Budd-Chiari syndrome is a complex clinical disorder of hepatic venous outflow obstruction, originating from the accessory hepatic vein (HV), large HV, and suprahepatic inferior vena cava (IVC). This disorder includes both HV and IVC obstructions and hepatopathy. This study aimed to conduct a systematic review of the survival rate and clinical success of different types of endovascular treatments for Budd-Chiari syndrome (BCS). All participant studies were retrieved from four databases and selected according to the eligibility criteria for systematic review of patients with BCS. The survival rate, clinical success of endovascular treatments in BCS, and survival rates at 1 and 5 years of publication year were calculated accordingly. A total of 3398 patients underwent an endovascular operation; among them, 93.6% showed clinical improvement after initial endovascular treatment. The median clinical success rates for recanalization, transjugular intrahepatic portosystemic shunt (TIPS), and combined procedures were 51%, 17.50%, and 52.50%, respectively. The median survival rates at 1 and 5 years were 51% and 51% for recanalization, 17.50% and 16% for TIPS, and 52.50% and 49.50% for combined treatment, respectively. Based on the year of publication, the median survival rates at 1 and 5 years were 23.50% and 22.50% before 2000, 41% and 41% in 2000‒2005, 35% and 35% in 2006‒2010, 51% and 48.50% in 2010‒2015, and 56% and 55.50% after 2015, respectively. Our findings indicate that the median survival rate at 1 and 5 years of recanalization treatment is higher than that of TIPS treatment, and recanalization provides better clinical improvement. The publication year findings strongly suggest progressive improvements in interventional endovascular therapy for BCS. Thus, interventional therapy restoring the physiologic hepatic venous outflow of the liver can be considered as the treatment of choice for patients with BCS which is a physiological modification procedure.

To assess the critical role of transjugular intrahepatic portosystemic shunt (TIPS) in the management of Budd-Chiari syndrome (BCS), as the data with respect to the safety and outcome of TIPS in patients with BCS are scarce because of the rarity of the disease.

A comprehensive search of literature of various databases from 2000 to October 2021 was conducted for studies evaluating the outcome of TIPS in patients with BCS. The primary outcomes of the analysis were technical and clinical success, adverse events and mortality associated with TIPS, dysfunction of TIPS, need for TIPS revision, need for liver transplantation (LT), and 1-year survival.

A total of 33 studies (1,395 patients) were included in this meta-analysis. The pooled rates and 95% confidence intervals of various outcomes were 98.6% (97.6-99.7) for technical success, 90.3% (86.0-94.6) for clinical success, 10.0% (6.5-13.6) for major adverse events, 0.5% (0.2-1.0) for TIPS-related mortality, 11.6% (7.8-15.4) for post-TIPS hepatic encephalopathy (HE), 40.1% (32.5-47.7) for TIPS dysfunction, 8.6% (4.9-12.4) for the need for TIPS revision, 4.5% (2.8-6.2) for the need for LT, and 94.6% (93.1-96.1) for 1-year survival. Publication bias was seen with all outcomes except for post-TIPS HE, TIPS dysfunction, and the need for LT.

The existing literature supports the feasibility, safety, and efficacy of TIPS in the treatment of BCS. Deciding the optimal timing of TIPS in BCS needs further studies.

Budd Chiari syndrome (BCS) is a diverse disease with regard to the site of obstruction, the predisposing thrombophilic disorders and clinical presentation across the Asia-Pacific region. The hepatic vein ostial stenosis and short segment thrombosis are common in some parts of Asia-Pacific region, while membranous obstruction of the vena cava is common in some and complete thrombosis of hepatic veins in others. Prevalence of myeloproliferative neoplasms and other thrombophilic disorders in BCS varies from region to region and with different sites of obstruction. This heterogeneity also raises several issues and dilemmas in evaluation and approach to management of a patient with BCS. The opportunity to recanalize hepatic vein in patients with hepatic vein ostial stenosis or inferior vena cava stenting or pasty among those membranous obstruction of the vena cava is a unique opportunity in the Asia-Pacific region to restore hepatic outflow closely mimicking physiology. In order to address these issues arising out of the diversity as well as the unique features in the region, the Asia Pacific Association for Study of Liver has formulated these guidelines for clinicians.

Budd-Chiari syndrome (BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt (TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.

Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare cause of liver disease that should not be missed. Variable clinical presentation among patients with BCS necessitates a high index of suspicion to avoid missing this life-threatening diagnosis. BCS is characterized as primary or secondary, depending on etiology of venous obstruction. Most patients with primary BCS have several contributing risk factors leading to a prothrombotic state. A multidisciplinary stepwise approach is integral in treating BCS. Lifelong anticoagulation is recommended. Long-term monitoring of patients for development of cirrhosis, complications of portal hypertension, hepatocellular carcinoma, and progression of underlying diseases is important.

