Supra-aortic diaphragmatic stenosis

Introduction 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. The stenotic lesion is located above the opening of the coronary artery. Aortic stenosis lesions are more common, accounting for about 90%.

Cause

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 with the left coronary valve, and the left coronary artery blood flow is obstructed, and the aortic valve leaf may be thickened. The ascending aorta has a normal appearance and is not accompanied by a stenosis and enlargement. Another type of localized stenosis of the stenosis, the ascending aorta is narrow in the stenosis, showing a sand-absorbent or "8" shape, where the aortic wall is thickened, the intima is thickened, and the tissue is thickened. The examination of the lesion is similar to aortic constriction.

Extensive aortic stenosis is rare, with stenosis extending from the ascending aorta above the ascending aorta and the origin of the innominate artery, and even invading the aortic arch.

Aortic stenosis is often accompanied by coronary artery tortuosity and coronary sinus enlargement, which can have multiple stenosis of the surrounding pulmonary artery, such as pulmonary stenosis, common pulmonary artery dysplasia, aortic arch branch stenosis, aortic coarctation or ventricle Interval defect.

Type of aortic stenosis:

(1) The aortic root is annularly narrowed.

(2) aponeurotic stenosis on the aortic valve.

(3) long ascending aortic dysplasia.

an examination

Related inspection

Two-dimensional echocardiography, cardiac vascular ultrasound, electrocardiogram

In most cases, the symptoms of aortic stenosis appear in childhood. Because coronary atherosclerotic lesions occur earlier, angina is more common. Some patients have a family history.

Signs are similar to other types of aortic outlet stenosis, but no systolic clicks are heard. The location of heart murmurs and tremors is higher than that of the stenosis of the valve, and aortic diastolic murmurs are rare. Some patients have poor growth and development, short stature, low intelligence, many words, and have a special face: mandibular retraction, nostril leaning forward, low nose bridge, thick lip, wide forehead, large eye distance, poor tooth occlusion. About 5% of patients have elevated blood calcium.

X-ray examination and ECG examination showed signs similar to other types of aortic outlet stenosis.

Cardiac catheterization: Left heart catheterization and continuous recording of the pressure curve may reveal a change in the pressure waveform above the aorta.

Selective left ventricular angiography can show the location, length and severity of the stenosis on the valve. The same can be seen to see if the shape and function of the aortic valve are normal, as well as the coronary sinus and coronary artery. Right heart angiography shows whether the common pulmonary artery and its branches also have lesions.

Sectional echocardiography: directly shows the location and length of the stenosis on the valve.

Aortic stenosis, especially in cases with mental retardation, special face and extensive stenosis of the pulmonary artery, often causes sudden death in the early years due to severe obstruction of the left ventricular outflow tract and coronary artery disease. Cases without surgery can rarely grow into adulthood.

Differential diagnosis

1. Identification of rheumatic congenital and degenerative aortic stenosis in the elderly.

2. The differential diagnosis of congenital aortic stenosis is divided into three types: aortic stenosis, subaortic stenosis and aortic stenosis. The most stenosis of the valve, less stenosis under the valve, and the stenosis of the valve is the least common.

3. Pulmonary stenosis aortic stenosis and pulmonary stenosis.

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