persistent arterial trunk

Introduction

Introduction to permanent arterial trunk Perpetual arterial stem refers to the failure to divide the primordial trunk into the aorta and pulmonary artery due to defects in the development of the ball and the ball, leaving a common arterial trunk. Therefore, both the left and right ventricles are ejected to a common arterial trunk. The semilunar valve of the arterial trunk rides over the high ventricular septal defect. The anatomy only sees the total trunk. There is no remnant of the main and pulmonary artery, the systemic circulation, and the pulmonary circulation. Both the coronary blood supply and the coronary blood supply are directly from the arteries. Permanent arterial trunk is an extremely rare complex congenital cardiovascular malformation. The incidence rate is about 0.5%, accounting for about 1 to 3% in the autopsy of congenital cardiovascular. basic knowledge Sickness ratio: 0.05% Susceptible people: no specific population Mode of infection: non-infectious Complications: pulmonary hypertension, patent ductus arteriosus, septal defect, single ventricle, single atrium

Cause

Permanent arterial stem cause

At the 3rd and 4th week of embryonic development, under normal circumstances, the development of the arterial trunk interval divides the total arterial trunk into the ascending aorta and the main pulmonary artery, and the arterial trunk interval spirals from the conical portion to the head end. The aorta is located in the left posterior, the main pulmonary artery is located in the right front, and the arterial trunk interval is connected to the conical interval of the conical portion. It participates in the formation of the septal compartment of the membrane, and closes the interventricular pore. If the heartball is absent or underdeveloped, the interventricular septum is formed. Defect, the arterial trunk is riding over the ventricular septal defect, the semilunar valve of the arterial trunk is usually 3 valves, there may be 2 to 6 leaflet malformations, the arterial catheter is often absent, even if it is functional and not important, the pulmonary artery Can be from the root of the artery, the trunk or the bow part, even the pulmonary artery is not developed, the blood of the pulmonary circulation only comes from the enlarged bronchial artery, so there are various types of permanent arterial trunk, but regardless of the type, systemic circulation, pulmonary circulation, The blood of the coronary arteries comes from the ventricles and arteries.

Prevention

Permanent arterial stem prevention

The disease is a congenital disease, so there is no effective preventive measure. Most of the children die in infancy. The main cause of death is heart failure. Others such as brain abscess, endocarditis, high operative mortality and poor prognosis. .

Complication

Permanent arterial complication Complications pulmonary hypertension, patent ductus arteriosus, atrial septal defect, single ventricle, single atrium

The disease is one of the severe fatal cardiovascular malformations, the prognosis is very poor, if not treated in time, about 75% of patients die within one year of age, delaying the operation time will soon develop into pulmonary hypertension, pulmonary vascular infarction Loss of surgery, and often combined with other malformations, makes the treatment of this disease more difficult, combined with other abnormalities of patent ductus arteriosus, left superior vena cava, complete anomalous pulmonary venous connection, atrial septal defect, single ventricle, single atrium and The aortic arch is interrupted.

Symptom

Perpetual arterial symptoms Symptoms Common symptoms Red blood cell dyspnea purpura finger (toe) Heart enlargement ventricular hypertrophy Heart failure tachycardia pulmonary stenosis ventricular septal defect

Within a few weeks after the baby is born, the pulmonary vascular bed has high resistance, the pulmonary blood flow is small, and the clinical symptoms are not obvious. As the pulmonary vascular bed resistance decreases, symptoms of heart failure and pulmonary infection can occur, and the increase in pulmonary blood flow is often presented. Difficulty breathing, heart failure and tachycardia, pulmonary blood flow decreased with purpura, accompanied by erythrocytosis and clubbing (toe), physical examination: patients with weaker general condition, weight loss, heart rate increased, heart enlargement, The liver is swollen, a single second heart sound is heard in the pulmonary valve area, the third and fourth intercostals of the sternal border are loud, rough systolic murmurs and tremors, and the apical area of the valvular insufficiency has early or mid-diastolic diastolic Noise, arterial dry valve insufficiency often has water pulse, chest radiograph shows increased heart shadow, pulmonary vascular texture increased, mainly ventricular enlargement, ascending aorta significantly widened, strong beat without pulmonary artery, about 25% of cases For the right aorta, the origin of the pulmonary artery is higher than normal. If the branch of the artery is as high as the level of the aortic arch, there is a diagnostic value. ECG: left and right heart when the blood is increased. Hypertrophy, pulmonary vascular resistance increased, right ventricular hypertrophy when pulmonary blood is low, echocardiography: see arterial trunk riding over the ventricular septal defect, common left atrium, left ventricle, arterial dry valve can be thickened, cardiac catheterization: Right ventricular pressure increased, left and right ventricular systolic pressure were similar, pulmonary artery and arterial dry pressure were similar, cardiac catheter can enter the aortic arch arm branch from the right ventricle, and angiography showed a single arterial trunk to straddle the ventricular septal defect, only There is a group of semilunar valves, the coronary arteries and pulmonary arteries originate from the arterial trunk.

