Defect aortolegochnoy partitions

August 12, 2017 17:51 | Genetic Diseases

septum defect aortolegochnoy makes communication between the adjacent portions of the aorta and the pulmonary artery trunk.Other names: aortopulmonary septal defect, partial arterial trunk, defective aortic walls, aortolegochnaya fenestration, fistula or window.Defect first described in 1830, is extremely rare (0.3 to 1% of all cases of congenital heart disease).

most common defect found in isolation, sometimes combined with other congenital heart defects, such as ventricular septal defect, atrial septal defect, aortic arch interruption, coarctation of the aorta, pulmonary stenosis.

severe disorders of blood circulation in the defect aortolegochnoy septum due to its size, location, value for resistance in the blood vessels of the lungs and blood vessels of the systemic circulation.During systole, blood flows from aorta under great pressure directly into the pulmonary artery.Thus shunt, as with patent ductus arteriosus occurs in systole and diastole.All this contributes to a more rapid development of th

e increase in pressure in the pulmonary artery (pulmonary hypertension), which is not only for children with a small defect.Due to the nature of blood circulation there is an overload of the left ventricle increased blood volume.

In young children the first sign of a blemish with increasing shortness of breath frequency, then join the lag in physical development, repeated pneumonia.Cyanosis of the skin may be the children of the first weeks of life, then the lower the pressure in the pulmonary artery, it disappears, and its appearance indicates nothing of the highly sclerotic nature of pulmonary hypertension that often occurs in older children and adults.Borders of the heart, the physician, extended in both directions.Above the heart, when it is feeling, determined shake that disappears with age, which is also a sign of pulmonary hypertension.When listening to the work of the heart is determined characteristic noise.

additional methods of examination of children are ECG, PCG (graphic registration heart murmurs), radiographs of the chest.If necessary, a catheterization of heart cavities.

With a large defect of poor prognosis, as the majority of children die in the first months of life of heart failure or pneumonia.

treatment comes down to surgery, in which the produce defect closure.For a relatively small defect in communication between the aorta and pulmonary artery ligated.In low-lying defects dissect it, and then the wall of the aorta and the pulmonary artery is sutured.In cases of larger defects surgery performed with cardiopulmonary bypass using a synthetic patch.The risk of this operation is higher than during the ligation of patent ductus arteriosus.