Management Of Budd-Chiari Syndrome Research Articles

Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers.

Management of Budd-Chiari syndrome (BCS) has improved over the last decades. The main aim was to evaluate the contemporary post-liver transplant (post-LT) outcomes in Europe. Data from all patients who underwent transplantation from 1976 to 2020 was obtained from the European Liver Transplant Registry (ELTR). Patients < 16 years, with secondary BCS or HCC were excluded. Patient survival (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression analysis identified predictors of PS and GS after 2000. Supplemental data was requested from all ELTR-affiliated centers and received from 44. In all, 808 patients underwent transplantation between 2000 and 2020. One-, 5- and 10-year PS was 84%, 77%, and 68%, and GS was 79%, 70%, and 62%, respectively. Both significantly improved compared to outcomes before 2000 ( p < 0.001). Median follow-up was 50 months and retransplantation rate was 12%. Recipient age (aHR:1.04,95%CI:1.02-1.06) and MELD score (aHR:1.04,95%CI:1.01-1.06), especially above 30, were associated with worse PS, while male sex had better outcomes (aHR:0.63,95%CI:0.41-0.96). Donor age was associated with worse PS (aHR:1.01,95%CI:1.00-1.03) and GS (aHR:1.02,95%CI:1.01-1.03). In 353 patients (44%) with supplemental data, 33% had myeloproliferative neoplasm, 20% underwent TIPS pre-LT, and 85% used anticoagulation post-LT. Post-LT anticoagulation was associated with improved PS (aHR:0.29,95%CI:0.16-0.54) and GS (aHR:0.48,95%CI:0.29-0.81). Hepatic artery thrombosis and portal vein thrombosis (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). LT for BCS results in excellent patient- and graft-survival. Older recipient or donor age and higher MELD are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.

Accessory Hepatic Vein Stenting in the Management of Budd-Chiari Syndrome: A Case Report

The Budd-Chiari Syndrome (BCS) is associated with hepatic venous outflow obstruction. A 22-year-old female patient presented with abdominal pain and backache for 12 days, along with generalised weakness. Physical examination revealed pitting pedal oedema and abdominal distension. An abdominal ultrasound with Doppler Ultrasonography (USG) was performed, revealing liver parenchymal disease with gross ascites and occlusion of all three Hepatic Veins (HV), suggestive of BCS. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) of the liver, as well as a right hepatic venogram using Digital Subtraction Angiography (DSA), showed chronic total occlusion at the ostium. An Accessory Hepatic Vein (AHV) was identified, joining the Right Hepatic Vein (RHV) to the Inferior Vena Cava (IVC). The accessory RHV exhibited high-grade stenosis (approximately 90-95%) at its junction with the IVC. Recanalisation of the AHV (balloon dilatation/stent insertion) was performed. Post-procedure accessory hepatic venogram showed a patent stented segment with a significant reduction in adjacent venous collaterals. No procedure-related complications were observed. The present case highlights the importance of AHV stenting in BCS patients, as it helps maintain normal physiology, in contrast to Direct Intrahepatic Porto-systemic Shunt (DIPS), which alters normal physiology by allowing portal venous blood to mix directly into the systemic circulation, bypassing the liver parenchyma.

Abstract No. 595 Outcome of various endovascular procedures in management of Budd-Chiari syndrome

Abstract No. 595 Outcome of various endovascular procedures in management of Budd-Chiari syndrome

The Role of a Medical Intermediate Care Unit in the Management of Budd-Chiari Syndrome: Case Series

Budd-Chiari syndrome (BCS) has a wide spectrum of presentations, from an asymptomatic status to acute liver failure (ALF). The therapeutic approach depends on disease severity and related etiology with patients with severe forms of presentation classically managed in intensive care units (ICUs). Here, we report a series of five BCS patients managed in a medical intermediate care unit (IntCU), with three of them presenting with acute liver injury. Progression to ALF was seen in three patients, two of whom died, with one being successfully submitted to liver transplantation. IntCUs allow a 24-h patient surveillance and a prompt management of BCS, with less economic impact when compared to ICUs. Mortality was related to the presence of associated comorbidities that limited therapeutic approach. J Med Cases. 2021;12(5):190-194 doi: https://doi.org/10.14740/jmc3659

