Objective To investigate clinical outcomes of one and a half ventricle repair for acute right ventricular dysfunction after biventricular repair. Methods Clinical data of 5 pediatric patients with congenital heart diseases who underwent emergency bidirectional Glenn shunt without cardiopulmonary bypass for acute right ventricular dysfunction after biventricular repair，converting the operation into one and a half ventricle repair，from February 2007 to June 2012 in Qingdao Women and Children Hospital were retrospectively analyzed. There were 4 male patients and 1 female patient with their age of 7-18 months and body weight of 6-13 kg. Preoperative diagnosis included pulmonary atresia with intact ventricular septum （PA/IVS） in 1 patient，tricuspid stenosis （TS） with ventricular septal defect （VSD） and atrial septal defect （ASD） in 2 patients，and tricuspid stenosis with tetralogy of Fallot （TOF） in 2 patients. Postoperative care focused on cardiopulmonary support and control of pulmonary artery pressure. Results Mean pulmonary artery pressure （mPAP） was 12-18 mm Hg at 72 hours after emergency bidirectional Glenn shunt. Mechanical ventilation time was 3-182 hours and ICU stay was 2-13 days. Postoperatively 1 patient died of pulmonary hypertension crisis. The other 4 patients were discharged with their transcutaneous oxygen saturation in the resting state of 93%-99%，which was improved in different degrees compared with preoperative value. These 4 patients were followed up from 6 months to 4 years. Three patients were in NYHA classⅠand 1 patient was in NYHA class Ⅱ during follow-up. Echocardiography showed smooth vena cava to pulmonary artery anastomosis without thrombosis formation. Conclusion One and a half ventricle repair can be used as an adjunct surgical strategy for acute right ventricular dysfunction after biventricular repair.