Aorto-pulpal septal defect

The defect of the aorto-pulmonary septum causes communication between the adjacent parts of the aorta and the pulmonary artery trunk. Other names: aortopulmonary septal defect, partial arterial trunk, aortic septal defect, aorto-pulmonary fenestration, fistula or window. The vice was first described in 1830, it is extremely rare( from 0.3 to 1% of all cases of congenital heart defects).

More often the vice meets in the isolated kind, is more rare combined with other congenital heart defects, such as the defect of the interventricular septum, the defect of the interatrial septum, the break of the aortic arch, coarctation of the aorta, stenosis of the pulmonary artery.

The severity of circulatory disorders in the defect of the aorto-pulmonary septum is due to its size, location, the ratio of resistance in the vessels of the lungs and blood vessels of the circulatory system. During systole, blood from the aorta comes under high pressure directly into the pulmonary artery. In this case, the discharge of blood, as in the open arterial duct, occurs in both systole and diastole. All this contributes to a more rapid development of increased pulmonary artery pressure( pulmonary hypertension), which is absent only in children with a small defect. In connection with the peculiarities of blood circulation, there is an overload of the left ventricle with an increased volume of blood.

In young children, the first sign of blemish is shortness of breath with an increased respiratory rate, then a lag in physical development, repeated pneumonia, is added. Cyanosis of the skin can be in children of the first weeks of life, then as the pressure in the pulmonary artery decreases, it disappears, and its appearance indicates already high pulmonary hypertension of the sclerotic nature, which is more common in older children and adults. The boundaries of the heart, determined by the doctor, are widened in both directions. Above the heart area, when it feels, the tremor is determined, which disappears with age, which is also a sign of pulmonary hypertension. When listening to the work of the heart determine the characteristic noise.

Additional methods for examining children are ECG, PCG( graphic recording of heart sounds), chest radiograph. If necessary, conduct a catheterization of the heart cavities.

With a large defect, the prognosis is poor, since most children die in the first months of life from heart failure or pneumonia.

Treatment is reduced to surgical intervention, in which the closure of the defect is performed. With a relatively small defect, the communication between the aorta and the pulmonary artery is bandaged. With a low defect location, it is dissected, and then the aortic and pulmonary artery walls are sutured. In cases of large defects, the operation is performed under conditions of artificial circulation using a synthetic patch. The risk in this operation is higher than with the ligation of the open ductus arteriosus.