Budd-Chiari syndrome in China: A 30-year retrospective study on survival from a single center

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Abstract

AIM

To investigate 30-year treatment outcomes associated with Budd-Chiari syndrome (BCS) at a tertiary hospital in China.

METHODS

A total of 256 patients diagnosed with primary BCS at our tertiary hospital between November 1983 and September 2013 were followed and retrospectively studied. Cumulative survival rates and cumulative mortality rates of major causes were calculated by Kaplan-Meier analysis, and the independent predictors of survival were identified using a Cox regression model.

RESULTS

Thirty-four patients were untreated; however, 222 patients were treated by medicine, surgery, or interventional radiology. Forty-four patients were lost to follow-up; however, 212 patients were followed, 67 of whom died. The symptom remission rates of treated and untreated patients were 81.1% (107/132) and 46.2% (6/13), respectively ( P = 0.009). The cumulative 1-, 5-, 10-, 20-, and 30-year survival rates of the treated patients were 93.5%, 81.6%, 75.2%, 64.7%, and 58.2%, respectively; however, the 1-, 5-, 10-, 20-, and 30-year survival rates of the untreated patients were 70.8%, 70.8%, 53.1%, 0%, and unavailable, respectively ( P = 0.007). Independent predictors of survival for treated patients were gastroesophageal variceal bleeding (HR = 3.043, 95%CI: 1.363-6.791, P = 0.007) and restenosis (HR = 4.610, 95%CI: 1.916-11.091, P = 0.001). The cumulative 1-, 5-, 10-, 20-, and 30-year mortality rates for hepatocellular carcinoma were 0%, 2.6%, 3.5%, 8%, and 17.4%, respectively.

CONCLUSION

Long-term survival is satisfactory for treated Chinese patients with BCS. Hepatocellular carcinoma is a chronic complication and should be monitored with long-term follow-up.

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Most cited references 32

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Etiology, management, and outcome of the Budd-Chiari syndrome.

Frits Rosendaal, Elwyn Elias, Nicole A H Janssen … (2009)

The Budd-Chiari syndrome (BCS) is hepatic venous outflow obstruction. What is known about the syndrome is based on small studies of prevalent cases. To characterize the causes and treatment of incident BCS. Consecutive case series of patients with incident BCS, enrolled from October 2003 to October 2005 and followed until May 2006. Academic and nonacademic hospitals in France, Spain, Italy, Great Britain, Germany, Belgium, the Netherlands, Portugal, and Switzerland. Persons older than 16 years with definite hepatic outflow obstruction diagnosed by imaging. Persons with hepatic outflow obstruction due to heart failure, sinusoidal obstruction syndrome, cancer, or liver transplantation were excluded. Signs and symptoms; laboratory and imaging findings; diagnosis; treatment; and overall, transplantation-free, and intervention-free survival. 163 incident cases of BCS were identified. Median follow-up was 17 months (range, 0.1 to 31 months). Most patients (84%) had at least 1 thrombotic risk factor, and many (46%) had more than 1; the most common was myeloproliferative disorders (49% of 103 tested patients). Patients were mainly treated with anticoagulation (140 patients [86%]), transjugular intrahepatic portosystemic shunting (56 patients [34%]), or liver transplantation (20 patients [12%]), and 80 patients (49%) were managed noninvasively. Only 3 patients underwent surgical shunting. The survival rate was 87% (95% CI, 82% to 93%) at 1 year and 82% (CI, 75% to 88%) at 2 years. Treatment was not standardized across all centers, and data on important clinical variables were missing for some patients. Most patients with BCS have at least 1 thrombotic risk factor, and many have more than 1; myeloproliferative disorders are most common. One- and 2-year survival rates are good with contemporary management, which includes noninvasive therapies (anticoagulation and diuretics) and invasive techniques. Transjugular intrahepatic portosystemic shunting seems to have replaced surgical shunting as the most common invasive therapeutic procedure. Fifth Framework Programme of the European Commission.

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Primary Budd-Chiari syndrome.

Dominique-Charles Valla (2009)

Primary Budd-Chiari syndrome is characterized by a blocked hepatic venous outflow tract at various levels from small hepatic veins to inferior vena cava, resulting from thrombosis or its fibrous sequellae. This rare disease affects mainly young adults. Multiple risk factors have been identified and are often combined in the same patient. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients; their diagnosis can be made by showing the V617F mutation in Janus tyrosine kinase-2 gene of peripheral blood granulocytes and, should this mutation be absent, by showing clusters of dystrophic megacaryocytes at bone marrow biopsy. Presentation and manifestations are extremely varied, so that the diagnosis must be considered in any patient with acute or chronic liver disease. Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and inferior vena cava are usually successful in demonstrating non-invasively the obstacle or its consequences, the collaterals to hepatic veins or inferior vena cava. The disease is considered to be spontaneously lethal within 3 years of first symptoms. A therapeutic strategy has been proposed where anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension are used first; then angioplasty for short-length venous stenoses; then TIPS; and ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 90%. Medium-term prognosis depends on the severity of liver disease. Long-term outcome might be jeopardized by transformation of underlying conditions and hepatocellular carcinoma.

