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Our purpose was to evaluate the effectiveness of percutaneous transhepatic direct simultaneous puncture of portal vein and inferior vena cava in Budd-Chiari syndrome and the clinical outcomes.From January 2006 to September 2017, we performed TIPS in 47 consecutive patients (mean age, 34 years) with BCS (9 patients with acute BCS and 38 patients with subacute and chronic BCS). Doppler US was performed first day, one week, one month and then 3 months apart. Portography and pressure measurements were performed every year if no shunt dysfunction was detected before. Mean follow-up of 54 months (range, 1-121 months).TIPS procedure was technically successful in all patients. In all patients, bare stents were used. Patients were anticoagulated with warfarin after TIPS procedure. Early thrombosis (within one week) was diagnosed in 12 (25%) patients and TIPS revision was required. One year primary patency was 53.6%. Clinical success was achieved in 42 patients. 5 patients required liver transplantation because of liver function deterioration but only 3 of them had liver transplantation (7 months, 1.5 years and 2 years after TIPS) other 2 patients died while on the waiting list.Percutaneous direct puncture of portal vein and inferior vena cava is safe and effective in patients with Budd-Chiari syndrome. This procedure may provide an effective alternative for the management of Budd-Chiari patients that necessitates TIPS. Close follow-up and second intervention is necessary to get good clinical outcome.