Clinical Performance Status and Technical Factors Affecting Outcomes from Percutaneous Transhepatic Biliary Interventions; A Multicentre, Prospective, Observational Cohort Study.

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.

Diastolic dysfunction on echocardiography does not predict survival after transjugular intrahepatic portosystemic stent-shunt in patients with cirrhosis.

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.

Outcomes of pregnancy in patients with known Budd-Chiari syndrome.

AIM: To analyse the risk of pregnancy (a prothrombotic state) in patients with Budd-Chiari Syndrome (BCS). METHODS: Retrospective study of pregnancy in women with known BCS at single center from January 2001 to December 2015. RESULTS: Out 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. CONCLUSION: Maternal 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.

Long-term outcomes following percutaneous hepatic vein recanalization for Budd-Chiari syndrome.

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.

Long-term follow-up after endovascular treatment of hepatic venous outflow obstruction following liver transplantation.

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.

Portal vein thrombosis in cirrhosis: Controversies and latest developments.

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.

U.K. guidelines on the management of variceal haemorrhage in cirrhotic patients.

These updated guidelines on the management of variceal haemorrhage have been commissioned by the Clinical Services and Standards Committee (CSSC) of the British Society of Gastroenterology (BSG) under the auspices of the liver section of the BSG. The original guidelines which this document supersedes were written in 2000 and have undergone extensive revision by 13 members of the Guidelines Development Group (GDG). The GDG comprises elected members of the BSG liver section, representation from British Association for the Study of the Liver (BASL) and Liver QuEST, a nursing representative and a patient representative. The quality of evidence and grading of recommendations was appraised using the AGREE II tool.The nature of variceal haemorrhage in cirrhotic patients with its complex range of complications makes rigid guidelines inappropriate. These guidelines deal specifically with the management of varices in patients with cirrhosis under the following subheadings: (1) primary prophylaxis; (2) acute variceal haemorrhage; (3) secondary prophylaxis of variceal haemorrhage; and (4) gastric varices. They are not designed to deal with (1) the management of the underlying liver disease; (2) the management of variceal haemorrhage in children; or (3) variceal haemorrhage from other aetiological conditions.

A case-control study of transjugular intrahepatic portosystemic stent shunts for patients admitted to intensive care following variceal bleeding.

INTRODUCTION: Variceal bleeding has a 6-week mortality of 20%. Recent evidence suggests that early covered transjugular intrahepatic portosystemic stent shunts (TIPSS) can improve outcomes following a variceal bleed in selected patients. We aim to assess the outcomes following the insertion of covered TIPSS in a real-life intensive care setting. MATERIALS AND METHODS: This is a retrospective matched cohort study of all patients referred for TIPSS with variceal bleeding admitted to intensive care (2007-2009). Patients were matched with others admitted to intensive therapy unit following a variceal bleed but did not proceed to TIPSS. All TIPSS procedures were carried out using polytetrafluoroethylene-covered stents. RESULTS: Thirty-eight patients [mean age 55.2 years; mean model for end-stage liver disease (MELD)=14.0; and median follow-up 458 days] were assessed. Nineteen underwent TIPSS and were well matched to the controls. All patients received terlipressin and antibiotics and 86% had active bleeding at endoscopy. Indication for TIPSS was salvage therapy (47%), rebleeding after day 5 (11%) and as secondary prophylaxis (42%). There was 34% all-cause inpatient mortality. The TIPSS group had lower mortality than the non-TIPSS group at 6 weeks (10.5 vs. 47.4%, P<0.05) that persisted at 1 year (21.1 vs. 52.6%, P<0.05). Multivariate analysis indicated MELD [HR 1.131, 95% confidence interval (CI) 1.018-1.257] and TIPSS (HR 0.301, 95% CI 0.091-0.995) as significant predictors of mortality (P<0.05). TIPSS was found to significantly reduce the incidence of failure to control bleeding and rebleeding (HR 0.120, 95% CI 0.015-0.978, P<0.05). CONCLUSION: Patients with recent severe variceal bleeding admitted to intensive care have significantly better outcomes following covered TIPSS insertion. These findings should be validated in randomized-controlled trials.

An update on the diagnosis and management of Budd-Chiari syndrome.

Budd-Chiari syndrome is a rare disorder caused by hepatic venous outflow obstruction and resulting hepatic dysfunction. Despite a lack of prospective randomized trials, much progress has been made in its management over the last 20 years. The main goals of treatment are to ameliorate hepatic congestion and prevent further thrombosis. The selective use of anticoagulation, vascular stents, transjugular intrahepatic portosystemic stent-shunt and liver transplant has resulted in a significant increase in survival. The diagnosis, initial management and long-term follow-up of patients with Budd-Chiari syndrome is reviewed. The concept of individualization of treatment and a stepwise approach to invasive procedures is also discussed.

