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Congenital diseases of the human heart

13 important questions on Congenital diseases of the human heart

Which septations are present in the heart?

There is a septum in the atrial region, there is the more muscular septum in the ventral region dividing left and right and finally there is a septum in the truncus arteriosus, a common outflow tract, from which the aorta and the common pulmonary trunk come from.

What disorder will be develpoed when septation is developing abnormally in the atrial region?

Atrial-septal defect (ASD)

What are the symptoms of ASD?

There are usually breathing problems later in life.
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What disorder will be developed when septatition is developing abnormal in the ventrical region?

In this case, there is no separation of left and right: ventrical-septal defect (VSD).

What are the symptoms of VSD?

Later in life, the pulmonary pressure will become lower. VSD causes large quantities of blood to flow back to the right side of the heart, because the pressure is higher in the left side. Oxygenated blood will therefore flow back to the lungs.

What disorder will be developed when there is no separation of the truncus arteriosus (so no separation of left and right in the outflow tract in the heart i.e no separation of the pulmonary artery and aorta?

Persistent truncus asrteriosus (PTA).

What is AVSD (atrio-ventricular channel septal defect)?

AVSD is a combined cardial defect, where the atrial and ventrical septa are not developed properly, which is genetic in nature. Atrial-ventrical septal defects like this are common in down syndrome.

How is endocardial-derived cushion tissue formed?

The myocardium deposits a unique ECm between the endocardium and itself. This induces an epithelial-to-mesenchymal transformation of the endocardium, resulting in the generation of migrating endocardial cushion cells that are necessary for cardiac septation.

What are the sites of cushion formation in the heart?

At the atrial, ventrical and truncus arteriosus.

What are the two main malformations of the outflow tract (septum truncus arteriosus), which eventually separates to form the aorta and pulmonary artery

Persistent truncus arteriosus and transposition of the great vessels/arteries (TGA)

What is persistent truncus arteriosus?

Here, a single outflow tract is fed by blood entering from the right and left ventricles. Normal septation hasn't occured, causing left and right blood to mix and thereby lowering the saturation of the blood. Septation and spiralling defects are often the cause for this disorder. The membranous part of the interventricular septum is commonly defective.

What is transposition of the great vessels/arteries (TGA)

TGA is caused by lack of spiralling of the truncoconal ridges in the early embryo. The aorta erroneously arises from the right ventricle, and the polmonary artery arisis from the left ventrible. Shunt between right and left is necessary. ASD, VSD and Open Ductus Ateriosus (ODA) are common.

What is ectopia cordis?

Fusion of the endocardial tubi without fusion of the throacal wall (sternum). The result of this is that the heart grows outside the troracic wall (ribbenkast).

The question on the page originate from the summary of the following study material:

Samenvatting

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