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.

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.

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.

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.

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.

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.

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.

Endovascular Management of Budd-Chiari Syndrome with Inferior Vena Cava Thrombosis: A 14-Year Single-Center Retrospective Report of 55 Patients.

PURPOSE: To evaluate safety and efficacy of balloon dilation and stent placement combined with thrombus aspiration and thrombolysis to treat patients with Budd-Chiari syndrome (BCS) with inferior vena cava (IVC) thrombosis. MATERIALS AND METHODS: Charts from 55 consecutive patients with primary BCS and IVC thrombosis treated between April 2000 and August 2014 were retrospectively analyzed. Transcatheter aspiration and percutaneous recanalization were attempted in all patients, and stents were placed if balloon dilation was successful. Catheter-directed thrombolysis was performed when evident clot burden was present after recanalization. RESULTS: Technically successful IVC recanalization was achieved in 53 of 55 patients (96.4%). Technical failures in 2 patients were due to long segment of IVC obstruction. A stent was placed in 47 of 53 patients (88.7%). Thrombus was successfully aspirated in 23 patients, and thrombolytic treatment was administered to 13 patients. Median follow-up was 58 months (range, 8-180 mo). No symptomatic pulmonary embolism occurred. Reocclusion occurred in 8 patients, and 6 of these patients (75%) underwent repeat recanalization by balloon dilation with or without stents. Cumulative 1-, 5-, and 10-year primary patency rates were 94%, 89%, and 66%. Alanine transaminase and alkaline phosphatase levels were independent risk factors for reocclusion. Cumulative 1-, 5-, and 10-year survival rates were 90%, 86%, and 86%. Child-Pugh score and reocclusion were independent predictors of survival. CONCLUSIONS: Percutaneous vena caval balloon dilation and stent placement with thrombus aspiration and thrombolytic therapy is safe and effective for treatment of patients with BCS and IVC thrombosis.

Long-term safety and outcome of percutaneous transhepatic venous balloon angioplasty for Budd-Chiari syndrome.

BACKGROUND AND AIMS: The restenosis following percutaneous transluminal balloon angioplasty (PTBA) is high for Budd-Chiari syndrome (BCS) patients with hepatic venous obstruction (HVO). We aim to evaluate the safety and long-term outcome of PTBA with a large balloon catheter in a large series of patients with HVO. METHODS: Between January 2005 and December 2013, 93 consecutive BCS patients with HVO were referred for PTBA and subsequently underwent color Doppler ultrasonography or angiography follow-up. Data were retrospectively collected, and follow-up observations were performed at 1-, 2-, 2- to 5-, and 5- to 8-years postoperatively. RESULTS: Percutaneous transluminal balloon angioplasty was technically successful in all patients. Ninety-one patients (97.85%) were treated with PTBA and two with PTBA and stent. Major procedure-related complications occurred in six of the 93 patients (6.45%). The cumulative 1-, 2-, 2- to 5-, and 5- to 8-year primary patency rates were 97.5%, 92.9%, 90%, and 86.5%, respectively. Cumulative 1-, 2-, 2- to 5-, and 5- to 8-year secondary patency rates were 100%, 100%, 98.6%, and 97.3%, respectively. Mean and median primary patency rates were 51.50 ± 3.01 months and 55.0 ± 3.63 months, respectively. Cumulative 1-, 2-, 2- to 5-, and 5- to 8-year survival rates were 98.75%, 98.6%, 100%, and 100%, respectively. Mean and median survival times were 53.10 ± 3.04 months and 55.0 ± 3.64 months, respectively. CONCLUSION: Percutaneous transluminal balloon angioplasty with a large balloon is a safe and effective treatment that could provide excellent rates of long-term patency and survival for the majority of Chinese patients with BCS and HVO.

Long-term outcome of liver transplant patients with Budd-Chiari syndrome secondary to myeloproliferative neoplasms.