The role of Transjugular Intrahepatic Portosystemic Stent-Shunt (TIPSS) in the management of variceal hemorrhage.

BACKGROUND: Variceal bleeding in cirrhosis represents a lethal complication of their disease. In the last 20 years, management of AVH has improved greatly with reduction in mortality from 43% in 1980 to 15% in 2000. AIM: Advances in endoscopic therapy, pharmacologic agents including vasoconstrictor therapy and antibiotics have played a large part in improving outcomes, but the role of Transjugular Intrahepatic Portosystemic Stent-Shunt (TIPSS) remains controversial, which this review will cover. METHODS: MEDLINE search for the following terms was performed to July 2011: variceal hemorrhage, portal hypertension, cirrhosis, transjugular intrahepatic portosystemic stent-shunt (TIPSS), PTFE, covered stents. Where possible randomized controlled studies were used for this review, although uncontrolled studies were also included if they made a significant contribution to the literature. RESULTS: Literature used for the present study was selected from a total of 252 publications and abstracts from meetings. RESULTS: TIPSS has been used as a salvage therapy after initial medical and endoscopic therapy for the bleed given its high success rate in arresting uncontrolled variceal bleeding. The recent trial by Garcia- Pagan et al. suggested beneficial effects of an earlier covered TIPSS in those at high risk of treatment failure (Childs C and those who are Childs B with active bleeding). CONCLUSIONS: TIPSS can reduce failure to control bleeding and rebleeding as well as mortality with no increase in the risk of hepatic encephalopathy.This needs to be confirmed in further trials. However, it is clear that prevention of rebleeding is the key to improved outcomes following a variceal bleed.

Presence and severity of non-alcoholic fatty liver disease in a large prospective primary care cohort.

BACKGROUND & AIMS: Non-alcoholic fatty liver disease (NAFLD) is a common cause of abnormal LFTs in primary care, but there are no data defining its contribution nor reporting the range of NAFLD severity in this setting. This study seeks to calculate the range of disease severity of NAFLD in a primary care setting. METHODS: Adult patients with incidental abnormal LFTs, in the absence of a previous history, or current symptoms/signs of liver disease were prospectively recruited from eight primary care practices in Birmingham. NAFLD was diagnosed as fatty liver on ultrasound, negative serological liver aetiology screen, and alcohol consumption ≤30 and ≤20 g/day in males and females, respectively. The NAFLD Fibrosis Score (NFS) was calculated to determine the presence or absence of advanced liver fibrosis in subjects identified with NAFLD. RESULTS: Data from 1118 adult patients were analysed. The cause of abnormal LFTs was identified in 55% (614/1118) of subjects, with NAFLD (26.4%; 295/1118) and alcohol excess (25.3%; 282/1118) accounting for the majority. A high NFS (>0.676) suggesting the presence of advanced liver fibrosis was found in 7.6% of NAFLD subjects, whereas 57.2% of NAFLD patients had a low NFS (<-1.455) allowing advanced fibrosis to be confidently excluded. CONCLUSIONS: NAFLD is the commonest cause of incidental LFT abnormalities in primary care (26.4%), of whom 7.6% have advanced fibrosis as calculated by the NFS. This study is the first of its kind to highlight the burden of NAFLD in primary care and provide data on disease severity in this setting.

Biliary dilatation and strictures after composite liver-small bowel transplantation in children: defining a newly recognized complication.

BACKGROUND: Biliary dilatation and strictures (BDS) are well recognized after liver transplantation but not reported after composite liver-small bowel transplantation (CLSBT). We aimed to describe and propose a classification of BDS in children undergoing CLSBT and analyze the potential risk factors. METHODS: Biliary complications of 47 consecutive children undergoing CLSBT were reviewed and classified according to presentation, location, and intervention required. The following variables were studied: small recipient (weight, <10 kg), donor-recipient weight ratio, liver/bowel graft reduction/not, partial/full pancreas, liver/bowel rejection, and median cold ischemia time (>454 min). RESULTS: Twenty-one (45%) children developed BDS at median 190 days (22 [7-138] months follow-up). Five distinct biliary lesions were identified. Most of the BDS (14/21; 67%) consisted of sphincter dysfunction-related bile duct dilatation (type I), whereas others (7/21; 33%) comprised extrahepatic bile duct (type II; n=3), hilar (type III; n=1), segmental (type IV; n=1), and diffuse (type V; n=2) intrahepatic strictures. None of the graft reduction strategies or other variables studied demonstrated a significant association with BDS. Therapeutic intervention was required in 1 of 14 type I and four of seven type II to V BDS in the form of percutaneous biliary dilatation with or without drainage. CONCLUSION: This article identifies BDS after CLSBT as a frequent late complication after CLSBT, which has a benign outcome in most cases. The natural history of children with extrahepatic and intrahepatic strictures is not yet clear and will need multicenter prospective studies.

