Pediatric Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver disease in Europe and North America. In order to understand the long-term effect of radiological intervention on BCS we performed a single center retrospective review. Fourteen cases were identified; 6 of 14 (43%) had a congenital thrombophilia with many having multiple prothrombotic mutations. Two were managed with medical anticoagulation alone and two required super-urgent transplant for acute liver failure. The remaining 10 of 14 (71%) underwent radiological intervention: 1 of 14 thrombolysis, 5 of 14 angioplasty, and 4 of 14 transjugular intrahepatic portosystemic shunt (TIPS). Six of 14 (43%) patients required repeat radiological intervention (1 angioplasty, 5 TIPS) but none required surgical shunts or liver transplantation for chronic liver disease. The time between diagnosis and treatment did not predict the need for repeat radiological intervention. These data show that radiological intervention can be highly effective, and reduces the need for surgery, though it requires specialist multidisciplinary teams for monitoring.

IntroductionRefractory ascites is a serious complication of cirrhosis and portal hypertension with a 1 year survival rate of 50%. TIPPS (transjugular intrahepatic portosystemic shunt) is a treatment option in selected patients with refractory ascites.1 The aim of this project was to assess the outcomes of patients who underwent a covered TIPSS to those who had large volume paracentesis (LVP).MethodsWe performed a retrospective study of all patients who underwent a covered TIPSS for refractory ascites during April 2010 to November 2017. This was compared to all patients who underwent LVP (mean 1.28±0.55 paracenteses per month) during a similar time period. Biochemical and clinical parameters were compared. The primary outcome was transplant free survival.ResultsThe sample size in each group was n=76 in TIPSS group, n=86 in LVP group, giving a ratio of 1:1.1. The mean ages were 59±9.5 & 61±11.4 years. There was a male predominance of 53% and 60% respectively in the TIPSS and LVP group. Alcohol related liver disease was the most prevalent aetiology (75% TIPSS group; 56% LVP group). The MELD score was significantly higher in the LVP group (11.5±3.8 vs 15.6±5.2, p<0.05). 26 patients in the LVP group underwent liver transplantation vs 10 patients in the TIPSS group. There was no difference in SBP presence between groups. Overall follow up was 20±20.6 months. Transplant free survival time at 6,12,24,60 months is as follow: TIPSS: 76%, 64%, 50%, 21%; LVP group 79%, 55%, 38%, 19% (p=NS, figure 1). No clinical or biochemical variables were associated with survival on cox regressions analysis. A subset of patients in the LVP group (n=48) who would be considered suitable for TIPSS based on the following parameters (platelet count≥ 75 109/L, bilirubin≤ 50 micromol/L, absence of pre-exist hepatic encephalopathy(2)) was compared with the TIPSS group (n=76). Further analysis showed transplant free survival remained similar in both groups.ConclusionsOur study shows that, in a real-world cohort of advanced liver failure patients, covered TIPSS did not result in improved transplant free survival compared to LVP. Therefore, liver transplantation remains the best option for refractory ascites in selected patients. Further controlled studies are required, to identify prognostic markers to assist in selecting appropriate candidates for TIPSS.ReferencesBureau C, Thabut D, Oberti F, Dharancy S, Carbonell N, Bouvier A, et al. Transjugular intrahepatic portosystemic shunts with covered stents increase transplant-free survival of patients with cirrhosis and recurrent ascites. Gastroenterology. 2017;152(1):157–63. Transjugular intrahepatic portosystemic stent-shunt in the management of portal hypertension; Tripathi D, et al. Gut 2020 in press

