Atrioventricular canal defect

Description

Formation of the atrioventricular valves and septa depends upon precise central fusion of the endocardial cushions with the septum primum and the primitive interventricular septum. Failure of this process results in a broad spectrum of congenital cardiac anomalies and the  most complex of these anomalies is complete atrioventricular (AV) canal defect. It is a serious and complex cardiac anomaly that is frequently associated with other congenital cardiac defects. Its natural course is ultimately fatal and  80% of children born with this defect will die within 2 years. Long-term medical therapy for complete atrioventricular canal defect is ineffective. Therefore, either palliative or curative surgery is required. The risk of corrective surgery for this defect in infancy has steadily decreased because of improvements in surgical techniques, anesthesia and postoperative management. The primary goals of the surgery is  closure of the left-to-right shunt and restoration of normal valvular function.