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Management of atrioventricular septal defects has grown leaps and bounds since the first correction of this malformation. Main reasons considered for the improvement are good understanding of lesion anatomy, precise preoperative diagnosis including imaging, progress in surgical technique, myocardial protection, and post-operative care. In this article we review the anatomy, pathophysiology, clinical features, associations, diagnosis, surgical therapy, complications, and results of atrioventricular septal defects.First introduced i