A case-controlled study of the safety and efficacy of transjugular intrahepatic portosystemic shunts after liver transplantation.

The role of transjugular intrahepatic portosystemic shunt (TIPS) insertion in managing the complications of portal hypertension is well established, but its utility in patients who have previously undergone liver transplantation is not well documented. Twenty-two orthotopic liver transplantation (OLT) patients and 44 nontransplant patients (matched controls) who underwent TIPS were analyzed. In the OLT patients, the TIPS procedure was performed at a median of 44.8 months (range = 0.3-143 months) after transplantation. Eight (36.4%) had variceal bleeding, and 14 (63.6%) had refractory ascites. The underlying liver disease was cholestatic in 10 (45.4%) and viral in 4 (18.2%). The mean pre-TIPS Model for End-Stage Liver Disease (MELD) score was 13.4 ± 5.1. There were no significant differences in age, sex, indication, etiology, or MELD score with respect to the control group. The mean initial portal pressure gradients (PPGs) were similar in the 2 groups (21.0 versus 22.4 mm Hg for the OLT patients and controls, respectively), but the final PPG was lower in the control group (9.9 versus 6.9 mm Hg, P < 0.05). The rates of both technical success and clinical success were higher in the control group versus the OLT group [95.5% versus 68.2% (P < 0.05) and 93.2% versus 77.2% (P < 0.05), respectively]. The rates of complications and post-TIPS encephalopathy were similar in the 2 groups, and there was a trend toward increased rates of shunt insufficiency in the OLT group. The mortality rate of the patients with a pre-TIPS MELD score > 15 was significantly higher in the OLT group [hazard ratio (HR) = 4.32, 95% confidence interval (CI) = 1.45-12.88, P < 0.05], but the mortality rates of the patients with a pre-TIPS MELD score < 15 were similar in the 2 groups. In the OLT group, the predictors of increased mortality were the pre-TIPS MELD score (HR = 1.161, 95% CI = 1.036-1.305, P < 0.05) and pre-TIPS MELD scores > 15 (HR = 5.846, 95% CI = 1.754-19.485, P < 0.05). In conclusion, TIPS insertion is feasible in transplant recipients, although its efficacy is lower in these patients versus control patients. Outcomes are poor for OLT recipients with a pre-TIPS MELD score > 15.

A reaudit of specialist-managed liver trauma after establishment of regional referral and management guidelines.

BACKGROUND: An earlier liver trauma audit (52 patients) noted that 50% were surgically managed at referring hospitals with a high morbidity and mortality, after which a regional referral and management algorithm was implemented in 2001. This study aims to reaudit specialist-managed liver trauma outcomes. METHODS: Prospective analysis of 99 patients (68 male) treated for liver injury (LI) between 2001 and 2008. Patient characteristics, management, and outcome results of these were compared with the results of previous audit. LI severity was determined by computed tomography, operative findings, and classified according to liver Organ Injury Scale. RESULTS: As implementation of guidelines, referrals increased from 5.2 patients/yr to 14.1 patients/yr, while LI profile was unchanged. Fewer patients were managed surgically with lower surgical intervention at referring hospitals (26 of 52 [50%] vs. 29 of 77 [38%]; p = 0.2). There has been a decrease in liver resection rates (14 of 26 [54%] vs. 3 of 37 [8%]; p = 0.0001]), overall mortality rate (12 of 52 [23%] vs. 11 of 99 [11%]; p = 0.059), and postoperative deaths. CONCLUSION: This reaudit confirms the role of nonoperative management of liver trauma. Early use of computed tomography scan with specialist discussion, selective use of perihepatic packing, and transfer to a specialist unit should be standard practice in the management of complex liver trauma.

Symptomatic portal biliopathy: a single centre experience from the UK.

