The atrioventricular septal defects encompass a spectrum of lesions in which the common etiology appears to be abnormal development of the superior and inferior endocardial cushions, resulting in a deficiency or absence of the atrioventricular septum [1-6]. This deficiency of the atrioventricular septum results in an ostium primum defect immediately above the atrioventricular valves and a scooped-out area in the inlet (basal) portion of the ventricular septum. Data from the New England Regional Infant Cardiac Program and the Baltimore-Washington infant study defined a prevalence of 0.118 and 0.352 per 1000 live births [7,8]. Approximately, 50-75% of these patients have trisomy 21 [7-9]. When viewed from a reverse perspective, approximately 35-40% of patients with Down’s syndrome have an atrioventricular septal defect [7-9].
The atrioventricular septal defects encompass a spectrum of lesions in which the common etiology appears to be abnormal development of the superior and inferior endocardial cushions, resulting in a deficiency or absence of the the atrioventricular septum [1,2]. Data from the New England Regional Infant Cardiac Program and the Baltimore-Washington infant study defined a prevalence of 0.118 and 0.352 per 1000 live births [3,4]. Approximately, 50-75% of these patients have trisomy 21 [5]. When viewed from a reverse perspective, approximately 35-40% of patients with Down’s syndrome have an atrioventricular septal defect [5].
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