congenital aortic stenosis
Introduction
Introduction to congenital aortic stenosis Aortic stenosis is the least common in congenital aortic stenosis, accounting for about 5 to 10%. Male and female incidence rates are similar. Stenosis of the stenosis may be accompanied by mental retardation, and the stenotic lesion is located above the opening of the coronary artery. Aortic stenosis lesions are more common, accounting for about 90%. basic knowledge The proportion of illness: 0.0035% Susceptible people: no special people Mode of infection: non-infectious Complications: ventricular septal defect
Cause
Congenital aortic stenosis
The cause of this disease is not clear, but scholars have found that there is a family tendency, some cases have idiopathic hypercalcemia in infants and young children, so some scholars have concluded that the cause is related to vitamin metabolism defects (genetic genes).
Prevention
Congenital prevention of aortic stenosis
The etiology of this disease is not clear, so there is no effective preventive measures at present, because the disease often combined with aortic valve malformation, coronary artery dilatation and abnormal opening position, right ventricular outflow tract stenosis and mitral valve disease, preoperatively such patients should be Detailed examination for timely treatment, in the removal of abnormal fibrous fistula or diaphragm in the aortic wall, to avoid damage to the valve and coronary artery, to prevent postoperative residual cardiac malformations and serious complications, postoperative stability should be maintained, control Blood pressure, reduce bleeding in the patch site, maintain stable heart function, prevent arrhythmia, in order to achieve good surgical results.
Complication
Congenital aortic stenosis complications Complications, ventricular septal defect
Often accompanied by coronary artery tortuosity and coronary sinus enlargement, can have multiple stenosis of the surrounding pulmonary artery, such as pulmonary stenosis, pulmonary artery dysplasia, aortic arch branch stenosis, aortic coarctation or ventricular septal defect, etc. If abnormal fibrosis or diaphragm in the aortic wall, if the valve and coronary artery are damaged, serious surgical complications will occur. The postoperative condition should be kept stable, blood pressure should be controlled, and complications of surgical incision bleeding should be prevented. The heart function is stable and prevents the occurrence of arrhythmia.
Symptom
Congenital aortic stenosis symptoms common symptoms tremor ventricular septal defect heart murmur ascending aortic root ring narrowing mandibular retraction angina mental retardation long segment ascending aorta dysplasia aortic calcification atherosclerosis
The most common lesion is a diaphragm-like stenosis above the coronary valve sinus. There is a small hole in the central part of the diaphragm. Sometimes the diaphragm is connected to the left coronary valve, and the left coronary artery blood flow is obstructed. The aortic valve leaf may be thickened. The appearance of the artery is normal, and there is no expansion after stenosis. Another type of limited stenosis on the stenosis, the ascending aorta has a narrow outer diameter at the stenosis, showing a sand-absorbent or "8" shape, where the aortic wall Fibrosis thickening, thickening of the intima, histological examination of lesions similar to aortic coarctation, extensive aortic stenosis is less common, stenosis extends from the ascending aorta above the coronary sinus and the origin of the innominate artery Department, even invading the aortic arch, aortic stenosis often accompanied by coronary artery tortuosity and coronary sinus enlargement, can have multiple stenosis of the surrounding pulmonary artery, such as pulmonary stenosis, pulmonary artery dysplasia, aortic arch branch Narrow, aortic coarctation or ventricular septal defect.
Types of aortic stenosis: (1) annular atrophy of the ascending aorta; (2) aponeurotic stenosis on the aortic valve; (3) ascending aortic dysplasia.
In most cases, the symptoms of aortic stenosis appear in childhood. Because coronary atherosclerotic lesions occur earlier, angina is more common, and some patients have a family history.
Signs are similar to other types of aortic stenosis, but no systolic snoring, heart murmurs and tremors are higher than stenosis, aortic diastolic murmurs are rare, and some patients have poor growth and posture. Short, low intelligence, multi-word, and has a special face: mandibular retraction, nostril leaning forward, low nose bridge, thick lip, forehead width, large eye distance, poor tooth occlusion, about 5% of patients with elevated blood calcium.
Examine
Examination of congenital aortic stenosis
(1) X-ray examination and electrocardiographic examination showed signs similar to other types of aortic outlet stenosis.
(2) Cardiac catheter: Left heart catheterization and continuous recording of the pressure curve may reveal that the pressure waveform changes above the aorta.
