LAUSR

OBJECTIVES Complete atrioventricular septal defect with tetralogy of Fallot is a rare congenital heart disease. The combination of these lesions occurs in about 1% of all patients with atrioventricular canal defects and in 5-6% of patients with tetralogy of Fallot. To assess the influence of surgical strategy on the survival and reintervention rate for the left atrioventricular valve and right ventricular outflow tract. METHODS We analyzed all related studies since 1986. Thirty-eight articles were initially retrieved via PubMed/MEDLINE, Cochrane Central Register of Controlled Trials and Google Scholar, from which 18 retrospective studies were included in the systematic review and 8 studies in the meta-analysis. RESULTS There was no significant difference in the 6-year survival between staged repair and primary repair [80 patients in the primary group and 81 patients in the staged group; I2 = 0%; time-to-event data Peto odds ratio 0.66, 95% confidence interval (CI) 0.3-1.5, P = 0.31; hazard ratio 0.66, 95% CI 0.3-1.3, P = 0.23]. Both groups had an equal reintervention rate for the left atrioventricular valve [75 patients in the primary group and 71 patients in the staged group; I2 = 0.26%; the Mantel-Haenszel odds ratio 0.60, 95% CI 0.22-1.68, P = 0.33], but patients who received an initial palliation had a higher rate of reoperation on the right ventricular outflow tract [I2 = 0%; the Mantel-Haenszel odds ratio 0.27, 95% CI 0.27-0.9988, P = 0.05]. CONCLUSIONS Results of this meta-analysis reveal no difference in 6-year survival and reoperation rate for the left atrioventricular valve; however, patients who underwent staged repair had a higher rate of reintervention for the right ventricular outflow tract, which could be related to initially poor pulmonary bed anatomy. Therefore, both the primary repair and the staged repair are acceptable options for repair of complete atrioventricular septal defect with tetralogy of Fallot. The choice of surgical strategy must consider the anatomy of the pulmonary bed, patient condition and associated anomalies, which could affect surgical risk.