AIM: To evaluate long-term clinical course of Budd- Chiari syndrome (BCS) and predictive factors associated with the development of hepatocellular carcinoma (HCC) and survival. METHODS: We analyzed 67 patients with BCS between June 1988 and May 2008. The diagnosis of BCS was confirmed by hepatic venous outflow obstruction shown on abdominal ultrasound sonography, computed tomography, magnetic resonance imaging, or venography. The median follow-up period was 103 ± 156 [interquartile range (IQR)] mo. RESULTS: The median age of the patients was 47 ± 16 (IQR) years. At diagnosis, 54 patients had cirrhosis, 25 (37.3%) Child-Pugh class A, 23 (34.3%) Child-Pugh class B, and six (9.0%) patients Child-Pugh class C. During the follow-up period, HCC was developed in 17 patients, and the annual incidence of HCC in patients with BCS was 2.8%. Patients in HCC group (n = 17) had higher hepatic venous pressure gradient (HVPG) than those in non-HCC group (n = 50) (21 ± 12 mmHg vs 14 ± 7 mmHg, P = 0.019). The survival rate of BCS patients was 86.2% for 5 years, 73.8% for 10 years, and 61.2% for 15 years. In patients with BCS and HCC, survival was 79% for 5 years, 43.1% for 10 years, and 21.5% for 15 years. CONCLUSION: The incidence of HCC in patients with BCS was similar to that in patients with other etiologic cirrhosis in South Korea. The HVPG is expected to provide additional information for predicting HCC development in BCS patients.
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