These guidelines on transjugular intrahepatic portosystemic stent-shunt (TIPSS) in the management of portal hypertension 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 guidelines are new and have been produced in collaboration with the British Society of Interventional Radiology (BSIR) and British Association of the Study of the Liver (BASL). The guidelines development group comprises elected members of the BSG Liver Section, representation from BASL, a nursing representative and two patient representatives. The quality of evidence and grading of recommendations was appraised using the GRADE system. These guidelines are aimed at healthcare professionals considering referring a patient for a TIPSS. They comprise the following subheadings: indications; patient selection; procedural details; complications; and research agenda. They are not designed to address: the management of the underlying liver disease; the role of TIPSS in children; or complex technical and procedural aspects of TIPSS.

Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract.

To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS.

Analysis of recent literature by using Medline, PubMed and EMBASE databases.

Primary BCS is usually caused by thrombosis and is further classified into "classical BCS" type where obstruction occurs within the hepatic vein and "hepatic vena cava BCS" which involves thrombosis of the intra/suprahepatic portion of the inferior vena cava (IVC). BCS patients often have a combination of prothrombotic risk factors. Aetiology and presentation differ between Western and certain Asian countries. Myeloproliferative neoplasms are present in 35%-50% of European patients and are usually associated with the JAK2-V617F mutation. Clinical presentation is diverse and BCS should be excluded in any patient with acute or chronic liver disease. Non-invasive imaging (Doppler ultrasound, computed tomography, or magnetic resonance imaging) usually provides the diagnosis. Liver biopsy should be obtained if small vessel BCS is suspected. Stepwise management strategy includes anticoagulation, treatment of identified prothrombotic risk factors, percutaneous revascularisation and transjugular intrahepatic portosystemic stent shunt to re-establish hepatic venous drainage, and liver transplantation in unresponsive patients. This strategy provides a 5-year survival rate of nearly 90%. Long-term outcome is influenced by any underlying haematological condition and development of hepatocellular carcinoma.

With the advent of newer treatment strategies and improved understanding of BCS, outcomes in this rare disease have improved over the last three decades. An underlying haematological disorder can be the major determinant of outcome.

This study aimed to evaluate the long-term efficacy and safety of percutaneous transhepatic balloon angioplasty (PTBA) and transjugular intrahepatic portosystemic stent-shunt (TIPSS) in the treatment of Budd-Chiari syndrome (BCS) with hepatic veins involvement. Between June 2008 and August 2016, a total of 60 BCS patients with hepatic vein involvement in our department were enrolled in this study. Thirty-three cases underwent hepatic vein balloon angioplasty in PTBA Group and 27 cases underwent TIPSS. Data were retrospectively collected, and follow-up observations were performed. TIPSS Group showed significantly higher thrombotic/segmental obstruction and peripheral stenosis/obstruction compared with PTBA Group. The success rates were 93.9% and 100.0% in PTBA Group and TIPSS Group, respectively. The mean portal vein pressure decreased significantly after stenting. Except for 1 patient died from repeated hemorrhage, other sever complications had not been observed in both group. Twenty-six patients and 21 patients were clinically cured in PTBA Group and TIPSS Group, respectively. The primary patency rates were 89.7%, 79.3%, and 79.3% for short-term, mid-term and longterm in PTBA Group, which were significantly higher than TIPSS Group for long-term follow up. The second patency rates were 100.0%, 96.6% and 96.6% for short-term, mid-term and long-term in PTBA Group, which were similar to TIPSS Group (P = 1.0000). In conclusion, PTBA and TIPSS are safe and effective in the treatment of BCS with hepatic veins involvement, with an excellent long-term patency rate of hepatic vein and TIPSS shunt. TIPSS can be used to treat patients with all 3 hepatic veins lesion and failure PTBA.

To evaluate the safety and efficacy of percutaneous transhepatic route creation for hepatic vein (HV) angioplasty in Budd-Chiari syndrome (BCS).

Between April 2012 and August 2016, a total of 19 BCS patients underwent percutaneous transhepatic route creation for HV angioplasty after transvenous catheterization failure in this study. Color Doppler ultrasonography was required in all patients after admission and during follow-up. Data were retrospectively collected, and follow-up observations were performed.

Technical and clinical success was achieved in 18 patients. Except for 1 failure of route creation, 19 routes were successfully created in 18 patients, with a technology success rate of 95.0%. Twenty-two balloon angioplasties were performed in 18 patients, with a mean balloon diameter of 13.6 ± 0.5 mm. Blood pressure and length of occlusive HV decreased significantly, and blood flow velocity and diameter of HV increased significantly after procedure. Abdominal distension/pain and ascites decreased significantly after procedure. One procedure-related death occurred, who died of gastrointestinal bleeding 6 days later. Except for the failure case, the rest of 18 patients were successfully followed up. The remaining 18 patients survived during follow-up, with a 5-year survival rate of 94.9%. One patient had a restenosis of HV after 47 days, and had undergone successful dilation. The 5-year primary and second patency rates were 94.1% and 100%, respectively.

