was seen in 27 (60.0%) patients. The most frequent clinical features included ascites (82.2%), abdominal pain (55.6%), and hepatomegaly (31.1%). Acombined hepatic vein/inferior vena cava block was found in 25 (55.6%) patients. Out of the 28 tested patients protein C and protein S deficiencies were detected in 22 (78.6%) and 17 (60.7%) patients, respectively. Antithrombin III deficiency was detected in 14 (58.3%) of those tested patients. Anticoagulants were used in 24 (53.3%) patients. TIPS was done in 11 (24.4%) patients. Mortality was 6.7% (n=3). CONCLUSION: Congenital thrombophilia was a major causal factor. Age, clinical features, biochemistry and management are important factors in survival.

Budd-Chiari syndrome: outcomes of endovascular intervention-A single-center experience.

AIM: Outcomes of endovascular intervention in Budd-Chiari syndrome (BCS) have been reported with varied results. Clinical outcomes of endovascular interventions in BCS and role of various prognostic scores were critically evaluated in this study. METHODS: This study retrospectively analyzed consecutive patients of BCS who underwent endovascular intervention between January 2007 and May 2016 at our center. Technical, clinical successes and complications were documented. The role of the prognostic scores such as Child-Turcotte-Pugh (CTP), model for end-stage liver disease (MELD), Rotterdam index, and original Clichy score in predicting mortality, clinical success, and need for re-interventions were also assessed. RESULTS: A total of 88 patients were analyzed. The median follow up was 12 months (range 1-96 months). Thirteen (14.8%) patients had combined inferior vena cava (IVC) and hepatic vein (HV) obstruction; HV obstruction in 33 (37.5%) and inferior vena cava IVC obstruction in 42 (47.7%) patients. The following interventions were done: IVC angioplasty alone (n = 11), IVC angioplasty with stenting (n = 36), HV angioplasty with stenting (n = 26), combined HV and IVC stent (n = 2), and direct intrahepatic porto-systemic shunt (DIPS) (n = 13). Overall technical success was 87/88 (98.86%), and clinical success was 76/88 (86.36%). Immediate complications were noted in 8 patients (10%). The 1-, 2-, 3-, and 4-year stent patency rates were 90.91%, 81.08%, 74.59%, and 70.45%, respectively. Re-interventions were required in 15 (17%). Overall mortality was 6 (6.8%). Apart from MELD >14, none of the other prognostic score could predict mortality, clinical success, and need for re-interventions. CONCLUSION: Endovascular interventions play an important role in the management of BCS, in properly selected patients, even if prognostic score is unfavorable.

Incidence rates and case fatality rates of portal vein thrombosis and Budd-Chiari Syndrome.

Little information is available on the incidence of splanchnic vein thrombosis and on mortality rates during the acute phase of the disease. We performed a large epidemiologic study on hospital admissions for portal vein thrombosis (PVT) and the Budd-Chiari syndrome (BCS) between 2002 and 2012 in Northwestern Italy. Primary and secondary discharge diagnoses of PVT and BCS were identified using the 9th edition International Classification of Diseases codes 453.0, 572.1 and 452. Hospitalisations for recurrent events were not included. Information was collected on age and gender, vital status at discharge, duration of hospitalisation, and up to five secondary discharge diagnoses. Comorbidity was evaluated using the Charlson comorbidity index (CCI). A total of 3535 patients with PVT and 287 with BCS were hospitalized. The overall gender-specific incidence rates for PVT were 3.78 per 100,000 inhabitants in males and 1.73 per 100,000 inhabitants in females; for BCS 2.0 and 2.2 per million inhabitants, respectively. In-hospital case fatality was 7.3 % in patients with PVT and 4.9 % in patients with BCS. Age, non-abdominal solid cancer, and CCI were independently associated with in-hospital mortality in both PVT and BCS after stepwise regression analysis, male gender and haematologic cancer were associated with mortality in BCS patients only. In this large study we confirmed the low incidence of BCS and we found an incidence of PVT higher than previously reported. This incidence was stable during the period of observation. In-hospital mortality is not negligible, in particular in PVT patients.

Budd-chiari syndrome causing acute liver failure: A multicenter case series.

Budd-Chiari syndrome (BCS) is a rare disease resulting from obstruction of the hepatic venous outflow tract that typically presents with abdominal pain, jaundice, and ascites without frank liver failure. However, BCS may also evolve more rapidly to acute liver failure (ALF). In this study, we describe the clinical features, treatment, and outcomes of ALF due to BCS and compare our results with those in the published literature. Twenty of the 2344 patients enrolled in the Acute Liver Failure Study Group (ALFSG) registry since 1998 presented with a clinical diagnosis of BCS. An additional 19 patients of ALF-BCS in the English language literature were reviewed and compared with the ALFSG cases. Most ALF-BCS patients were white (84%) and female (84%) in their fourth decade. A hypercoagulable state was noted in 63% of patients. BCS was diagnosed by Doppler ultrasonography or abdominal computed tomography in all patients. Liver biopsies (n = 6) all had evidence of severe pericentral necrosis. Treatments used included most commonly anticoagulation (71%), but also transjugular intrahepatic portosystemic shunt (TIPS; 37%) and orthotopic liver transplantation (37%). In-hospital mortality was approximately 60%. In conclusion, BCS is a rare cause of ALF and mandates prompt diagnosis and management for successful outcomes. Once the diagnosis is confirmed, prompt anticoagulation is recommended in conjunction with evaluation for malignancy or thrombophilic disorder. Mortality may have improved in recent years with use of TIPS and/or orthotopic liver transplantation compared with prior published reports. Liver Transplantation 23 135-142 2017 AASLD.

Techniques of TIPS in the treatment of liver cirrhosis combined with incompletely occlusive main portal vein thrombosis.

The patients of liver cirrhosis associated with portal vein thrombosis (PVT) can be effectively treated by transjugular intrahepatic portosystemic stent shunt (TIPS). Although the corresponding TIPS procedures have already performed on the patients to different types of PVT, the procedures are not specific and the relationship between different types of PVT and technical success rate of TIPS is unclear. What's more, we aimed to explore the relationship between survival and vascular patency immediately after TIPS. 191 subjects underwent retrospective assessment. Appropriate TIPS procedures were performed based on our more specific classification. The overall success rate of TIPS was 95.8% (183/191). Success rate was significantly different between Grade II and Grade IV thrombosis (χ(2) = 5.294, P = 0.021). The 1-, 2-, 3-, 4-and 5-year survival rates were 95.6%, 89.1%, 83.1%, 76.5% and 67.8%, respectively. The overall survival time of completely patent PV and incomplete patent PV immediately after TIPS was 57.05 ± 0.75 vs. 39.12 ± 2.64 months, respectively (P < 0.0001). We conclude that appropriate TIPS procedures and lower grade of PVT are essential for better technical success rate of TIPS. The patency of target vessels is important for survival.