Transposition of the great vessels

August 12, 2017 17:51 | Genetic Diseases

complete transposition of the great vessels - the most common cyanotic heart disease in children in the first months of life, the frequency is 12-20% of all congenital heart defects.In older children due to the high mortality in the first year of life, its frequency is much smaller.Life expectancy at transposition of the great vessels is about 13 months.Eastern Promises is more common in boys.Sex ratio is 2: 1 - 3: 1. The first description of the transposition of the great vessels refers to the 1797

At full transposition of the great vessels of the aorta extends from the right ventricle and the pulmonary artery - the left, while in the normal heart has a reverse vascular interposition.

In transposition of the great vessels of the venous ( "work") is carried by the blood of the aorta for the systemic circulation and returns at the top and bottom of the hollow veins into the right atrium and right ventricle.From the right ventricle arterial blood rich in oxygen is released into the pulmonary artery an

d pulmonary veins of the left atrium enters the ventricle and left.Consequently, there are two open-loop circulation.Life with transposition of the great vessels is possible only with accompanying communications, which may be patent foramen ovale, atrial septal defect, ventricular septal defect, patent ductus arteriosus.Through these holes oxygen-rich blood circulating in a small circle of blood circulation, mixed with venous ( "waste") blood.From the dimensions of communications and the resistance ratio in both the systemic circulation depends on the magnitude of cross-reset and the amount of blood to be mixed, ie. E. The degree of oxygen deficiency in the body.

cyanotic heart in most cases are diagnosed in children with transposition of the great vessels in the hospital on the basis of skin cyanosis, shortness of breath and listening to the characteristic heart murmur.Intensity blueness of the skin is determined by the size of the communication and is the maximum in the open oval window.

When crying baby takes his skin purple.Quite early, in the first months of life, develop deformation terminal phalanges, underweight (malnutrition), the child behind in motor development.Repeated pneumonia in patients with transposition of the great vessels indicate overflow blood of the pulmonary circulation.With concomitant narrowing of the pulmonary artery, they occur much less frequently.

heart size may be normal or slightly increased in the first week of life in the future is rapidly developing cardiomegaly (an excessive increase in the size of the heart), which explains the early appearance of a heart hump.When listening to the noise of the work often is not the heart.Almost 1/3 of the children from the first days or weeks of life there are signs of a total heart failure.With concomitant stenosis (narrowing) of the pulmonary artery, by contrast, heart failure insignificant or non-existent, and these patients are difficult to distinguish from children with tetralogy of Fallot.

additional methods of examination of children are ECG, PCG (graphic check heart sounds), X-ray examination of the chest.If necessary, a catheterization of heart cavities and Angiocardiography.

Current progressive disease.With insufficient blood oxygen saturation and heart failure, most children quickly die.Children live longer with concomitant stenosis (narrowing) of the pulmonary artery, as it prevents an excessive pressure rise in the pulmonary artery.The hospital provides treatment with cardiac glycosides and diuretics, potassium preparations, vitamins of group B. In severe cases, surgical treatment.