16.10 Pathologie



Heart abnormality with right-left shunt (cyanotic)


In cardiac abnormalities with right-left shunts oxygen-poor blood gets from the right half of the heart into the left side and thus into the systemic circulatory system. In most of the defects with right-left shunts the physiologic shunts present before birth remain functional because no reversal of pressure happens following birth.



Fallot's tetralogy

Fallot's tetralogy, described in 1888, is met with in roughly 8% of all congenital cardiac abnormalities. It comprises the following defects:
  • Pulmonary stenosis (PS)
  • Ventricle septum defect (VSD)
  • Overriding aorta
  • Right ventricular hypertrophy
Fig. 40 - Fallot's tetralogy  Legend

1
2
3
4		 Pulmonary stenosis
Ventricle septum defect
Hypertrophy of the right ventricle
Aorta riding over the septum defect

Fig. 40
The pulmonary stenosis (PS) determines the degree of severity of this defect. Pulmonary perfusion depends on the size of the PS.

More info

The hemodynamic is determined by the size of the pulmonary stenosis. With increasing degree of the pulmonary stenosis the severity of the right-left shunt via the ventricular septum defect, facilitated by the overriding of the aorta, increases. The origin of this cardiac abnormality is not known in detail, but a correlation with a chromosome aberration has been found (22q11, compare catch syndrome, 12).

Overview, according to the clinical picture, of the diagnostic possibilities as well as the therapy.



Pulmonary valve atresia

This cardiac abnormality is very rare and accounts for only 1-3%. In this affliction the pulmonary valve is atretic and there is no exit from the right ventricle. Thus all of the blood regurgitates into the left atrium via the foramen ovale and the lungs get perfused retrogradely via a very wide ductus arteriosus.

More info

One distinguishes several forms.


Tricuspid atresia

With an incidence of 3% tricuspidal atresia also belongs to the rare cardiac abnormalities. Through atresia of the tricuspidal valve a connection between the right atrium and the right ventricle is missing. Since this abnormality is mostly combined with a VSD, its effects depend on the size of the VSD.


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