Retrospective Cohort Study
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Mar 14, 2018; 24(10): 1134-1143
Published online Mar 14, 2018. doi: 10.3748/wjg.v24.i10.1134
Budd-Chiari syndrome in China: A 30-year retrospective study on survival from a single center
Wei Zhang, Qiao-Zheng Wang, Xiao-Wei Chen, Hong-Shan Zhong, Xi-Tong Zhang, Xu-Dong Chen, Ke Xu
Wei Zhang, Xu-Dong Chen, Department of Interventional Radiology, Shenzhen People’s Hospital, the Second Affiliated Hospital of Jinan University, Shenzhen 518020, Guangdong Province, China
Wei Zhang, Qiao-Zheng Wang, Xiao-Wei Chen, Hong-Shan Zhong, Xi-Tong Zhang, Ke Xu, Department of Radiology, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China
Wei Zhang, Xiao-Wei Chen, Hong-Shan Zhong, Ke Xu, 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 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.
Institutional review board statement: This study was reviewed and approved by the Research Ethics Committee of Faculty of Medicine, The First Affiliated Hospital of China Medical University Institutional Review Board.
Informed consent statement: All study participants provided written informed consent for personal and medical data collection prior to study enrollment and each patient agreed to management via written consent.
Conflict-of-interest statement: The authors declare that there are no conflicts of interest regarding the publication of this paper.
Data sharing statement: The technical appendix, statistical code, and dataset are available from the corresponding author at The participants gave informed consent for the data sharing.
Open-Access: 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. See:
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.
Telephone: +86-24-83282730 Fax: +86-24-83282629
Received: December 8, 2017
Peer-review started: December 8, 2017
First decision: December 20, 2017
Revised: January 2, 2018
Accepted: January 24, 2018
Article in press: January 24, 2018
Published online: March 14, 2018
Research background

Budd-Chiari syndrome (BCS) is a rare disease. For Asian patients, especially Chinese patients, the predominant obstructive lesions are membranous and segmental obstructions of the supra-hepatic or retro-hepatic portion of the inferior vena cava. Till the year 2014, more than 20000 cases of BCS have been published in China, and interventional radiological procedures (mainly percutaneous re-canalization) have become the most common treatment option. However, outcomes from more than 10-year follow-up are scarcely reported for Chinese BCS patients.

Research motivation

As Chinese BCS patients are characterized by insidious onset and chronic development, ten years may not be long enough for long-term outcome observations. We want to find the 20-year and 30-year survival rates at our single center, which may represent the Chinese BCS population to a certain extent. Furthermore, for the chronic complications, such as hepatocellular carcinoma, the incidence and mortality of Chinese BCS patients are still unknown, and we are very interested in these issues.

Research objectives

The objectives were to analyze a 30-year follow-up outcome Chinese BCS patients in a single Chinese center, specifically, to find the 20-year and 30-year cumulative survival rates for the different treatment modalities applied in our center; to find the factors related to long-term survival; and to calculate the cumulative mortalities of major causes.

Research methods

We retrospectively analyzed a 30-year follow-up outcome of BCS patients at our center. Medical records of 410 patients treated between November 1983 and September 2013 with an admission diagnosis of BCS were identified in our hospitalization register system. Only the primary hospitalization medical records were enrolled. Finally, 256 patients were eligible for our study. In this case series, 34 patients were untreated (did not receive any regular treatments) and 222 patients received treatment, including 30 treated by medicine, 14 by surgery, and 178 by interventional radiology. Patients were followed until the end of this study (December 31, 2014). Symptom remission was defined as complete remission or substantial partial remission of the main symptoms that the patients complained about most urgently. Patients were examined by color Doppler ultrasonography, computed tomography, or magnetic resonance imaging for restenosis evaluation, and the results were confirmed by venography at our hospital. Cumulative survival rates and cumulative mortalities associated with major causes were analyzed. The Cox regression model was employed for the analysis of factors related to survival. Variables reaching statistical significance in the univariate analysis were incorporated into a multivariate analysis as covariates. P < 0.05 was considered statistically significant.

Research results

About 212 patients (44 were lost to follow-up) were followed with a median time of 89 (0.2-360) mo; 67 of the followed patients died, with a median follow-up period of 28 (0.2-289) mo. A statistically significant difference was found in cumulative survival rates between these two groups. A statistically significant difference was also found in cumulative survival rates among these three treatment modalities. The independent predictors of survival were gastroesophageal variceal bleeding and restenosis. For the treated patients, the major causes of death were liver or multiple organ failure, gastroesophageal variceal bleeding, and hepatocellular carcinoma (HCC), which accounted for more than 80% of the total deaths.

Research conclusions

The present study is the first large case series that evaluated interventional treatment outcomes of Chinese BCS patients with more than 20-year follow-up, to the best of our knowledge. We suggest that the long-term follow-up span should not be less than 10 years for Chinese BCS patients. Less than half of the patients had intermittent, spontaneous relief of clinical symptoms, and none survived for more than 20 years. Restenosis and gastroesophageal variceal bleeding were critical factors for predicting the long-term survival. To monitor the chronic complications of BCS such as HCC, long-term follow-up should not be less than 10 years.

Research perspectives

In future studies, prospective and multi-center research should be encouraged to overcome the high rate of loss and to do the subgroup analysis.