Left ventricular hypoplasia syndrome

August 12, 2017 17:51 | Genetic Diseases

syndrome hypoplasia (underdevelopment) of the left ventricle is a rare congenital heart disease.The essence of the disease is the formation of a reduced left ventricular cavity, and vice aortic valve (or) of the valve between the left atrium and ventricle (the mitral valve).The incidence of heart disease is 1-6% of all congenital heart defects.In newborns with heart defects, this anomaly has become one of the most frequent causes of death (15-25%).The first description of the syndrome, left ventricular hypoplasia refers to 1952

Because inadequate (too small), left ventricular dimensions it can not adequately pump blood through the systemic circulation.As a result, a large amount of blood remains in the right ventricle and the pulmonary circulation vessels, leading to the development of pulmonary hypertension.In addition, often when there is a message in this vice between the atria as a patent foramen ovale.Thus arterial (oxygenated) blood from the left atrium due to obstacles at the level of a diseased

mitral valve through the foramen ovale enters the right atrium, right ventricle, and then into the pulmonary artery.Since this defect is also combined with a patent ductus arteriosus, the blood from the pulmonary artery passes through the duct into the aorta.

Defect flows is extremely difficult, the duration of a child's life is on average from 4 to 17 days.The first signs of the suffering that arise shortly after birth, are in violation of breathing, there a variety of signs of a lesion of the central nervous system.All children with hypoplastic left ventricle is marked pale gray skin, dyspnea 80-100 breaths per minute with indrawing of compliant places thorax (intercostal spaces, supraclavicular area).When listening to the lung doctor determines crackles.From birth moderately expressed skin cyanosis, then it becomes widespread and significant.The boundaries of the heart are determined by the attending physician, has increased dramatically, particularly in the left side.An important feature of the syndrome of hypoplasia of the left ventricle is barely perceptible pulse in the legs and arms, combined with increased heartbeat (determined by putting a hand on the heart area) and visible pulsation of the abdomen above the navel.Systolic ( "upper") blood pressure less than 60-80 mm Hg.Art.When listening to the heart is determined by heart palpitations (tachycardia) to 160-200 bpm. / Min, sometimes determined by the noise.The liver is usually enlarged, and 1/3 of children in the terminal (final) stage of the disease develops edema syndrome.

additional methods of examination of children are an electrocardiogram, chest X-ray, angiocardiography.In the latter method, research into the right ventricle, the pulmonary artery and the aorta was injected contrast agent.After this is carried out X-ray examination of the chest, which can be seen ductus arteriosus.

syndrome hypoplastic left ventricle refers to a fatal flaw.Radical surgery until now not been developed.