Introduction Budd Chiari syndrome (BCS) is a rare but potentially life-threatening condition. Recanalization using TIPSS or hepatic venous stenting is key to relieving hepatic congestion. These procedures are impossible in complete HV occlusion. Direct intrahepatic portocaval shunt (DIPS) is a new procedure where a stent is placed directly from the inferior vena cava, often through the caudate lobe, to the portal vein and therefore bypassing the thrombosed HVs. We report our experience in using DIPS for recanalization in BCS. Methods Single centre retrospective analysis from May 2015 to January 2019 comparing outcomes following a DIPS insertion compared to our centre’s previously published data. Results 14 patients were referred for a DIPS procedure. M:F ratio 8:6; age 40.5±13.2; follow up 23.1±15.0 months. HV-BCS type in all. Aetiology: myeloproliferative neoplasm (MPN) in 7, all JAK2+ve with mutation load 17.3±10.2%; PNH, 1; idiopathic, 6 (all –ve following next generation sequencing). Pre-DIPS: MELD 13.1±3.2, UKELD 49.1±13.35, BCS-TIPS PI score 4.45±1.1. Post DIPS portal pressure gradient was 6.9±2.2 mmHg. Clinical indication: variceal bleeding and ascites (n=1) or ascites (n=13). Multidisciplinary consensus to undertake a DIPS insertion as a first line procedure was reached in 13 patients, in 1 patient a TIPSS insertion was initially attempted, when this failed a rescue DIPS was performed. One DIPS insertion (7%) was not successful, this patient is now on the waiting list for transplantation. In all remaining patients, successful stent placement was achieved, and none required escalation to transplantation. Ascites resolution was seen in 7 out of 11 patients at follow up (64%). 2 patients developed hepatic encephalopathy post DIPS (14%). Primary patency rates at 6 months, 1 year, and 2 years were 83%, 83%, 58% respectively. Secondary patency was 100%. Transplant free survival 100% to date. The outcomes are comparable to a previously reported series from the same institution, with similar BCS-TIPS PI but slightly lower MELD. Conclusion Our data demonstrates that with technical excellence, multidisciplinary management, and careful patient selection, DIPS results in very good clinical outcomes in patients unsuitable for standard TIPSS. The outcomes are comparable to standard TIPSS from our historic data. We strongly recommend early referral of all patients with BCS to multidisciplinary teams in centres that offer advanced interventional radiology and liver transplantation.

Purpose The purpose of this study was to evaluate the predictive value of a 'Modified Karnofsky Scoring System' on outcomes and provide real-world data regarding the UK practice of biliary interventions. Materials and Methods A prospective multi-centred cohort study was performed. The pre-procedure modified Karnofsky score, the incidence of sepsis, complications, biochemical improvement and mortality were recorded out to 30 days post procedure. Results A total of 292 patients (248 with malignant lesions) were suitable for inclusion in the study. The overall 7 and 30 day mortality was 3.1% and 16.1%, respectively. The 30 day sepsis rate was 10.3%. In the modified Karnofsky 'high risk' group the 7 day mortality was 9.7% versus 0% for the 'low risk' group (p = 0.002), whereas the 30 day mortality was 28.8% versus 13.3% (p = 0.003). The incidence of sepsis at 30 days was 19% in the high risk group versus 3.3% at the low risk group (p = 0.001) Conclusion Percutaneous biliary interventions in the UK are safe and effective. Scoring systems such as the Karnofsky or the modified Karnofsky score hold promise in allowing us to identify high risk groups that will need more careful consideration and enhanced patient informed consent but further research with larger studies is warranted in order to identify their true impact on patient selection and outcomes post biliary interventions.

