Background This article summarized the experience of atrioventricular valve replacement operation for functional single ventricle patients whose atrioventricular valve was insufficiency.The follow-up time of operation was arranged from early-to medium-term.Methods From July 2009 to July 2015,there were 40 patients of functional single ventricle receiving the operation of atrioventricular valve replacement,including 23 male and 17 female patients.The age at A-Ⅴ valve replacement was ranged from 6 days to 32 years (the medium age of 9 years),and the body weight ranged from 2.7 to 57 kg (the medium body weight of 21.5 kg).Moreover,the atrioventricular valve replacement was performed on all patients under extracorporeal circulation.Results Atrioventricular valve replacement was performed on 40 patients with function single ventricle in hospital,of which eight (20.0％) died in hospital including two neonate and infant (age range:6 days-2 years old) accounting for 25％ (2/8),Among them,seven patients died of low cardiac output syndrome two patients with repeated hypoxemia concurrent infection after operation,and one with bilateral diaphragmatic paralysis and malignant arrhythmia.For the patients suffering the severe perivalvular leakage,there was a reoperation,and the cardiac function before the first operation was class Ⅳ.One patient received heart transplantation finally for severe heart failure after AV valve replacement.For the rest of patients,their cardiac function recovered to class Ⅰ-Ⅱ after operation.Conclusions For function single ventricle patients with atrioventricular valve regurgitation,the mortality rate of atrioventricular valve replacement can be accepted if the operation was indicated.For patients with severe atrioventricular value regurgitation,the result of the operation in the early and medium term was satisfying.Thus,the atrioventricular valve replacement served as a useful treatment for patients diagnosed of A-Ⅴ valve regurgitation and functional single ventricle.