Isolated pulmonary artery stenosis

August 12, 2017 17:51 | Genetic Diseases

isolated stenosis (narrowing) of the pulmonary artery is one of common congenital heart defects and is 6,8-9% of all heart defects.This pathology is equally common in boys and girls.The first description of the defect relates to the 1749

If there is an obstacle release blood from the right ventricle, the pressure in it at the time of contraction (systole) significantly increased (up to 200 mm Hg. Art.).This results in a pressure difference between the right ventricle and the pulmonary artery that ensures the safety of the blood discharge into the pulmonary circulation.In children with severe stenosis of the pulmonary artery of the isolated right atrial pressure rises.Against the backdrop of increased pressure in the right atrium expands the oval window, and reset the "waste" of blood occurs in the left ventricle, ie. E. In the systemic circulation.The amount of blood discharge determines the severity of bluish color of the skin (from crimson to dark blue).

Features manifestations of isolated stenosis

of the pulmonary artery is entirely dependent on the degree of narrowing.When "pale" isolated cases of stenosis of the pulmonary artery children form good complaints irrelevant and mostly reduced to shortness of breath with exertion, often fingertips, nose, cheeks are crimson hue.The only blemish is a manifestation of the rough noise, which is determined by the physician during auscultation of the heart.The intensity and duration of the noise is directly proportional to the degree of stenosis.In "blue" forms of the isolated pulmonary artery stenosis (narrowing of the sharp) leading signs are cyanosis of the skin, resistant to heart failure treatment, including an enlarged liver, and edema syndrome.Intensity of skin cyanosis is determined by the discharge "waste" blood in the systemic circulation at the level of the atria.With age, its intensity increases, evolving symptom of "time windows" and "drumsticks", shortness of breath occurs at the slightest exertion, children complain of pain in the heart.When the isolated pulmonary artery stenosis lag behind in physical development, only children with severe deficiency of oxygen in the blood.Most of the children formed sided heart hump.On palpation (feeling) the heart is determined by shaking the left, which is slightly expressed.

additional child survey methods are an electrocardiogram, chest X-ray, cardiac catheterization and angiocardiography cavities (in doubtful cases).

Indications for surgical treatment of isolated pulmonary artery stenosis at an early age are expressed bluish coloration of the skin, cardiac enlargement, heart failure, the pressure difference between the right ventricle and the pulmonary artery over 40 mmHg.Art.