BACKGROUND: A considerable proportion of patients receiving liver transplants for Budd-Chiari syndrome (BCS) suffer from myeloproliferative neoplasms (MPN). This study evaluated the long-term prognosis of liver-transplanted patients with BCS secondary to MPN and the effect of immunosuppression on MPN progression. METHODS: A total of 78 patients with BCS were evaluated between 1982 and 2013. Of those, 40 patients suffered from polycythaemia vera (PV) and essential thrombocythaemia (ET). One patient had primary myelofibrosis (PMF). All patients received the standard immunosuppressive regimen. We retrospectively evaluated the long-term survival, clinical course and laboratory parameters of patients with MPN. RESULTS: Exactly 29/41 patients (71%) with MPN survived ≥ 3 years [mean age 36 ± 11 years; females n = 27 (93%)]. Mean follow-up after orthotopic liver transplantation (OLT) was 12.4 ± 7.3 years (range 3-28 years). Five- and 10-year survival rates were not significantly different in patients with and without MPN (P = 0.81 and P = 0.66 respectively) or in patients with PV and ET (P = 0.29 and P = 0.55 respectively). Thrombosis and bleeding developed in 7/29 (24%) long-term MPN survivors with no significant difference between ET and PV (P = 0.18). In the long-term follow-up, there was no evidence of progression to overt myelofibrosis or acute myeloid leukaemia (AML). In the uni- and multivariate Cox-regression analyses, MPN did not influence survival after OLT. CONCLUSIONS: Budd-Chiari syndrome patients with and without underlying MPN had similar long-term survival rates after OLT. There was no evidence of enhanced progression of MPN after OLT secondary to immunosuppressive therapy. However, major haemorrhage and recurrent thrombosis contributed to morbidity and mortality after OLT in those patients.

Budd-Chiari syndrome: investigation, treatment and outcomes.

Budd-Chiari syndrome is a rare disorder characterised by hepatic venous outflow obstruction. It affects 1.4 per million people, and presentation depends upon the extent and rapidity of hepatic vein occlusion. An underlying myeloproliferative neoplasm is present in 50% of cases with other causes including infection and malignancy. Common symptoms are abdominal pain, hepatomegaly and ascites; however, up to 20% of cases are asymptomatic, indicating a chronic onset of hepatic venous obstruction and the formation of large hepatic vein collaterals. Doppler ultrasonography usually confirms diagnosis with cross-sectional imaging used for complex cases and to allow temporal comparison. Myeloproliferative neoplasms should be tested for even if a clear causative factor has been identified. Management focuses on anticoagulation with low-molecular-weight heparin and warfarin, with the new oral anticoagulants offering an exciting prospect for the future, but their current effectiveness in Budd-Chiari syndrome is unknown. A third of patients require further intervention in addition to anticoagulation, commonly due to deteriorating liver function or patients identified as having a poorer prognosis. Prognostic scoring systems help guide treatment, but management is complex and patients should be referred to a specialist liver centre. Recent studies have shown comparable procedure-related complications and long-term survival in patients who undergo transjugular intrahepatic portosystemic shunting and liver transplantation in Budd-Chiari syndrome compared with other liver disease aetiologies. Also, the optimal timing of these interventions and which patients benefit from liver transplantation instead of portosystemic shunting remains to be answered.

Clinical presentation and predictors of survival in patients with Budd Chiari syndrome: experience from a tertiary care hospital in Pakistan.

