Isolated stenosis of the pulmonary artery
Isolated stenosis( narrowing) of the pulmonary artery is one of the most common congenital heart defects and accounts for 6.8-9% of all heart defects. This pathology is equally common in boys and girls. The first description of the defect refers to 1749.
If there is an obstacle to the release of blood from the right ventricle, then the pressure in it at the time of its contraction( systole) increases significantly( up to 200 mm Hg).As a result, a pressure difference is created between the right ventricle and the pulmonary artery, which ensures the safety of the release of blood into the small circle of the circulation. In children with severe forms of isolated pulmonary artery stenosis, the pressure in the right atrium increases. Against the background of increased pressure in the right atrium, the oval window widens and there is a discharge of the "spent" blood into the left ventricle, i.e., into the large circle of blood circulation. The magnitude of the discharge of blood determines the severity of the cyanotic color of the skin( from crimson to dark blue).
Features of isolated pulmonary artery stenosis entirely depend on the degree of constriction. With "pale" versions of isolated pulmonary artery stenosis, children are well formed, complaints are unimportant and mainly reduce to dyspnoea with exercise, often fingertips, nose, cheeks have a crimson tint. The only manifestation of vice is a rough noise, which is determined by the doctor when he listens to the work of the heart. The intensity and duration of noise are directly proportional to the degree of stenosis. In the "blue" forms of isolated pulmonary artery stenosis( sharp narrowing) the leading signs are cyanotic skin, resistant to treatment of heart failure, including increased liver size and edematous syndrome. Expression of cyanosis of the skin is determined by the amount of discharge of "spent" blood into the large circle of blood circulation at the level of the atria. With age, its intensity increases, the symptom of "watch glass" and "drumsticks" develops, shortness of breath arises at the slightest physical exertion, children complain of pain in the region of the heart. With isolated stenosis of the pulmonary artery, only children with pronounced oxygen deficiency in the blood lag behind in the physical development. Most children develop a right-sided heart hump. When palpation( palpation) of the heart region, the left tremor is determined, which is slightly expressed.
Additional methods of examining a child are ECG, chest X-ray, cardiac catheterization and angiocardiography( in doubtful cases).
Indications for surgical treatment of isolated pulmonary artery stenosis at an early age are marked bluish staining of the skin, increased heart size, heart failure, a difference in pressure between the right ventricle and pulmonary artery of more than 40 mm Hg. Art.