complete dislocation of the great arteries
Introduction
Introduction to complete aortic dislocation The meaning of complete aortic dislocation is that the two aortic positions are misplaced. The aorta receives venous blood from the right ventricle, while the pulmonary artery receives oxygen from the left ventricle, thus forming two isolated circulatory systems, the right atrium. Right ventricle aorta whole body body vein right atrium is a cycle; left atrium left ventricle pulmonary artery lung pulmonary vein left atrium is another circulatory system. The ventricular position is normal and the aortic opening is located on the right side of the pulmonary artery. It is called right axillary aortic dislocation (D-TGA). D-TGA is a common clinical type, often accompanied by atrial septal defect, ventricular septal defect, patent ductus arteriosus, Pulmonary artery stenosis, atrioventricular tube malformation, etc. basic knowledge The proportion of illness: 0.0009% Susceptible people: no special people Mode of infection: non-infectious Complications: cerebral hemorrhage, cerebral embolism, heart failure, respiratory failure, atrioventricular block
Cause
Complete aortic dislocation
Cause:
Complete aortic dislocation (TGA) is due to the fifth to seventh week of embryonic development, mediastinal torsion or non-helical torsion, resulting in the transposition of the main and pulmonary arteries, so the body and lung circulation become independent cycles, that is, the veins of the body veins. The blood flows back into the right atrium, the right ventricle, and reaches the various tissues and organs of the whole body through the aorta. The arterial blood of the pulmonary veins flows back into the left atrium, and the left ventricle passes through the pulmonary artery and reaches the lungs. Therefore, the child is difficult to survive, such as combining other cardiac malformations. The shunt channel between the two cycles can exchange a small amount of mixed blood to temporarily maintain life, and the flow rates on both sides are different. Repeatedly, it can cause pulmonary hypertension, obstruction, ventricular dilatation, hypertrophy, heart failure and death.
Prevention
Complete aortic dislocation prevention
Complete aortic dislocation without ventricular septal defect, poor prognosis, about 80 to 90% of cases died within 1 year of age, about 45% died within 1 month without surgery, 69% died within 3 months, 75% Within 8 months of death, 80% died within 1 year of age. In recent years, due to the development of infant heart surgery in China, the Department of Cardiology of Pediatrics attaches great importance to the diagnosis and treatment of aortic dislocation, which has increased the survival rate of infants after birth. Cardiac surgery has created conditions for radical surgery. Common causes of death without surgery are heart failure and pulmonary infection, hypoxia, cerebral hemorrhage, and erythrocytosis, which cause cerebral embolism. Common causes after surgery are heart failure, low-discharge synthesis. Signs, respiratory failure and complete atrioventricular block.
Complication
Complete aortic dislocation complications Complications, cerebral hemorrhage, cerebral embolism, heart failure, respiratory failure, atrioventricular block
Complete aortic dislocation is common with ASD, VSD, right ventricular outflow tract stenosis, PDA, pulmonary stenosis, aortic coarctation, left ventricular dysplasia, coronary artery origin and abnormal walking, etc., the common cause of death without surgery is heart Failure and lung infection, hypoxia, cerebral hemorrhage, and erythrocytosis cause cerebral embolism. Common causes after surgery are heart failure, low-displacement syndrome, respiratory failure, and complete atrioventricular block.
Symptom
Symptoms of complete aortic dislocation common symptoms dyspnea qi ventricular septal defect heart failure systolic murmur heart enlargement cyanotic vascular malformation
Clinical classification: According to coexisting cardiovascular vascular malformations, the disease can be divided into four types:
Type I: ventricular septum intact but with atrial septal defect or patent foramen ovale (may have patent ductus arteriosus), accounting for about 50%.
Type II: with ventricular septal defect (may have patent foramen ovale or patent ductus arteriosus), accounting for about 25%.
Type III: with pulmonary stenosis and / or ventricular septal defect (may have patent foramen ovale or patent ductus arteriosus), accounting for about 10%.
Type IV: with ventricular septal defect and pulmonary vascular obstructive lesions or other malformations, accounting for about 15%.