OBJECTIVE: To determine aetiology, clinical presentation and predictors of survival in Budd Chiari Syndrome patients. METHODS: The prospective observational study based on non-probability convenient sampling was conducted at the Sindh Institute of Urology and Transplantation (SIUT), Karachi, and comprised Budd Chiari Syndrome patients between January 2004 and December 2013. The patients were evaluated for onset of symptoms, causes, mode of presentation and predictors of survival. SPSS 20 was used for statistical analysis. RESULTS: Of the 25 patients, 16(64%) were males, and 16(64%) belonged to the paediatric age group. Overall age range was 2-50 years with a mean of 14.7 ± 12.41 years. Presentation was chronic in 14(56%) patients, acute in 10(40%) and acute on chronic in 1(4%). Commonest morphological abnormality involved was hepatic veins alone in 14(56%). Probable aetiologies were hypercoagulable states in 21(84%) patients, infections in 2(8%) and malignancy in 1(4%). Among hypercoagulable states, protein C deficiency was the commonest, affecting 9 (36%) patients. Seven (28%) patients died; acute 4 (16%) and chronic 3 (12%). Causes of death included sepsis 4 (16%), fulminant hepatic failure 1 (4%), gastrointestinal bleeding 1 (4%), and bleeding from liver biopsy site 1 (4%). Poor survival was associated with bilirubin > 5 mg/dl (p < 0.031), serum alanine transaminase > 40 U/L (p < 0.005), serum albumin < 2.8 g/dl (p < 0.008), Child-Turcotte-Pugh score > 10 (p < 0.001) and absence of varices (p < 0.025). Cox regression analysis failed to show any significant independent predictors of survival. CONCLUSION: Budd Chiari Syndrome affected young patients more frequently and was associated with high mortality. The commonest aetiology was hypercoagulable state. Survival was poor in patients with decompensated liver disease and those with an acute clinical presentation.

Surgical treatment of hepatocellular carcinoma associated with hepatic vein tumor thrombosis.

BACKGROUND & AIMS: Presence of hepatic vein tumor thrombosis (HVTT) in patients with hepatocellular carcinoma (HCC) is regarded as signaling an extremely poor prognosis. However, little is known about the prognostic impact of surgical treatment for HVTT. METHODS: Our database of surgical resection for HCC between October 1994 and December 2011 in a tertiary care Japanese hospital was retrospectively analysed. We statistically compared the patient characteristics and surgical outcomes in HCC patients with tumor thrombosis in a peripheral hepatic vein, including microscopic invasion (pHVTT), tumor thrombosis in a major hepatic vein (mHVTT), and tumor thrombosis of the inferior vena cava (IVCTT). Among 1525 hepatic resections, 153 cases of pHVTT, 21 cases of mHVTT, and 13 cases of IVCTT were identified. RESULTS: The median survival time (MST) in the pHVTT and mHVTT groups was 5.27 and 3.95 years, respectively (p=0.77), and the median time to recurrence (TTR) was 1.06 and 0.41 years, respectively (p=0.74). On the other hand, the MST and TTR in the patient group with IVCTT were 1.39 years and 0.25 year respectively; furthermore, the MST of Child-Pugh class B patients was significantly worse (2.39 vs. 0.44 years, p=0.0001). Multivariate analyses revealed IVCTT (risk ratio [RR] 2.54, p=0.024) and R 1/2 resection (RR 2.08, p=0.017) as risk factors for the overall survival. CONCLUSIONS: Hepatic resection provided acceptable outcomes in HCC patients with mHVTT or pHVTT when R0 resection was feasible. Resection of HCC may be attempted even in patients with IVCTT, in the presence of good liver function.

Good long-term outcome of Budd-Chiari syndrome with a step-wise management.

UNLABELLED: Budd-Chiari syndrome (BCS) is a rare, life-threatening disease caused by obstruction of hepatic venous outflow. The aim of the study was to assess long-term outcome and identify prognostic factors in BCS patients managed by a step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPS), and orthotopic liver transplantation (OLT). We reviewed long-term data on 157 patients previously included by the European Network for Vascular Disorders of the Liver, a multicenter prospective study of newly diagnosed BCS patients in nine European countries. Patients were followed for a median of 50 months (range, 0.1-74.0). During the study, 88 patients (56%) received at least one invasive intervention (22 patients angioplasty/thrombolysis, 62 TIPS, and 20 OLT) and 36 (22.9%) died. Most interventions and/or deaths occurred in the first 2 years after diagnosis. The Rotterdam score was excellent in predicting intervention-free survival, and no other variable could significantly improve its prognostic ability. Moreover, BCS-TIPS prognostic index (PI) score (based on international normalized ratio, bilirubin, and age) was strongly associated with survival and had a discriminative capacity, which was superior to the Rotterdam score. CONCLUSIONS: The current study confirms, in a large cohort of patients with BCS recruited over a short period, that a step-wise treatment approach provides good long-term survival. In addition, the study validates the Rotterdam score for predicting intervention-free survival and the BCS-TIPS PI score for predicting survival.