Summary Background Cardiac dysfunction is frequently observed in patients with cirrhosis. There remains a paucity of data from routine clinical practice regarding the role of echocardiography in the pre‐assessment of transjugular intrahepatic portosystemic stent‐shunt. Aim Our study aimed to investigate if echocardiography parameters predict outcomes after transjugular intrahepatic portosystemic stent‐shunt insertion in cirrhosis. Methods Patients who underwent echocardiography and transjugular intrahepatic portosystemic stent‐shunt insertion at the liver unit (Birmingham, UK) between 1999 and 2016 were included. All echocardiography measures (including left ventricle ejection fraction; early maximal ventricular filling/late filling velocity ratio, diastolic dysfunction as per British Society of Echocardiography guidelines) were independently reviewed by a cardiologist. Predictors of 30‐day and overall transplant free‐survival were assessed. Results One Hundred and Seventeen patients with cirrhosis (median age 56 years; 54% alcohol; Child‐Pugh B/C 71/14.5%; Model For End‐Stage Liver Disease 12) underwent transjugular intrahepatic portosystemic stent‐shunt for ascites (n = 78) and variceal haemorrhage (n = 39). Thirty‐day and overall transplant‐free survival was 90% (n = 105) and 31% (n = 36), respectively, over a median 663 (IQR 385‐2368) days follow‐up. Model for End‐Stage Liver Disease ( P < 0.001) and Child‐Pugh Score ( P = 0.002) significantly predicted 30‐day and overall transplant‐free survival. Model for End‐Stage Liver Disease ≥15 implied three‐fold risk of death. Six per cent (n = 7) of patients pre‐transjugular intrahepatic portosystemic stent‐shunt had a history of ischaemic heart disease and 34% (n = 40) had 1 or more cardiovascular disease risk factors. Fifty per cent (n = 59) had an abnormal echocardiogram and 33% (n = 39) had grade 1‐3 diastolic dysfunction. On univariate analysis none of the echocardiography measures pre‐intervention were related to 30‐day or overall transplant‐free survival post‐transjugular intrahepatic portosystemic stent‐shunt. Conclusions Ventricular, in particular diastolic dysfunction in patients with cirrhosis does not predict survival after transjugular intrahepatic portosystemic stent‐shunt insertion. Model for End‐Stage Liver Disease and Child‐Pugh scores remain the best predictors of survival. Further prospective study is required to clarify the role of routine echocardiography prior to transjugular intrahepatic portosystemic stent‐shunt insertion.

AIMTo analyse the risk of pregnancy (a prothrombotic state) in patients with Budd-Chiari Syndrome (BCS).METHODSRetrospective study of pregnancy in women with known BCS at single center from January 2001 to December 2015.RESULTSOut of 53 females with BCS, 7 women had 16 pregnancies. Median age at diagnosis of BCS in these women was 25 years (range 21-34 years). At least one causal factor for BCS was identified in 6 women (86%). Six women had undergone radiological decompressive treatment. All patients had anticoagulation. Six fetuses were lost before 20 wk gestation in 2 women. There were 9 deliveries over 32 wk gestation and one delivery at 27 wk. All infants did well. Seven babies were born by emergency caesarean section. There were no cases of thrombosis. Two patients had notable vaginal (PV) bleeding in 3 pregnancies. None of the patients had variceal haemorrhage. Two patients were diagnosed with pulmonary hypertension, one during pregnancy and the other in the post-partum period. There was no maternal mortality.CONCLUSIONMaternal outcomes in patients with treated BCS are favourable and fetal outcomes beyond 20 wk gestation are good. There has been increased rate of caesarean section. Pulmonary hypertension is an important finding that needs further validation. These patients should be managed in centers experienced in treating high-risk pregnancies.

SummaryBackgroundValidated diagnostic tools that are accurate, cost effective and acceptable to patients are required for disease stratification and monitoring in NAFLD.AimsTo investigate the performance and cost of multiparametric MRI alongside existing biomarkers in the assessment of NAFLD.MethodsAdult patients undergoing standard of care liver biopsy for NAFLD were prospectively recruited at two UK liver centres and underwent multiparametric MRI, blood sampling and transient elastography withing 2 weeks of liver biopsy. Non‐invasive markers were compared to histology as the gold standard.ResultsData were obtained in 50 patients and 6 healthy volunteers. Corrected T1 (cT1) correlated with NAFLD activity score (ρ = 0.514, P < .001). cT1, enhanced liver fibrosis (ELF) test and liver stiffness differentiated patients with simple steatosis and NASH with AUROC (95% CI) of 0.69 (0.50‐0.88), 0.87 (0.77‐0.79) and 0.82 (0.70‐0.94) respectively and healthy volunteers from patients with AUROC (95% CI) of 0.93 (0.86‐1.00), 0.81 (0.69‐0.92) and 0.89 (0.77‐1.00) respectively. For the risk stratification of NAFLD, multiparametric MRI could save £150,218 per 1000 patients compared to biopsy. Multiparametric MRI did not discriminate between individual histological fibrosis stages in this population (P = .068).ConclusionsMultiparametric MRI accurately identified patients with steatosis, stratifies those with NASH or simple steatosis and reliably excludes clinically significant liver disease with superior negative predictive value (83.3%) to liver stiffness (42.9%) and ELF (57.1%). For the risk stratification of NAFLD, multiparametric MRI was cost effective and, combined with transient elastography, had the lowest cost per correct diagnosis.

