[Study on clinical features and etiology of primary Budd-Chiari Syndrome]

To prospectively study clinical features and etiology in patients with incident Budd-Chiari syndrome (BCS) in China.

Taking consecutive case series of patients with incident BCS as who were diagnosed in our hospital, enrolled from September 2010 to January 2012 as the object of research, and the follow-up was lasting until June 2012. Taking records for all patients' symptoms, signs, laboratory findings, radiology findings, treatment, interventional treatment survival and symptom-free period.

There are total 149 incident cases of BCS. In which, the median age was 46 years old (range 10 to 82); 61.7% of them were male patients, 38.3% were female patients; 85.9% of them were chronic, the other patients (14.1%) were diagnosed during acute or subacute periods; the median duration of symptoms before diagnosis was 96 months (range 1 day to 360 months). In terms of causes, 30.9% of the patients caused by hepatic venous block, 5.4% of them resulted from inferior vena cava block, and the rest 63.8% were suffered from combined hepatic venous and inferior vena cava block. 80.5% patients have at least one etiological factor, Furthermore, the most common cause was the web (61.1%), only 4.8% have myeloproliferative diseases (JAK2 V617F mutation), and none Factor V Leiden mutation cases was found. 144 patients were treated by percutaneous transluminal angioplasty, the technical success rate was 95.1%, and took oral anticoagulation therapy for 12 months. At 18 months, The survival rate and the symptom-free survival rate after percutaneous transluminal angioplasty were 97.8% and respectively.

Web is the most prevalent etiological factor for BCS in China. It is different in western countries; the common reasons of BCS are risk factors related to thrombosis, such as myeloproliferative disease and Factor V Leiden mutation, etc., which are seldom found in Chinese BCS patients. In China, most chronic BCS patients were treated with percutaneous transluminal angioplasty and have excellent clinical outcome.