Good Clinical Outcomes in Budd–Chiari Syndrome with Hepatic Vein Occlusion

Fan, Xinxin; Liu, Kai; Che, Yuan; Wang, Shi-Kai; Wu, Xiaohu; Cao, Jiashu; Li, Jieshou

doi:10.1007/s10620-016-4208-0

Cited by 24 publications

(9 citation statements)

References 27 publications

(29 reference statements)

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“…After successful IVC recanalization, TIPS was created for selected aBCS Type Ic patients. The details of TIPS procedures have been previously described . In brief, based on an indirect portovenogram, portal vein access was obtained between the IVC and the portal vein using Rosch‐Uchida system (Cook, Bloomington, IN, US).…”

Section: Methodssupporting

confidence: 92%

Long‐term outcomes of individualized treatment strategy in treatment of type I Budd‐Chiari syndrome in 456 patients

Ding

Han

Liu

et al. 2019

Liver International

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Abbreviations: BCS, Budd-Chiari syndrome; HV, hepatic vein; ITS, individualized treatment strategy; IVC, inferior vena cava; MELD score, model for end-stage liver disease score; MOVC, membranous obstruction of the inferior vena cava; PTA, percutaneous transluminal balloon angioplasty; SOVC, segmental obstruction of the inferior vena cava; TIPS, transjugular intrahepatic portosystemic shunt.Abstract Aim: To evaluate individualized treatment strategy (ITS) and long-term outcomes of endovascular treatment of Budd-Chiari syndrome (BCS) with obstructed inferior vena cava (IVC) based on different degrees of hepatic vein (HV) involvement. , 456 consecutive patients with BCS with obstructed IVC underwent endovascular treatment with ITS. All patients received IVC recanalization. Then, 426 patients with at least one patent HV received no additional treatment. Twenty-fivepatients with membranous or segmental occlusion of HVs underwent HV recanalization and for the remaining five patients with diffuse HVs occlusion, a transjugular intrahepatic portosystemic shunt (TIPS) was performed.Results: The endovascular treatment was technically successful in 455 of the 456 patients (99.8%). The complication rate was 5.0% (23/456), with major complications in 13 patients (2.8%) and minor complications in 10 patients (2.2%). Median follow-up time was 60.5 months (range, 4-120 months). The cumulative 1-, 2-, 5-and 10-year primary vessel patency rates were 93.6%, 89.9%, 80.5% and 74.3% respectively and the cumulative 1-, 2-, 5-, 10-year secondary patency rates were 99.8%, 99.8%, 98.2% and 97.2% respectively. The cumulative 1-, 2-, 5-and 10-year survival rates were 98.4%, 95.8%, 91.2% and 76.5% respectively. Illness duration and decreased serum albumin were independent predictors of survival. Conclusion:The ITS for Asian BCS with obstructed IVC and varying degrees of HV involvement appears to be effective and with good long-term outcomes. K E Y W O R D Sangioplasty, Budd-Chiari syndrome, hepatic vein, inferior vena cava

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Section: Methodssupporting

confidence: 92%

Long‐term outcomes of individualized treatment strategy in treatment of type I Budd‐Chiari syndrome in 456 patients

Ding

Han

Liu

et al. 2019

Liver International

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“…HVC‐BCS patients have undergone percutaneous recanalisation more frequently as compared to classical BCS patients . Several studies have shown good long‐term efficacy and survival‐benefit of this procedure (Table ) …”

Section: Radiological Interventionmentioning

confidence: 99%

“…Angioplasty has been extensively used in Asia with good long‐term outcomes (Table ) . Han et al published their experience in 168 Chinese BCS patients undergoing successful percutaneous angioplasty with median follow‐up of 30 months .…”

Section: Radiological Interventionmentioning

confidence: 99%

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

Khan

Armstrong

Mehrzad

et al. 2019

Aliment Pharmacol Ther

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Background: Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract. Aim: To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS. Methods: Analysis of recent literature by using Medline, PubMed and EMBASE databases. Results: 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. Conclusions: 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. Primary BCS is considered a multifactorial disease and multicentre data found a combination of several prothrombotic conditions in 25% to 46% of the patients with BCS, 11,19-21 several times greater than expected in the general population. The discovery of one causal factor should not discourage further investigation to identify other prothrombotic conditions. Multifactorial causes may explain the rarity of BCS. 2 The prothrombotic conditions found in BCS, in particular the classical type, include: Factor V Leiden mutation, prothrombin (PT) gene mutation, protein C deficiency, protein S deficiency, antithrombin deficiency, antiphospholipid syndrome, hyperhomocysteinemia and paroxysmal nocturnal haemoglobinuria. 22 BCS is also associated with systemic inflammatory diseases, such as Behçet's disease, sarcoidosis, vasculitis and other connective tissue diseases. 22 A detailed description of the frequency of inherited/acquired thrombophilias and risk factors found in patients with BCS compared to those with portal vein thrombosis is summarised by Poisson and colleagues. 23

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“…However, the use of TIPS in Asia is increasing, and available reports show similar positive results as in the West. [136][137][138][139][140] Recent data suggest that liver elastography measurements may be a good non-invasive test to evaluate the effectiveness of liver decompression. Currently this has been evaluated after invasive techniques such as balloon angioplasty (with or without stenting) in a small number of patients, showing a reduction of elastography measurement when the liver is adequately decompressed.…”

Section: Stepmentioning

confidence: 99%

Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis

Hernández‐Gea

Gottardi

Leebeek

et al. 2019

Journal of Hepatology

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Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities.

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Good Clinical Outcomes in Budd–Chiari Syndrome with Hepatic Vein Occlusion

Abstract: HV angioplasty and TIPS yield excellent long-term outcomes in patients with HV occlusive BCS.

Cited by 24 publications

References 27 publications

Long‐term outcomes of individualized treatment strategy in treatment of type I Budd‐Chiari syndrome in 456 patients

Long‐term outcomes of individualized treatment strategy in treatment of type I Budd‐Chiari syndrome in 456 patients

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

Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis

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