Controversies in Budd-Chiari Syndrome Management: Potential Role of Liver Stiffness

Controversies in Budd-Chiari Syndrome Management: Potential Role of Liver Stiffness

Dabigatran as an oral anticoagulant in patients with Budd–Chiari syndrome post‐percutaneous endovascular intervention

Anticoagulants play an important role in the management of Budd-Chiari syndrome. There is a paucity of data on the efficacy and safety of direct-acting oral anticoagulants-dabigatran, among patients with Budd-Chiari syndrome. In a retrospective analysis of prospectively maintained data, the stent patency rates, major bleeding episode, and a composite endpoint of major bleed and/or mortality rates were compared between Budd-Chiari syndrome patients treated with dabigatran (n=36) or vitamin K antagonists (n=62) following endovascular intervention. The baseline characteristics, including sites of block and types of interventions, were similar between the two groups. The mean duration of follow-up in the dabigatran and vitamin K antagonist groups was 10.5±6.7 and 14.1±6.9months (P=0.006), respectively. The endovascular stent patency rates were comparable between the dabigatran and vitamin K antagonist groups at 6months (91% vs 96.5%) and 12months (91% vs 93%), P=0.296 (log-rank test), respectively. Major bleeding events were comparable between the dabigatran and vitamin K antagonist groups at 6months (3.5% vs 2%) and 12months (3.5% vs 6.5%), P=0.895 (log-rank test), respectively. The composite endpoint of mortality and major bleed was comparable between dabigatran and vitamin K antagonists at 6months (4% vs 5%) and 12months (4% vs 8%), P=0.875 (log-rank test), respectively. Dabigatran, as compared with vitamin K antagonists, is associated with similar stent patency rates and complications among patients with Budd-Chiari syndrome post-endovascular intervention.

Milestones in the discovery of Budd-Chiari syndrome.

In 1845, George Budd published a brief report regarding three patients who developed an obstruction of the hepatic veins. The condition has never been reported before, and was related to sepsis and alcoholism. Fifty-three years later, Hans Chiari postulated that syphilis was causing the obstruction of the hepatic veins, and enriched the debate with clinical and pathological correlations. Following the hypothesis on the 'phlebitis obliterans', several authors proposed other pathophysiological explanations including congenital causes, chronic trauma and exogenous toxins. RG Parker, in 1959, first recognized the relationship between obstruction of hepatic veins and thrombophilic conditions such as polycythaemia vera, pregnancy and hormonal therapy. Based on that, anticoagulant treatment was attempted, but with unsatisfactory outcome. We need to wait until the mid 1980s to see a widespread adoption of anticoagulants, with a consequent improvement of patients' survival. The fear of haemorrhagic events in patients with liver disease discouraged this therapeutic approach, and other surgical interventions (mainly port-systemic shunts) were conceived, but with high morbidity and mortality. The first liver transplantation in 1976 and the first trans-jugular intra-hepatic porto-systemic shunt in 1993 represented two major cornerstones in the management of Budd-Chiari syndrome (BCS). Such progresses allowed modifying the treatment of BCS until the modern concept of stepwise therapy. The present review thoroughly reviews the major landmarks in the discovery, treatment and clinical management of patients with BCS.

Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome.

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.