Introduction TIPSS is widely used to treat refractory ascites and variceal haemorrhage. Cardiac dysfunction is frequently observed in patients with cirrhosis. However, there remains a paucity of data from routine clinical practice regarding the use of echocardiography in the pre-assessment of TIPSS. Aim To investigate if echocardiography predicts outcomes post-TIPSS in patients with cirrhosis. Method Patients who underwent echocardiography and TIPSS at the liver transplant centre (Birmingham, UK) between 1999–2016 were included. All echocardiography measures [left ventricle (LV) ejection fraction (EF); LV diastolic diameter (LVDD); LV systolic diameter (LVSD); early maximal ventricular filling velocity/late filling velocity (E/A) ratio, deceleration time (DT), diastolic dysfunction as per ASE/BSE guidelines; regional wall abnormality) were independently reviewed/graded by a senior cardiologist (RS). Clinical predictors of 30 day, 90 day and overall transplant free-survival were assessed. Results 117 patients with cirrhosis (median age 56 years; 54% alcohol; CPB/C 71/14.5%) underwent TIPSS for ascites (n=78) and variceal haemorrhage (n=39). Median MELD and UKELD was 12 (IQR 9–17) and 53 (IQR 50–57), respectively. Median post-TIPSS portal pressure gradient was 8 (IQR 6–10) mmHg. Post-TIPSS complications ( 30 day, 90 day and overall transplant-free survival was 90% (n=105), 80% (n=93), and 31% (n=36) over a median 663 (IQR 385–2368) days follow-up. MELD (p /=15 had an AUROC 0.855 (95% CI 0.74–0.98) for 30 day transplant free-survival. 6% (n=7) of patients pre-TIPSS had a history of IHD and 34% (n=40) had 1 or more CVD risk factors. 50% (n=59) had a normal echocardiography, 33% (n=39) had grade 1–3 LV diastolic dysfunction and 6% (n=7) had LVEF Conclusion Echocardiography in patients with cirrhosis does not predict post-TIPSS survival. MELD score remains the best predictor of early and late mortality post-TIPSS. Disclosure of Interest None Declared

Hepatic venous outflow obstruction (HVOO) is a rare complication after liver transplantation (LT) associated with significant morbidity and reduced graft survival. Endovascular intervention has become the first-line treatment for HVOO, but data on long-term outcomes are lacking. We have analysed outcomes after endovascular intervention for HVOO in 905 consecutive patients who received 965 full-size LT at our unit from January 2007 to June 2014. There were 27 (3%) patients who underwent hepatic venogram for suspected HVOO, with persistent ascites being the most common symptom triggering the investigation (n = 19, 70%). Of those, only 10 patients demonstrated either stricture or pressure gradient over 10 mmHg on venogram, which represents a 1% incidence of HVOO. The endovascular interventions were balloon dilatation (n = 3), hepatic vein stenting (n = 4) and stenting with dilatation (n = 3). Two patients required restenting due to stent migration. The symptoms of HVOO completely resolved in all but one patient, with a median follow-up period of 74 (interquartile range 39-89) months. There were no procedure-related complications or mortality. In conclusion, the incidence of HVOO in patients receiving full-size LT is currently very low. Endovascular intervention is an effective and safe procedure providing symptom relief with long-lasting primary patency.

