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AVCD is caused by an abnormal or inadequate fusion of the superior and inferior endocardial cushions with the mid portion of the atrial septum and the muscular portion of the ventricular septum. [3] Unlike some heart defects, the condition will not resolve over time and most infants must undergo open heart surgery.
The endocardial cushions are thought to arise from a subset of endothelial cells that undergo epithelial-mesenchymal transition, a process whereby these cells break cell-to-cell contacts and migrate into the cardiac jelly (towards the interior of the heart tube).
A ventricular septal defect is when this lower wall—the ventricular septum—has a gap in it after development. The septum is formed during development as this muscular ridge of tissue grows upward from the apex, or the tip, and then fuses with a thinner membranous region coming down from the endocardial cushions.
The ostium primum atrial septal defect is a defect in the atrial septum at the level of the tricuspid and mitral valves. This is sometimes known as an endocardial cushion defect because it often involves the endocardial cushion, which is the portion of the heart where the atrial septum meets the ventricular septum and the mitral valve meets the tricuspid valve.
Failure of the septum primum to fuse with the endocardial cushion can lead to an ostium primum atrial septal defect. [1] This is the second most common type of atrial septal defect [2] and is commonly seen in Down syndrome. Typically this defect will cause a shunt to occur from the left atrium to the right atrium.
The severity of symptoms depends on the type of TGV, and the type and size of other heart defects that may be present (ventricular septal defect, atrial septal defect, or patent ductus arteriosus). Most babies with TGA have blue skin color (cyanosis) in the first hours or days of their lives, since dextro-TGA is the more common type.
Heart septal defect refers to a congenital heart defect [1] of one of the septa of the heart. Atrial septal defect; Atrioventricular septal defect; Ventricular septal defect; Although aortopulmonary septal defects are defects of the aorticopulmonary septum, which is not technically part of the heart, they are sometimes grouped with the heart ...
Heart sounds of a ventricular septal defect in a 14-year-old girl. A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The extent of the opening may vary from pin size to complete absence of the ventricular septum, creating one common ventricle.