Long Term Survival of Patients Undergoing TIPS in Budd-Chiari Syndrome

There has been significant improvement in understanding the etiology and management of Budd-Chiari Syndrome (BCS). Patients with chronic or acute-on-chronic BCS need radiological interventions in the form of angioplasty, hepatic vein/inferior vena cava stenting or Transjugular Intrahepatic Portosystemic Shunt (TIPS). Data regarding the long term follow up of patients undergoing TIPS is limited. We thus prospectively followed-up BCS patients who underwent TIPS at our center. This study included 42 patients with BCS who underwent TIPS with a covered stent between 2004 and 2014. We analyzed the etiology, symptoms, severity, laboratory parameters and imaging pre and post TIPS. All patients underwent surveillance for hepatocellular carcinoma. Patients demographics included 26 males and 16 females with a mean age of 40.5 years (19-68 years). The mean Model for End-Stage Liver Disease score of the entire cohort was 15.38 (range: 9-25). Thirty-four patients were grouped into Rotterdam Class 2 and remaining into Class 3. There was significant improvement in ascites, gastrointestinal bleed, renal function and transaminase levels post TIPS. There were 11 deaths over the follow-up period - 4 within one month, 2 within six months and the rest after 3 years following TIPS. Median duration from clinical presentation to TIPS was 2.1 weeks and median survival till follow-up was 45.5 months (0-130 months). 33/42 patients underwent TIPS prior to 2013, and their median survival till follow-up was 55 months. Six out of eleven deaths that occurred within six months post-TIPS were before 2006; when the technique of TIPS creation was evolving. The cumulative 1 year, 5 years and 10 years OLT-free survival was 86%, 81% and 76%, respectively. Two patients underwent a liver transplant at 4 and 7 years after TIPS. Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, the requirement for liver transplantation has become rare.

Endovascular treatment of Budd-Chiari syndrome: Single center experience.

Percutaneous radiologic interventions are increasingly being used in management of Budd-Chiari syndrome (BCS). Minimal invasive approach has resulted in excellent long-term outcomes. We evaluated the treatment efficacy and safety of radiological intervention in patients with BCS. Between January 2008 and June 2014, 190 patients with BCS underwent endovascular procedures (hepatic vein, collateral vein or inferior vena cava [IVC] plasty with or without stenting, or transjugular intrahepatic portosystemic shunting [TIPSS]). Clinical features, biochemical profile, and stent patency were monitored pre-procedure and post-procedure, and for a median duration of 42 (12-88) months. Of 190 patients (mean [SD] age = 26.9 [11.5] years; 102 men), imaging revealed hepatic vein obstruction in 147 patients, IVC obstruction in 40 patients, and concomitant hepatic vein and IVC obstruction in three patients. At presentation, the radiological interventions included hepatic vein plasty/stenting in 38 patients, collateral vein stenting in three patients, IVC plasty/stenting in 40 patients, both IVC and hepatic vein stenting in three patients, and TIPSS in 106 patients. Response was seen in 153 patients (80.5%). Repeat interventions were required in 19 patients (10.0%). Complications were noted in nine patients (4.7%). Our study demonstrates that venous recanalization and TIPSS for BCS are safe and efficacious.

Successful local thrombolytic therapy in subacute budd-chiari syndrome: Case report and review

Introduction: Management of Budd-Chiari syndrome (BCS) includes different interventions and surgical procedures. There is limited data regarding catheterdirected thrombolysis when treating this condition but it appears to be helpful in the case illustrated below. Case report: A 29 year-old woman was referred to our center with one month history of right upper abdominal pain, progressive abdominal distension and intermittent fever not associated with rigors. There was no history of oral contraceptive use. She had mild right upper quadrant tenderness and abdominal distention with moderate elevation of liver enzymes. Her serology was negative for viral hepatitis, autoimmune or cholestatic liver disease. Computed tomography (CT) angiogram of the abdomen showed a large amount of ascites with extensive thrombosis of the inferior vena cava (IVC) involving the hepatic and left renal veins. There was also complete occlusion of the left common iliac vein confirmed by venogram. An infusion catheter was placed through the thrombosed segment of the IVC and right hepatic artery. Thrombolytic therapy was started with the injection of 5 mg of recombinant tissue plasminogen activator (t-PA) as a loading dose followed by 0.3 mg per hour. Enoxaparin and oral warfarin were started simultaneously and once the target INR was reached, enoxaparin was stopped and warfarin continued indefinitely. Ascites was well controlled with diuretics and large-volume paracentesis. A follow up venogram showed partial recanalization of IVC and hepatic veins. A repeat CT scan after 14 weeks showed complete resolution of the thrombus. After 28 months, she is asymptomatic with normal liver function tests and total resolution of the ascites. Conclusions: The data on local thrombolysis is limited and the agents and doses are not uniform among reported cases. This case report shows that it can be considered in acute BCS with partial obstruction, followed by angioplasty or TIPS if unsuccessful.