Introduction Hepatic fibrosis is an important determinant of outcome in non-alcoholic fatty liver disease (NAFLD). The severity of non-alcoholic steatohepatitis (NASH) has relevance to clinical follow up intensity and assessment of the effectiveness of treatment interventions. Magnetic resonance imaging (MRI)-acquired T1 measurement has been shown to correlate with hepatic fibrosis and is also increased by inflammation, indicating potential as a tool to stratify NAFLD. Methods Patients undergoing liver biopsy (LB) were invited for MRI and blood sampling prior to LB. Iron accumulation has been shown to reduce T1 independent of fibrosis, so an iron corrected hepatic T1 (cT1) was calculated using LiverMultiscan (Perspectum Diagnostics, Oxford). Histology was graded by two expert liver histopathologists according to the NASH-CRN system. Results 50 patients had complete data sets following exclusions (1 inadequate biopsy, 3 MRI data unavailable). 28 (56%) patients were male. Median age 54 years. 26 (52%) patients had type-2 diabetes. Mean (±SD) body mass index was 33.6 (±5.1) Kg/m2. Median (IQR) of ALT, fasting glucose and cholesterol were 54 (52) u/L, 6.2 (4.0) mmol/L and 4.8 (1.5) mmol/L, respectively. 38 (76%) LBs demonstrated NASH and the remainder simple steatosis (SS). Mean(±SD) cT1 for NASH and SS were 1007(±95) milliseconds (ms) and 907(±120) ms, respectively (p = 0.004). The correlation between cT1 and NAS was highly significant (Rho = 0.51, p < 0.0001). To differentiate NASH from SS, cT1 had an AUROC (95%CI) of 0.71 (0.53–0.89). To diagnose significant (>F1) and advanced (>F2) fibrosis cT1 had an AUROC (95% CI) of 0.65 (0.48–0.83) and 0.62 (0.47–0.78), respectively. To identify patients with SS and no significant fibrosis cT1 had an AUROC (95%CI) of 0.75 (0.56–0.93). Conclusion Multiparametric MRI using LiverMultiscan has the ability to non-invasively stage fibrosis in patients with NAFLD. The additional benefit of this novel technology is the ability to concurrently establish the severity of NASH. The correlation of cT1 and NAS suggests that multiparametric MRI has potential for monitoring the effectiveness of treatment interventions in NAFLD. Disclosure of Interest P. Eddowes: None Declared, N. McDonald: None Declared, N. Davies: None Declared, S. Semple: None Declared, S. Hübscher: None Declared, T. Kendall: None Declared, C. Kelly Employee of: Perspectum Diagnostics, M. Mavar Employee of: Perspectum Diagnostics, A. Herlihy Employee of: Perspectum Diagnostics, P. Newsome: None Declared, S. Olliff: None Declared, J. Fallowfield: None Declared, G. Hirschfield: None Declared

Background & Aims: A proportion of patients with Budd-Chiari Syndrome (BCS) associated with stenosis or short occlusion of the hepatic vein (HV) or upper inferior vena cava (IVC) can be treated with recanalization by percutaneous venoplasty +/- HV stent insertion. We studied the long-term outcomes of this approach. Methods: Single-centre retrospective analysis of patients referred for radiological assessment +/- intervention over a 27-year period. Of 155 BCS patients, 63 patients who underwent venoplasty were studied and compared to a previously reported series treated by TIPSS (n = 59). Results: Patients treated with HV interventions (32 venoplasty alone, 31 endovascular stents): mean age, 34.9 +/- 10.9; M:F ratio 27: 36; median follow-up, 113.0 months; 62% of patients had >= 1 haematological risk factor. Technical success was 100%, with symptom resolution in 73%. Cumulative secondary patency at 1, 5, 10 years was 92%, 79%, 79% and 69%, 69%, 64% in the stenting and venoplasty groups respectively. Where long-term patency was not achieved, 10 patients required TIPSS, and 8 underwent surgery. Actuarial survival at 1, 5, 10 years was 97%, 89% and 85%. When compared to TIPSS, HV interventions resulted in similar patency and survival rates but significantly lower procedural complications (9.5% vs 27.1%) and hepatic encephalopathy (0% vs 18%). Patient age predicted survival following multivariate analysis. Conclusions: Our data support the stepwise approach to management of BCS, with very good outcomes from venoplasty combined with stenting when required. TIPSS should only be offered where HV interventions are not feasible or unsuccessful.