Percutaneous transhepatic route creation is safe and effective for HV angioplasty, and can be used to treat BCS patients after transvenous catheterization failure.

We aimed to investigate the feasibility and effectiveness of accessory hepatic vein recanalization (balloon dilatation/stent insertion) for patients with Budd-Chiari syndrome (BCS) due to long-segment obstruction of the hepatic vein.

From March 2010 to December 2013, 20 consecutive patients with BCS, due to long-segment obstruction of three hepatic veins, treated with accessory hepatic vein recanalization (11 males, 9 females; mean age, 33.4±10.9 years; range, 22-56 years) were included in this retrospective study. Data on technical success, clinical success, and follow-up were collected and analyzed.

Technical and clinical success was achieved in all patients. Each patient was managed with a single accessory hepatic vein recanalization procedure. No procedure-related complications occurred. The diameter of the accessory hepatic vein was 8.45±1.47 mm (6-11 mm) at the stem, and there were many collateral circulations between the hepatic vein and the accessory hepatic vein. The mean pressure of accessory hepatic vein decreased from 47.50±5.59 cm H2O before treatment to 28.80±3.47 cm H2O after treatment (P < 0.001). Abnormal levels of total bilirubin, albumin, aspartate aminotransferase, and alanine transaminase improved after the treatment. During the follow-up, three patients experienced restenosis or stenting of the accessory hepatic vein.

In BCS due to long-segment obstruction of the hepatic veins, it is important to confirm whether there is a compensatory accessory hepatic vein. For patients with a compensatory but obstructed accessory hepatic vein, recanalization is a simple, safe, and effective treatment option.

Treatment of Budd-Chiari syndrome (BCS) has shifted from mainly medical treatment, with surgical shunt and orthotopic liver transplantation (OLT) as rescue, to medical treatment combined with an early endovascular intervention in the past two decades.

To assess the safety and efficiency of endovascular treatment of symptomatic patients with BCS and to compare mortality with symptomatic BCS patients in the same region treated with only sporadic endovascular techniques.

This was a retrospective review of clinical data, treatment and survival in 14 patients diagnosed with BCS and treated with endovascular methods from 2003 to 2015. A national epidemiology study of BCS from 1986 to 2003 was used for comparison.

Thirteen of the 14 patients eventually had transjugular intrahepatic portosystemic shunt (TIPS), four after previous liver vein angioplasty. TIPS were performed with polytetrafluoroethylene-covered stents and technical success was 100%. Calculated preinterventional prognostic indices indicated a high risk of TIPS dysfunction, OLT and death. However, only one patient died and one had an OLT, and the 1- and 2-year primary TIPS-patency was 85 and 67%, respectively. Episodes of de-novo hepatic encephalopathy occurred in three patients. Overall 1- and 5-year transplantation-free survival was 100 and 93% compared with 47 and 28%, respectively, in 1986 to 2003.

TIPS seems to be a safe and effective treatment for symptomatic BCS and there is an obvious improvement in transplantation-free survival compared with conservatory medical treatment. It should, therefore, be considered early, as first-line intervention, in patients with insufficient response to medical treatment.

In Western countries, transjugular intrahepatic portosytemic shunt (TIPS) is widely applied for the treatment of Budd-Chiari syndrome (BCS). However, the outcome of Chinese BCS patients treated with TIPS is extremely limited. Furthermore, the timing of conversion from percutaneous recanalization to TIPS remains uncertain.

All consecutive BCS patients treated with TIPS between December 2004 and June 2012 were included. Patients were classified as the early and converted TIPS groups. Indications, TIPS-related complications, post-TIPS hepatic encephalopathy, shunt dysfunction and death were reported.

Of 51 patients included, 39 underwent percutaneous recanalization for 1024 days (0-4574) before TIPS. Early TIPS group (n = 19) has a shorter history of BCS and a lower proportion of prior percutaneous recanalization than converted TIPS group (n = 32). Main indications were diffuse obstruction of three HVs (n = 12), liver failure (n = 2), liver function deterioration (n = 8), refractory ascites (n = 10) and variceal bleeding (n = 19). Procedure-related intraperitoneal bleeding was reversible in three patients. The cumulative 1-year rate of being free of first episode of post-TIPS hepatic encephalopathy and shunt dysfunction was 78.38 and 61.69% respectively. The cumulative 1-, 2-, and 3-year survival rates were 83.82, 81.20 and 76.93% respectively. BCS-TIPS score, but not Child-Pugh, MELD, Clichy or Rotterdam score, could predict the survival. Age, total bilirubin and inferior vena cava thrombosis were also significantly associated with overall survival. Survival was similar between early and converted TIPS groups.

TIPS can achieve an excellent survival in Chinese patients in whom percutaneous recanalization is ineffective or inappropriate. BCS-TIPS score could effectively predict these patients' survival.