Long-term follow-up study in Budd-Chiari syndrome: single-center experience in 22 years.

GOALS: We aimed to present our long-term surveillance experience in patients with Budd-Chiari syndrome (BCS), and we retrospectively evaluated the natural history, results of thrombophilia studies, and the factors related to mortality. BACKGROUND: Primary BCS is a rare form of vascular disease, secondary to underlying thrombophilia. Because of its rarity and heterogeneous nature, there is a scarcity of knowledge about the natural history of the disease. STUDY AND RESULTS: In 22 years, a total of 62 patients with primary BCS were followed in our tertiary hospital. We identified an acquired cause of BCS in 40 out of 62 patients (64.5%), whereas in 6 patients (9.7%), we found no identifiable cause. One or more thrombophilia causes were identified in 56 patients (90.3%). In 19 patients with myeloproliferative disease, 15 had Janus tyrosine kinase 2 mutation analysis and Janus tyrosine kinase 2 positivity was found in 10 patients. In regression analysis, portal vein thrombosis was found to be the only indicator of mortality, with an estimated instantaneous risk of 8.4. CONCLUSIONS: In this study, we present one of the largest series of BCS in the English literature. We have shown that the multifactorial nature of underlying thrombophilia should be thoroughly investigated. In a patient with BCS, a clinician should be alert for the development or coexistence of portal vein thrombosis due to its deleterious effect on mortality.

Transjugular intrahepatic portosystemic shunt in the treatment of Budd-Chiari syndrome: a critical review of literatures.

An increasing number of patients with Budd-Chiari syndrome (BCS) have undergone transjugular intrahepatic portosystemic shunt (TIPS). However, the critical role of TIPS in the treatment of BCS has not been systematically reviewed. The authors identified all relevant literatures via the PubMed, EMBASE and Cochrane library databases. Overall, 160 papers from 29 countries reported the application of TIPS for BCS. The number of publications was increased over time, but the level of evidence in this field was low. Common indications for TIPS in BCS patients included refractory ascites, recurrent variceal bleeding, diffuse hepatic vein thrombosis and progressive liver failure. Successful TIPS insertion could improve the hemodynamic and clinical parameters. TIPS procedure-related complications were not infrequent (range: 0-56%), but procedure-related death was rare. Shunt dysfunction rate appeared to be higher (range: 18-100%). Compared with bare stents, covered stents could significantly decrease the rate of shunt dysfunction. Hepatic encephalopathy rate after TIPS was relatively low (range: 0-25%). Short- and long-term prognosis of BCS-TIPS patients was excellent with 1-year cumulative survival rate of 80-100% and 5-year cumulative survival rate of 74-78%. In conclusions, existing literatures supported the feasibility, safety and efficacy of TIPS in the treatment of BCS. Prospective cohort studies or randomized controlled trials were difficult due to the rarity of BCS, but might be very necessary to precisely identify the timing of transition from medical therapy and/or percutaneous recanalization to TIPS insertion and the real candidates in whom early TIPS should be promptly employed with no need of any prior therapy.

Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins.

BACKGROUND: Collateralized intra- and extra-hepatic routes in patients with Budd-Chiari syndrome (BCS) were important. This study aimed to investigate the feasibility and clinical outcomes of the staged management of BCS based on the degree of compensation provided by intra- or extra-hepatic collateral circulations. METHODS: A total of 103 adult patients with BCS caused by co-obstruction of the inferior vena cava (IVC) and main hepatic veins (MHVs) between March 2001 and October 2009 were enrolled in this study. Based on the pathological classification and degree of hemodynamic compensation by collateral circulations, treatment priority for IVC hypertension was determined in the first-stage treatment. Patients were deemed eligible for second-stage treatment when the first-stage treatment failed to relieve. RESULTS: Imaging results revealed that most patients had collateral circulations to different extents. Based on the degree of compensation provided by these collateral circulations, 74 patients underwent single-stage treatment for IVC hypertension, i.e., radiologic intervention (RI) for 61 patients and surgical procedures (SPs) for 13. One patient was treated for portal hypertension. Twenty-nine patients underwent second-stage treatment (25 underwent RI and SP, and 4 only SP). The general morbidity and mortality after all procedures were 8.3% and 1.5%, respectively. After a median follow-up of 35 months, 4 patients underwent second-stage treatment and 7 underwent recanalization of the IVC/MHVs. Two patients died of hepatocellular carcinoma and 1 died of graft obstruction. CONCLUSION: Staged management produces excellent outcomes for patients with BCS caused by co-obstruction of the IVC and MHVs.