Retrograde puncture assisted hepatic vein recanalization in treating Budd-Chiari syndrome with segmental obstruction of hepatic vein.

This study aimed at investigating the feasibility and effectiveness of retrograde puncture assisted hepatic vein (HV) recanalization in management of Budd-Chiari syndrome (BCS) patients with segmental obstruction of HV. From May 2011 to August 2014, 76 BCS patients with obstruction of HV were treated by routine transjugular HV recanalization in our center. Among them, 17 patients with segmental obstruction (obstruction length >1cm) of HV experienced failure of the routine transjugular HV recanalization and underwent retrograde puncture assisted HV recanalization. Data on technical success, clinical success and follow-up were collected and analyzed retrospectively. Retrograde puncture assisted HV recanalization was technically successful in 14 of 17 (82%) patients. Of these 14 patients, 12 patients underwent HV balloon dilation, and 2 patients underwent HV stent insertion. No procedure-related complications occurred in any of our patient. Clinical success was achieved in all of the 14 patients who experienced technical success. The mean HV pressure decreased from 43.6±6.7 cmH2O before treatment to 18.4±4.8 cmH2O after treatment (P<0.001). BCS-related symptoms began to improve on the next day following the treatment. During 4-43months (mean 17.4±10.8months) of follow-up, three patients experienced re-obstruction of HV. Retrograde puncture assisted HV recanalization is a simple, safe, and effective treatment for BCS patients with segmental obstruction of HV. It can serve as an additional treatment option for patients who experience the technical failure of routine transjugular HV recanalization.

Diagnosis and management of Budd Chiari syndrome: an update.

Imaging plays a crucial role in the early detection and assessment of the extent of disease in Budd Chiari syndrome (BCS). Early diagnosis and intervention to mitigate hepatic congestion is vital to restoring hepatic function and alleviating portal hypertension. Interventional radiology serves a key role in the management of these patients. The interventionist should be knowledgeable of the clinical presentation as well as key imaging findings, which often dictate the approach to treatment. This article concisely reviews the etiology, pathophysiology, and clinical presentation of BCS and provides a detailed description of imaging and treatment options, particularly interventional management.

Budd-chiari syndrome management: Timing of treatment is an open issue

Budd-chiari syndrome management: Timing of treatment is an open issue

Management of Budd Chiari Syndrome by liver transplantation: A case report

Management of Budd Chiari Syndrome by liver transplantation: A case report

Budd-Chiari syndrome management: Lights and shadows

Budd-Chiari syndrome (BCS) is a rare disease whose management should follow a step by step strategy. Anticoagulation and medical therapy should be the first line treatment. Revascularization or TIPS are indicated in case of no response to medical therapy. OLT should be indicated as a rescue therapy and anticoagulation be started soon after OLT. However, no clear indication can actually be given about the timing of different treatments. Moreover, there is some concern about treatment of some subgroup of patients, especially regarding the risk of recurrence after liver transplantation. The topic of this paper is to critically review the actual knowledge of BCS management.

