Published online Mar 14, 2018. doi: 10.3748/wjg.v24.i10.1134
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
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.
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.
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.
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.
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.
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.
In future studies, prospective and multi-center research should be encouraged to overcome the high rate of loss and to do the subgroup analysis.