H. Eberhardt (Marburg), S. Kallsen (Landshut)  

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Newborn  

N/A  

Thorax-Heart  

congenital  

Mature newborn with retarded postnatal adaptation. On the first day of life, noticeable cyanotic skin color, oxygen saturation 70-80%. 1/6 systolic bruit.  

Congenital defect  

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Pathology 1: Obvious generalized skin cyanosis.






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Ultrasound 1: Echocardiography: In the parasternal longitudinal axis, a so-called "Pistol double run" in the parallel lying aorta and the pulmonary trunk.

Next to the shown (pathognomonic) sign of the pistol double run, there are typical sonographic-anatomical signs that show from which ventricle each of the vessels originate from.

For a morphological right ventricle are the following criteria: The corresponding AV-valve is attached to the tip of the heart and is fixated with it's tendons to the septum. There is a coarse trabecularization (septomarginal trabecula), no fibrous continuity between the AV-valve and semilunar valve, but rather a muscular infudibulum.

For the left ventricle: AV-valve - furthest away from the tip of the heart, no attachment to the septum, therefore 2 papillary muscles, smooth walls, fibrous continuity between the AV-valve and semilunar valve.

All these morphological criteria can be well-seen in the parasternal longitudinal axis, the parasternal short axis and the 4 ventricle view.

 

Total constellation (Consens)  

N/A  

Shortly after birth, the execution of a hyperoxic testes indicates differentiation of a pulmonary or a cardiac cause of the cyanosis in a persistent cyanotic child. A saturation to 100% makes the thought of a TGA impossible.

TGA means that pulmonary and systemic circulation is in parallel, in the pulmonary circulation, the O2 saturated blood circulates through the lung; in the body, the blood is de-saturated. To increase the supply of O2 in the brain and organs, a shunt must be placed, which leads to the mixing of blood. It is therefore important to assess the shunt flow of the ductus arteriosus, the atria and in the case of VSD, at the level of the ventricles.

If opening the ductus arteriosus with prostaglandine application, the pulmonary blood flow is increased. This means that the blood flow in the left atrium is increased, which leads to the inter-atrial connection. In increased flow through PgE application, one may observe an increase in the flow speed (in restrictive foramen ovale/ASD) in doppler sonography. This means that the gradient between the left and the right atrium is seen. This not only means that the blood which is mixed is reduced, but also that the pressure in the left atrium increases and can lead to congestion in the pulmonary circulation. Therefore, it is important to detect this by pw-doppler sonography after application of PgE. Clinically, cyanosis can continue to exist, signs of beginning pulmonary edema can be seen. The child does not improve. In fact, the child will worsen. It is recommended that the sonographic placement is substernal, so that the atrial septum is caught vertically, the resolution is the greatest that way, and therefore the doppler study can be used to assess the atrial shunt ideally.

There must be a follow-up examination, because in bad situations, there is also an indication for a Rashkind Manoveur. After stabilization, a operative procedure should be considered (Switch surgery, Rastell surgery).  

N/A  

N/A  

1. Medline:
Waldman JD, Holmes G
Transposition of the great arteries S.193-195
in Skinner J, Alverson D, Hunter S
Echocardiography for the neonatologist
Churchill Livingstone, 1st Edition, 2000
 

cyanotic heart defect, TGA, transposition of the great arteries, transposition of the great vessels, systolic murmur, cyanosis, desaturation, child, childhood, pediatric radiology  

H Eberhardt, S Kallsen. Transposition of great arteries (TGA). PedRad [serial online] vol 3, no. 6.
URL: www.PedRad.info/?search=20030616161230