The Role of Intravascular Intervention in the Management of Budd-Chiari Syndrome

To evaluate the efficacy of intravascular intervention in the management of different types of Budd-Chiari syndrome. Fifty-three patients of BCS were clinically diagnosed and interventionally treated in terms of their signs and symptoms of portal hypertension and occlusive inferior vena cava/or hepatic veins with the combination of Doppler ultrasonography, CT scan, and angiography. The interventional methods applied in this study included percutaneous transluminal angioplasty and IVC stent implantation (PTA+IVC stent); transjugular hepatic veno-stent placement (PTA+HV stent) or transjugular transluminal hepatic veno-inferior vena cava stent placement and transcaval transjugular intrahepatic portocaval shunt. The success rate of intravascular interventional therapy was 92.45% (49/53). After interventional therapy, the patients' pleural effusion, ascites, prominent veins formation of bilateral flanks or backs alleviated, hepatomegaly reduced, and the urinary output increased. The longest follow-up case was 13 years with patent stent. Two patients died of pulmonary embolization or pericardial tamponade during surgery. Intravascular intervention is a safe and effective therapy for most types of BCS.

Feasibility and midterm outcomes of percutaneous transhepatic balloon angioplasty for symptomatic Budd-Chiari syndrome secondary to hepatic venous obstruction

This study evaluated the feasibility and outcomes of percutaneous transhepatic balloon angioplasty (PTBA) of the hepatic vein in the management of Budd-Chiari syndrome (BCS) secondary to hepatic venous outflow obstruction. From September 1996 to October 2008, 101 patients (52 males, 49 females) with BCS secondary to occlusion of the hepatic veins were prospectively treated using PTBA of the hepatic vein. Average age was 31.3 years (range, 15-57 years). Nineteen had concurrent inferior vena cava (IVC) obstruction. All the patients presented with symptomatic portal hypertension. PTBA, with or without stenting, was performed after hepatovenography. PTBA was successfully performed in 92 of the 101 patients. Sixty-eight patients underwent PTBA of right hepatic vein, followed by stent placement in two. PTBA was performed in 11 patients with left hepatic vein occlusion and in 13 patients with dominant accessory hepatic vein occlusion. The technical success rate was 92 of 101 (91%). Hepatic venous pressure was significantly decreased after balloon angioplasty/stenting (P < .01, paired t test). Symptoms were significantly improved in the 92 patients who had successful PTBA. Three patients had acute hepatic vein thrombosis during or after PTBA. Two patients sustained intraperitoneal bleeding from the transhepatic puncture track, and one had intrahepatic hematoma. Pulmonary embolism developed in one patient during the operation. All complications were managed nonoperatively. There were no perioperative deaths. Within 1 year, 74 of the 101 patients returned for follow-up, and 51 patients had follow-up at 2 years. The primary patency rates were 84% (62 of 74), 78% (58 of 74), and 76% (39 or 51) at 6, 12, and 24 months after PTBA, respectively. The secondary patency rates were 95% (70 of 74), 92% (68 of 74), and 84% (43 of 51) at 6, 12, and 24 months. PTBA of the hepatic vein is a safe and effective treatment of BCS. It is currently the most physiologic procedure, and the risk of postoperative encephalopathy is minimized because portal flow is not diverted. Midterm outcomes are satisfactory. Further investigation of the long-term outcomes is needed.

Liver Transplantation With Reconstruction of the Inferior Vena Cava for Hepatocarcinoma on Chronic Budd-Chiari: A Case Report

Management of Budd-Chiari syndrome, from simple medical treatment to liver transplantation, depends on the acute and chronic evolution of the disease and on the degree of hepatic insufficiency. Herein we have reported the case of a man who underwent transplantation after evolution of a Budd-Chiari syndrome with membranous obstruction of the vena cava and developed 2 lesions of hepatocellular carcinoma. Surgery was difficult due to previous procedures requiring reconstruction of the supra-hepatic vena cava. This case emphasized the timing of liver transplantation versus other treatments to decrease the operative risk.

Interventional Radiology in the Management of Budd Chiari Syndrome

Budd Chiari syndrome is an uncommon condition in the Western world but interventional radiology can contribute significantly to the management of the majority of patients. This review examines the role and technique of interventions including hepatic vein dilatation and stent insertion as well as thrombolysis and TIPS. Liver transplantation and surgical shunt surgery are discussed in relation to radiological interventions. With appropriate selection and technique, surgery is only required in a minority of patients.