Syndrome of hypoplasia of the left ventricle
The syndrome of hypoplasia( underdevelopment) of the left ventricle refers to a rare congenital heart disease. The essence of this disease lies in the formation of a reduced left ventricular cavity, aortic valve aortic and( or) valve between the left atrium and the ventricle( mitral valve).The incidence of this heart defect is 1-6% of all congenital heart defects. In newborns with heart defects, this anomaly becomes one of the most frequent causes of death( 15-25%).The first description of left ventricular hypoplasia syndrome refers to 1952.
Because of the inadequate( excessively small) dimensions of the left ventricle, it can not adequately pump blood over a large range of blood circulation. As a result, a large amount of blood remains in the right ventricle and vessels of the small circle of blood circulation, leading to the development of pulmonary hypertension. In addition, often with this defect there is a message between the atria in the form of an oval window. At the same time, arterial( oxygen-enriched) blood from the left atrium due to an obstruction at the level of the pathologically altered mitral valve through the open oval window enters the right atrium, into the right ventricle, and then into the pulmonary artery. Since this defect is also combined with the absence of an arterial duct, the blood from the pulmonary artery flows through this duct to the aorta.
The vice is extremely difficult, the life expectancy of the child is on average from 4 to 17 days. The first signs of suffering, occurring soon after birth, are the violation of breathing, there are various signs of damage to the central nervous system. All children with left ventricular hypoplasia have a pale gray color of the skin, pronounced dyspnea up to 80-100 respiratory movements per minute with retraction of compliant places of the chest( intercostal spaces, supraclavicular area).When you listen to the lungs, the doctor determines wet rales. From birth, the blueness of the skin is moderately pronounced, then it becomes widespread and significant. The boundaries of the heart, which determines the attending physician, are sharply increased, especially in the left side. An important sign in the left ventricular hypoplasia syndrome is an almost palpable pulse on the legs and hands in combination with an intensified contraction of the heart( determined by placing the palm on the heart area) and a visible pulsation of the abdomen above the navel. Systolic( "upper") blood pressure does not exceed 60-80 mm Hg. Art. When listening to the heart, the heart rate increases( tachycardia) to 160-200 beats / min, noise is sometimes determined. The liver, as a rule, is enlarged in size, and in 1/3 of children in the terminal( final) stage of the disease, edematous syndrome develops.
Additional methods of examination of children are ECG, chest X-ray, angiocardiography. In the case of the latter method, a contrast agent is injected into the right ventricle, pulmonary artery and aorta. After this, an x-ray examination of the chest organs is performed, where an open arterial duct is visible.
Left ventricular hypoplasia syndrome refers to fatal defects. Radical surgical treatment has not been developed to date.