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