Significant atrioventricular valve (AVV) insufficiency has been associated with increased mortality and morbidity in patients with single ventricle. Although techniques such as annuloplasty, chordal elongation, and the use of pericardial patches can improve AVV function in many patients, a number of patients continue to have significant AVV insufficiency and may require AVV replacement. The present review made a comprehensive literature review concerning the outcome, optimal timing, key points of technique skills and prognosis evaluation.