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Good long-term outcome of Budd-Chiari syndrome with a step-wise management.

Susana Seijo, Aurélie Plessier, Jildou Hoekstra … (2013)

Budd-Chiari syndrome (BCS) is a rare, life-threatening disease caused by obstruction of hepatic venous outflow. The aim of the study was to assess long-term outcome and identify prognostic factors in BCS patients managed by a step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPS), and orthotopic liver transplantation (OLT). We reviewed long-term data on 157 patients previously included by the European Network for Vascular Disorders of the Liver, a multicenter prospective study of newly diagnosed BCS patients in nine European countries. Patients were followed for a median of 50 months (range, 0.1-74.0). During the study, 88 patients (56%) received at least one invasive intervention (22 patients angioplasty/thrombolysis, 62 TIPS, and 20 OLT) and 36 (22.9%) died. Most interventions and/or deaths occurred in the first 2 years after diagnosis. The Rotterdam score was excellent in predicting intervention-free survival, and no other variable could significantly improve its prognostic ability. Moreover, BCS-TIPS prognostic index (PI) score (based on international normalized ratio, bilirubin, and age) was strongly associated with survival and had a discriminative capacity, which was superior to the Rotterdam score.

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Author and article information

Contributors

Wei Zhang

Qiao-Zheng Wang

Xiao-Wei Chen

Hong-Shan Zhong

Xi-Tong Zhang

Xu-Dong Chen

Ke Xu

Journal

Journal ID (nlm-ta): World J Gastroenterol

Journal ID (iso-abbrev): World J. Gastroenterol

Journal ID (publisher-id): WJG

Title: World Journal of Gastroenterology

Publisher: Baishideng Publishing Group Inc

ISSN (Print): 1007-9327

ISSN (Electronic): 2219-2840

Publication date (Print): 14 March 2018

Publication date (Electronic): 14 March 2018

Volume: 24

Issue: 10

Pages: 1134-1143

Affiliations

Department of Interventional Radiology, Shenzhen People’s Hospital, the Second Affiliated Hospital of Jinan University, Shenzhen 518020, Guangdong Province, China

Department of Radiology, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China

Key Laboratory of Diagnostic Imaging and Interventional Radiology of Liaoning Province, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China

Department of Radiology, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China

Key Laboratory of Diagnostic Imaging and Interventional Radiology of Liaoning Province, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China

Department of Radiology, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China

Key Laboratory of Diagnostic Imaging and Interventional Radiology of Liaoning Province, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China

Department of Radiology, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China

Department of Interventional Radiology, Shenzhen People’s Hospital, the Second Affiliated Hospital of Jinan University, Shenzhen 518020, Guangdong Province, China

Department of Radiology, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China. kexu@ 123456vip.sina.com

Key Laboratory of Diagnostic Imaging and Interventional Radiology of Liaoning Province, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China

Author notes

Author contributions: Zhang W, Xu K and Chen XD designed the research; Xu K, Zhang XD and Zhong HS performed the research; Zhang W, Wang QZ and Chen XW analyzed the data; Zhang W wrote the paper; Wang QZ, Chen XW, Zhong HS, Zhang XT, Chen XD and Xu K critically revised the manuscript for important intellectual content.

Correspondence to: Ke Xu, MD, PhD, Professor, Department of Radiology and Key Laboratory of Diagnostic Imaging and Interventional Radiology of Liaoning Province, the First Affiliated Hospital of China Medical University, 155 Nanjing Bei Street, Shenyang 110001, Liaoning Province, China. kexu@ 123456vip.sina.com

Telephone: +86-24-83282730 Fax: +86-24-83282629

Article

Other ID: jWJG.v24.i10.pg1134

DOI: 10.3748/wjg.v24.i10.1134

PMC ID: 5850132

PubMed ID: 29563757

SO-VID: 94c7b5a8-815e-45a2-b269-2a0de8d56767

License:

This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial.

History

Date received : 8 December 2017

Date revision received : 2 January 2018

Date accepted : 24 January 2018

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Budd-Chiari syndrome in China: A 30-year retrospective study on survival from a single center

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Abstract

AIM

METHODS

RESULTS

CONCLUSION

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Transnational Chinese identity

Most cited references 32

Etiology, management, and outcome of the Budd-Chiari syndrome.

Primary Budd-Chiari syndrome.

Good long-term outcome of Budd-Chiari syndrome with a step-wise management.

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Cited by 8

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