Grown-up Congenital Heart Disease (GUCH): Difference between revisions

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Grown-up Congenital Heart Disease (GUCH) (view source)

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The atrioventricular septal defect (AVSD) consist of several different lesions with a common atrioventricular (AV) junction and abnormal AV valves, consisting of five leaflets (Figure 5). The AVSD makes 3 procent of all congenital heart defects in children.
The atrioventricular septal defect (AVSD) consist of several different lesions with a common atrioventricular (AV) junction and abnormal AV valves, consisting of five leaflets (Figure 5). The AVSD makes 3 procent of all congenital heart defects in children.


When the AVSD is complete it consists of a defect on the atrial and on the ventricular side of the common AV-valve ring. (figure 5, middle). The complete AVSD is usually associated with Down Syndrome, but also with other cardiac defects like ASD type 2, persisting left inferior caval vein and tetralogy of Fallot.  
When the AVSD is complete it consists of a defect on the atrial and on the ventricular side of the common AV-valve ring. (Figure 5, middle). The complete AVSD is usually associated with Down Syndrome, but also with other cardiac defects like ASD type 2, persisting left inferior caval vein and tetralogy of Fallot.  




In an incomplete AVSD the superior and inferior bridging leaflets are connected with each other and with the interventricular septum in the centre. (figure 5, right) Due to this connection there are two divided AV inlets, leaving no open communication between the ventricles, thus no VSD exists. However there is a rather large defect in the interatrial septum. The incomplete AVSD is often referred to as ostium primum defect or ASD type 1.
In an incomplete AVSD the superior and inferior bridging leaflets are connected with each other and with the interventricular septum in the centre. (Figure 5, right) Due to this connection there are two divided AV inlets, leaving no open communication between the ventricles, thus no VSD exists. However there is a rather large defect in the interatrial septum. The incomplete AVSD is often referred to as ostium primum defect or ASD type 1.
The left AV-valve consist of three leaflets (there is a cleft in the mitral valve) and is usually incompetent. Due to one common AV junction in both types of AVSD, the aortic valve is not in the usual wedged position between the two separate AV inlets, but located more anterior. Therefore the outflow tract of the left ventricle is elongated and slightly constricted. In angiography this abnormally shaped outflow tract is known as a gooseneck.
The left AV-valve consist of three leaflets (there is a cleft in the mitral valve) and is usually incompetent. Due to one common AV junction in both types of AVSD, the aortic valve is not in the usual wedged position between the two separate AV inlets, but located more anterior. Therefore the outflow tract of the left ventricle is elongated and slightly constricted. In angiography this abnormally shaped outflow tract is known as a gooseneck.


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