Hemodynamics: permanent arterial dry cases, blood from the left and right ventricles all enter the arterial trunk, venous blood and left ventricular jets of oxygen from the pulmonary circulation and right ventricular jets of blood from the systemic circulation are mixed into the arterial trunk, so The degree of blood oxygen saturation reduction depends on how much blood flow in the pulmonary circulation is more than the clinical blood purpura is not obvious or mild, but the heart load is aggravated with arterial dry valve closure is not easy to cause heart failure, left atrial pressure Elevation can cause pulmonary edema, pulmonary blood flow is less, and purpura is obvious, resulting in decreased pulmonary blood flow. The most common cause is that pulmonary blood vessel bed undergoes massive blood flow of systemic high blood pressure and gradually produces pulmonary small vessel obstructive lesions, resulting in increased pulmonary circulation resistance. Reduced blood flow, pulmonary stenosis can also reduce pulmonary blood flow but is relatively rare.

Examine

Permanent examination of the arterial trunk

First, X-ray chest

X-ray chest X-ray shows that there is obvious heart enlargement and pulmonary congestion in infants and young children. The main pulmonary artery segment is absent, but the beginning of the left pulmonary artery is often obvious (senior children can see the "comma" sign in the upper left mediastinum). When the isolated right pulmonary artery originates from the left side of the arterial trunk, a similar sign can occur. When the blood supply to one side of the pulmonary artery originates from the bronchial artery or the small patent ductus arteriosus, the lateral thorax can be smaller and the blood vessel The distribution is less, and when the concurrent aortic arch is interrupted, the descending aorta in the chest radiograph is often more pronounced.

Second, the ECG

Often shown as left, right ventricular hypertrophy, mostly left ventricular hypertrophy, normal or mild right deviation.

Third, echocardiography

Echocardiography can confirm the diagnosis, and can distinguish the type of permanent arterial trunk, determine the size and position of the ventricular septal defect, can show that a single vessel rides over the interventricular septum, and shows abnormalities of the arterial valve, such as leaflets The number, the presence or absence of stenosis and incomplete closure.

Fourth, cardiac catheterization and cardiovascular angiography

It can provide information on pulmonary artery, aorta and hemodynamic status. Cardioangiography can show the origin, size and branch of the pulmonary artery. Inject the contrast agent into the root of the artery to visualize the coronary artery and understand the distribution of coronary artery. In the case, right heart catheterization showed an increase in blood oxygen content in the right ventricular outflow tract, and right ventricular pressure was equal to systemic arterial pressure. If the catheter can directly enter the left and right pulmonary arteries to measure pressure and blood oxygen content, then determine the location, direction and pulmonary vessels of the shunt. Resistance is helpful.

Diagnosis

Permanent diagnosis of arterial trunk

Differential diagnosis

Clinically, it should be identified with the following diseases:

(1) Severe tetralogy of the pulmonary artery atresia

Immediately after birth, the sputum and heart failure, X-ray examination showed pulmonary ischemia, right atrium, ventricular enlargement, ECG showed right atrium, room hypertrophy, right heart catheterization can get right atrium, increased room pressure, Doppler echocardiography Figures and cardiovascular angiography can confirm the diagnosis.

(B) the main, pulmonary artery septal defect

Similar to the type I of the permanent arterial trunk, echocardiography can be seen in the valves of the two groups of aorta, and aortic angiography can show the location of two large arteries and septal defects.

(three) dislocation of the great artery

After birth, cyanosis and heart failure are present. If the atrial septal defect or ventricular septal defect is accompanied by mild purpura, the symptoms appear later. X-ray examination shows that the heart is enlarged in egg shape. Cardioangiography shows that the aorta originates from the right ventricle. The main pulmonary artery originates from the left ventricle.

(four) tricuspid atresia

The right atrium is enlarged, the left ventricle is hypertrophied, the electrocardiogram shows the axial center to the left, and the permanent arterial trunk is common to the right ventricular hypertrophy. Doppler echocardiography and cardiovascular angiography can be seen in the tricuspid atresia.

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