(3) section echocardiography: can directly display the location and length of the stenosis on the valve.
(4) Selective left ventricular angiography: showing the location of the stenosis on the stenosis, the length and severity of the stenosis, the same can be seen in the shape and function of the aortic valve, as well as the coronary sinus and coronary artery, right heart angiography It can show whether the common pulmonary artery and its branches also have lesions.
Diagnosis
Diagnosis and diagnosis of congenital aortic stenosis
First, there are two diseases that need to be identified clinically with this disease:
1. Aortic stenosis caused by senile degenerative aortic valve calcification
Common in the elderly, calcification is mostly located at the root of the valve, so the valve leaf activity is still good, no systolic jet sound, X-ray can be seen valvular calcification.
2, primary hypertrophic obstructive cardiomyopathy
Hypertrophic obstructive cardiomyopathy has the following characteristics to help distinguish:
1 The systolic jet murmur is lower, mostly in the III-IV rib of the left sternal border, and is less likely to be transmitted to the neck. The murmur is enhanced when the blood volume is reduced or the ventricular contractility is strengthened, and vice versa.
2 The second heart sound of the aorta is normal.
3 no systolic jet sound.
4 echocardiography showed a ratio of ventricular septum to free wall thickness >1.3:1, left ventricular stenosis, left ventricular outflow stenosis (<20mm), anterior mitral systolic anterior motion, aortic valve The contraction is closed in the middle.
5 cardiovascular angiography, the left ventricular cavity showed a reverse cone change.
Second, the clinical need to identify the aortic valve, valvular and subvalvular stenosis, mainly rely on imaging techniques:
1, aortic stenosis
(1) X-ray plain film shows enlargement of left ventricle, ascending aortic dilatation or aortic valve calcification, which is the main manifestation, combined with systolic murmur in the aortic valve auscultation area, which may indicate aortic stenosis.
(2) MRI is performed by MR spin echo technique. In addition to the individual augmentation of the fixed aortic valve, it is limited to the secondary aortic stenosis caused by left ventricular enlargement, cardiac hypertrophy and ascending aortic dilatation. change.
(3) CAG adopts left ventricular anterior lateral angiography, which is the most effective method for determining the degree of aortic stenosis and the pathological morphology of the valve. The "dome" sign and the "spike" sign all reflect the adhesion between the valves, and the systolic period cannot be opened. Protrusion into the aortic cavity, the latter aortic valve thickening, severe adhesion, loss of elasticity, indicating that the degree of leaflet lesions is more serious than the former, the root width of the "jet" sign reflects the size of the stenosis, echocardiography in The value of diagnosis and differential diagnosis of congenital aortic stenosis has been clinically recognized], because it is simple and easy to be the first choice for clinical diagnosis, rheumatic aortic stenosis, almost all with mitral valve disease, it is not difficult to identify.
2, subaortic stenosis
(1) X-ray plain film showed similar aortic stenosis except for ascending aortic dilatation without obvious stenosis, and there was no specific diagnosis.
(2) MRI using spin echo imaging has important clinical application value in showing the shape and extent of subaortic stenosis. Unlike the aortic valve leaflets, the aortic valve stenosis is slightly wider than the base. The circumference is fixed on the wall of the ventricle, and the activity of the cardiac cycle is not obvious, so it can be displayed on the spin echo image.
(3) A group of CAG data show that echocardiography is superior to CAG in showing the morphology, extent and diagnosis of subvalvular stenosis. Therefore, CAG is generally not the first choice or main method for diagnosis. Hypertrophic cardiomyopathy can cause left ventricular outflow tract. Obstruction, its echocardiography and MRI have characteristic features: the myocardium is generally hypertrophic, mainly ventricular septum, cardiac cavity shrinkage, can be identified with the disease.
3, aortic stenosis
(1) X-ray plain film shows similar aortic stenosis, but the heart enlargement is not obvious, the ascending aorta does not expand, and even in the case of hypoplasia, it can be reduced, lacking diagnostic specific signs.
(2) MRI In the diagnosis of various congenital aortic malformations, the clinical application value of MRI has been recognized.
(3) CAG is the "gold standard" examination method for congenital aortic malformation. It is beyond the reach of other images in judging the degree of stenosis, type, and other abnormalities of the aorta.
