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 kexu@vip.sina.com. 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: http://creativecommons.org/licenses/by-nc/4.0/
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@vip.sina.com
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
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.

Keywords: Budd-Chiari syndrome, Chinese, Survival, Interventional radiology

Core tip: This is the first study to evaluate interventional treatment outcomes of Chinese Budd-Chiari syndrome (BCS) patients with more than 20-year follow-up, and the cumulative 20-year survival rate was 69.5% for patients treated by interventional radiological procedures. The cumulative 1-, 5-, 10-, and 20-year survival rates for untreated BCS patients were 70.8%, 70.8%, 53.1%, and 0%, respectively. Restenosis and gastroesophageal variceal bleeding were critical factors for predicting long-term survival. Long-term follow-up to monitor the chronic complications of BCS should not be less than 10 years, and deaths greatly increase after 10-year follow-up, especially those of patients who died from hepatocellular carcinoma.