BACKGROUND AND METHODS: Biliary obstruction as a consequence of portal biliopathy, because of extrahepatic portal vein occlusion is an uncommon cause of biliary disease in the western world. We reviewed all patients presenting to the Regional Liver Transplant Unit in Birmingham, UK with symptomatic portal biliopathy between 1992 and 2005 and report the presentation, investigation, management and outcome of these complex patients. RESULTS: Thirteen patients with symptomatic portal biliopathy were followed up for a median of 2 years (range 1-18 years). Jaundice was the presenting feature in all cases and was associated with bile duct stones or debris in 77% (10 of 13) of cases. Successful treatment of biliary problems was achieved by biliary decompression in six cases (metallic stent=three, plastic stent=one, combined procedure=one and sphincterectomy=one) and portal decompression in three cases (transjugular intrahepatic portosystemic shunt=two, meso-caval shunt=one). Successful biliary drainage could not be achieved endoscopically or by portal decompression in one case that was accepted for combined liver and small bowel transplantation. Three patients had spontaneous resolution without recurrence over the follow-up period. Ten patients (77%) experienced gastrointestinal bleeding. Two deaths over the follow-up period occurred; both were associated with portal hypertensive bleeding. CONCLUSION: Endoscopic management (sphincterectomy and stone extraction or stent insertion) is effective initial therapy for patients with symptomatic portal biliopathy. In the case of persistent biliary obstruction porto-systemic shunting (transjugular intrahepatic portosystemic shunt or surgical) should be considered, however, the extent of vascular thrombosis precludes this in most cases.

A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts.

Previously, we have found that the absence of the colon after liver transplantation (LT) protects the patient from recurrent primary sclerosing cholangitis (rPSC). As our previous observation has not been confirmed in other series, we have reviewed our cohort of patients grafted for primary sclerosing cholangitis (PSC) with greater numbers and longer follow-up to reassess the rate, consequences, and risk factors for rPSC. We collected data on patients who underwent LT for PSC between January 1986 and April 2006. Data were collected for cytomegalovirus status, inflammatory bowel disease status, time of colectomy, type of colectomy, donor-recipient gender mismatch, recipient sex, extended donor criteria (EDC), and donor risk index. Accepted criteria were used to diagnose rPSC. Of a total of 230 consecutive adult patients, 61 (27%) underwent colectomy pre-/peri-LT, and 54 (23.5%) developed rPSC at a median of 4.6 (range, 0.5-12.9) years post-LT. A total of 263 deceased donor grafts were used, and 73 were EDC grafts. A diagnosis of rPSC was made in 61 of the 263 grafts (23%). The recurrence-free patient survival was significantly better (P < 0.05) in patients who underwent pre-/peri-LT colectomy and in those with non-EDC grafts. In conclusion, in this larger cohort of 230 patients and with longer follow-up of 82.5 (range, 0.0-238.6) months [in comparison with the previous report of 152 recipients with a follow-up of 52.8 (range, 1-146) months], we have shown that colectomy remains a significant risk factor for rPSC and that colectomy before and during initial LT for PSC confers a protective effect against rPSC in subsequent graft(s). Moreover, we have shown that EDC grafts are also a significant risk factor for rPSC.

TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients.

BACKGROUND & AIMS: Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder secondary to hepatic venous outflow obstruction. Small series of BCS patients indicate that transjugular intrahepatic portosystemic shunt (TIPS) may be useful. However, the influence of TIPS on patient survival and factors that predict the outcome of TIPS in BCS patients remain unknown. METHODS: One hundred twenty-four consecutive BCS patients treated with TIPS in 6 European centers between July 1993 and March 2006 were followed until death, orthotopic liver transplantation (OLT), or last clinical evaluation. RESULTS: Prior to treatment with TIPS, BCS patients had a high Model of End Stage Liver Disease and high Rotterdam BCS prognostic index (98% of patients at intermediate or high risk) indicating severity of liver dysfunction. However, 1- and 5-year OLT-free survival were 88% and 78%, respectively. In the high-risk patients, 5-year OLT-free survival was much better than that estimated by the Rotterdam BCS index (71% vs 42%, respectively). In the whole population, bilirubin, age, and international normalized ratio for prothrombin time independently predicted 1-year OLT-free survival. A prognostic score with a good discriminative capacity (area under the curve, 0.86) was developed from these variables. Seven out of 8 patients with a score >7 died or underwent transplantation vs 5 out of 114 patients with a score <7. CONCLUSIONS: Long-term outcome for patients with severe BCS treated with TIPS is excellent even in high-risk patients, suggesting that TIPS may improve survival. Furthermore, we identified a small subgroup of BCS patients with poor prognosis despite TIPS who might benefit from early OLT.