Primary Budd-Chiari syndrome - a single center experience.

BACKGROUND/AIMS: Budd-Chiari syndrome (BCS) is recognized as a clinical manifestation of various prothrombotic conditions which may be lethal within 3 years of the onset of symptoms if untreated. This study is a retrospective analysis of patients with BCS managed between 2004 and 2011. METHODOLOGY: The diagnosis was confirmed with contrast CT-angiography and/or Doppler ultrasound. RESULTS: BCS was diagnosed in 20 patients (11 females and 9 males), median age 38 years (ranging from 18 to 56). Twelve patients were referred as acute BCS for the liver transplant (LTx) assessment. Thrombosis of the hepatic veins was caused by myeloproliferative disorders (n=8), end-stage liver disease (n=4), protein C deficiency (n=3), paroxysmal nocturnal hemoglobinuria (PNH) (n=1), antiphospholipid syndrome (n=1) and secondary poliglobulia (n=1). In two patients the origin of BCS could not be established despite appropriate screening. Median follow-up was 29 months. Low molecular heparin with subsequent conversion to vitamin K antagonists was routinely applied in all patients. Two patients underwent TIPS procedure with good long term outcome and 10 subjects received LTx; 1 patient was lost to follow-up and 1 died of chest infection 9 years since the diagnosis of BCS was made; 14 patients, including those who received LTx, were alive and well at least one year after BCS diagnosis. All survivors remain stable and are followed-up on a regular basis. CONCLUSIONS: Strict adherence to the diagnostic and therapeutic guidelines plays a crucial role in the management of BCS patients. Our results confirm the efficacy of anticoagulation as well as TIPS and/or OLT in treatment of this rare condition.

Long-term follow-up study and comparison of meso-atrial shunts and meso-cavo-atrial shunts for treatment of combined Budd-Chiari syndrome.

BACKGROUND: To investigate therapeutic effects of meso-atrial shunts (MASs) and meso-cavo-atrial shunt (MCASs) in combined Budd-Chiari syndrome (BCS). METHODS: We retrospectively gathered 29 cases of combined BCS with all or bilateral hepatic vein occlusion and long range occlusion or obstruction of inferior vena cava (IVC). Of them, 12 were treated with a MAS and 17 with a MCAS. We analyzed pre- and postoperative clinical symptoms, IVC, and portal venous (PV) pressure, occurrence rate of postoperative complications, patency rate of artificial vessels, and survival rate. RESULTS: One patient in the MAS group died in the preoperative period. During the follow-up period from 3 d to 60 mo, 96.6% were included. The effective rates were 54% in MAS and 90.8% in MCAS. The occurrence rates of postoperative complications were 12.5% and 1.8%, respectively. The average decreases in IVC and PV pressure were 15.5 mm saline and 62.4 mm saline for MAS, and 12.3 mm saline and 184.7 mm saline for MCAS. The survival rates at 1, 3, and 5 y were 41.7%, 41.7%, and 16.7% for MAS, and 94.1%, 88.2%, and 82.4% for MCAS. The 5-y patency rates were 41.7% and 94.1%. Comparing these two groups, all of the studied factors, with the exception of PV pressure, were significantly different (P<0.05). Thus, the therapeutic effects of MCASs were better than those of MASs. CONCLUSION: MCAS can simultaneously relieve high IVC and PV pressure in combined BCS. Compared with MASs, MCASs showed a decreased postoperative complication rate, a higher 5-y survival and artificial vessel patency rate.