Portal vein thrombosis (PVT) is encountered in liver cirrhosis, particularly in advanced disease. It has been a feared complication of cirrhosis, attributed to significant worsening of liver disease, poorer clinical outcomes and potential inoperability at liver transplantation; also catastrophic events such as acute intestinal ischaemia. Optimal management of PVT has not yet been addressed in any consensus publication. We review current literature on PVT in cirrhosis; its prevalence, pathophysiology, diagnosis, impact on the natural history of cirrhosis and liver transplantation, and management. Studies were identified by a search strategy using MEDLINE and Google Scholar. The incidence of PVT increases with increasing severity of liver disease: less than 1% in well-compensated cirrhosis, 7.4%-16% in advanced cirrhosis. Prevalence in patients undergoing liver transplantation is 5%-16%. PVT frequently regresses instead of uniform thrombus progression. PVT is not associated with increased risk of mortality. Optimal management has not been addressed in any consensus publication. We propose areas for future research to address unresolved clinical questions.

SummaryBackgroundThere have been encouraging reports on transjugular intrahepatic portosystemic stent‐shunt (TIPSS) for Budd–Chiari syndrome (BCS). Long‐term data are lacking.AimTo assess long‐term outcomes and validate prognostic scores following TIPSS for BCS.MethodsA single centre retrospective study. Patients underwent TIPSS using bare or polytertrafluoroethane (PTFE)‐covered stents.ResultsSixty‐seven patients received successful TIPSS between 1996 and 2012 using covered (n = 40) or bare (n = 27) stents. Patients included had a Male: Female ratio of 21:46, and were characterised (mean ± s.d.) by age 39.9 ± 14.3 years, Model of end stage liver disease (MELD) 16.1 ± 7.0 and Child's score 8.8 ± 2.0. Seventy‐eight percent had haematological risk factors. Presenting symptoms were ascites (n = 61) and variceal bleeding (n = 6). Nine patients underwent hepatic vein dilatation or stenting prior to TIPSS. Mean follow‐up was 82 months (range 0.5–184 months). Fifteen percent had post‐TIPSS encephalopathy. Two have been transplanted. Primary patency rates (76% vs. 27%, P < 0.001) and shunt re‐interventions (22% vs. 100%, P < 0.001) significantly favoured covered stents. Secondary patency was 99%. Six‐, 12‐, 24‐, 60‐ and 120‐month survival was 97%, 92%, 87%, 80% and 72% respectively. Six patients had liver related deaths. Two patients developed hepatocellular carcinoma. The BCS TIPS PI independently predicted mortality in the whole cohort, but no prognostic score was a significant predictor of mortality after subgroup validation.ConclusionsLong‐term outcomes following TIPSS for Budd–Chiari syndrome are very good. PTFE‐covered stents have significantly better primary patency. The value of prognostic scores is controversial. TIPSS should be considered as first line therapy in symptomatic patients in whom hepatic vein patency cannot be restored.

Tripathi et al. report their experience with transjugular intrahepatic portosystemic stent-shunts (TIPSS) in Budd-Chiari syndrome (BCS).1 In this single-centre retrospective study of 67 BCS patients who underwent TIPSS, Tripathi et al. show encouraging long-term outcomes, particularly with the use of covered stents. Long-term survival was excellent (80% at 5 years and 72% at 10 years). While procedure-related complications were common, they were not life threatening. The authors also validated the BCS-TIPS prognostic score in their entire cohort over the other scores. This excellent outcome, however, may be contingent on local expertise and patient characteristics. It is to be noted that all the procedures were performed single-handedly by two experienced physicians, cases had been carefully selected (only 68 of the 104 patients actually underwent TIPSS) and all patients were maintained on full anticoagulation throughout. It would have been helpful to know what criteria were used for patient selection to generalise these findings, and whether the improved survival was a function of effective anticoagulation. Owing to the lack of randomised controlled evidence and the relative rarity of BCS, the management of BCS will likely continue to be based on expert opinions and local expertise.2 In general, recanalisation attempts are advocated as the first line, and TIPSS as the rescue step when the former is unsuitable or ineffective.3 While angioplasty is common in Asia4 and surgical shunts are favoured in certain US centres,5, 6 TIPSS has become increasingly popular in Europe.3, 7 Given the feasibility, safety, efficacy profile and excellent long-term survival, the authors add to the growing body of evidence regarding the importance of TIPSS as a viable option for Budd-Chiari syndrome. Declaration of personal and funding interests: None.