Clinical manifestations: dyspnea, purpura, progressive heart enlargement and early heart failure. Due to different types of lesions, the degree of pulmonary congestion and blood circulation of the pulmonary circulation are different, and the symptoms and time of occurrence are also different.
Type I infants appear hypoxia, purpura, shortness of breath, acidosis and heart failure at birth or within a few days. They can hear systolic jet murmurs and often die of severe hypoxemia within a few days after birth.
Type II has a late onset of symptoms, and shortness of breath occurs within weeks or months after birth. Patients with large ventricular septal defect with purpura and congestive heart failure have a large amount of pulmonary circulation, enlarged heart, enlarged liver, and often rough at the lower left sternal border. Full systolic or jet systolic murmur.
Type III with pulmonary valve, annulus or subvalvular stenosis, decreased pulmonary blood flow, delayed pulmonary hypertension and pulmonary vascular obstructive disease, late onset, clinical manifestations similar to tetralogy of Fallot, with purpura, hypoxia And acidosis, but heart failure is rare.
Type IV is generally characterized by pulmonary vascular obstructive disease due to pulmonary hypertension after 1 year of age, showing dyspnea, heart failure and progressive purpura. In addition to systolic murmur, the second sound of the pulmonary valve is often hyperthyroidism.
Examine
Complete aortic dislocation examination
Chest X-ray examination: the heart size is normal at birth, the future is increasing, the pulmonary vascular pattern is increased, the outline of the heart is obliquely egg-shaped, because the aorta, pulmonary artery shadow overlap before and after, so the upper mediastinum is small, unless accompanied by pulmonary artery Narrow, generally pulmonary vascular stenosis increased, there is a large ventricular septal defect with pulmonary hypertension, the heart is significantly enlarged, pulmonary vascular shadow increases and can present pulmonary edema.
Electrocardiogram examination: electric axis electrical deviation, right ventricular hypertrophy, and ventricular septal defect or patent ductus arteriosus, left and right ventricular hypertrophy and myocardial damage.
Echocardiography: The transverse section of the aortic root shows the pulmonary artery in the left posterior, the aorta in the right front, the pulmonary artery originating in the left ventricle is divided into two left and right, the aorta originates from the right ventricle, and the pulmonary valve is more open than the aortic valve. Late and late.
Right heart catheterization: the catheter is inserted into the right atrium through the femoral vein, and the right ventricle enters the aorta. It can also enter the left atrium through the foramen ovale in the right atrium and enter the pulmonary artery through the left ventricle. The right ventricular systolic pressure is close to the systemic pressure. The blood oxygen saturation in the aorta is low.
Right ventricular angiography: Immediate development of the aorta, if there is a ventricular septal defect, not only can show its size and position, but also the left ventricle and pulmonary artery are also developed.
Diagnosis
Diagnosis and diagnosis of complete aortic dislocation
Differential diagnosis
(a) tetralogy of Fallot
The second sound of the pulmonary artery was weakened, the X-ray showed lung ischemia, the heart shadow showed a shoe-shaped enlargement, the second artery of the aortic dislocation was normal or hyperactive, the pulmonary vasculature increased, the heart expanded, echocardiography, and cardiovascular angiography confirmed the diagnosis.
(2) Permanent arterial trunk
Echocardiography showed that the arterial trunk straddles the ventricular septum, the right heart catheter examines the left and right ventricular pressures equally, and the cardiovascular angiography shows that the single arterial trunk is above the interventricular septum, and the coronary arteries and pulmonary arteries originate from the arterial trunk.
(C) complete type of pulmonary venous connection
The clinical symptoms of the two are similar, the children are poorly developed, and there are different degrees of purpura. When the chest is filmed, the patients with complete venous connection of the pulmonary veins show the "8" sign; the ultrasound shows that the pulmonary veins form a common venous trunk, which is not connected with the left atrium. From the vertical vein into the left innominate vein, often with ASD.
(4) Complete type of atrioventricular septal defect
The sick child has a diverticulum in the atrioventricular plane. When the common atrioventricular valve is closed and closed, the symptoms appear early, uncontrollable heart failure occurs, the heart enlarges obviously, the severe pulmonary hypertension occurs prematurely, and the echocardiogram shows the atrioventricular septal defect. The cross disappears and forms a common anterior and posterior lobes.
