Abstract: AIM:To report our experience of modified Fontan procedure for adults with functional single ventricle. METHODS: Data of 15 consecutive patients with single ventricle who were treated with one-stage extracardiac Fontan procedure between November 2001 and November 2010 were retrospectively analyzed. Preoperatively, four patients were in NYHA class II and 11 were in class III. The main factors for the selection of patients before surgery were well-developed pulmonary arteries with lower lobe index >(120±30) mm/m2 and mean pulmonary artery pressure ≤15 mmHg. A fenestration 5 mm in size was constructed in six patients. RESULTS: Two cases of perioperative death occurred (total mortality 15%). The mean extubation time and hospital stay were 11 h and 13 days, respectively. The mean central venous pressure (CVP) was 13 mmHg and the mean arterial oxygen saturation was 96%. During the 41-month follow-up (range: 3-72 months), no patient died or developed arrhythmias. NYHA class improved significantly in the 12 survivors. CONCLUSION: The extracardiac Fontan procedure achieves good early- and mid-term outcome in adults with functional single ventricle, resulting in either complete or marked relief of cyanosis and enhanced exercise tolerance in all survivors. The results of long-term follow-up have yet to be established.