Hypoplasia of long ascending aorta

Introduction The clinical manifestations of congenital aortic stenosis are: (1) annular atrophy of the ascending aorta; (2) aponeurosis of the aortic valve; (3) dysplasia of the ascending aorta. 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%. The disease has a family tendency, and some cases have idiopathic hypercalcemia in infants and young children.

Cause

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).

an examination

Related inspection

Chest radiography Doppler echocardiography

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) Ascending aortic root ring narrowing

(2) Aortic valve aponeurosis

(3) long ascending aortic hypoplasia

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

Aortic ring dilatation: About 50% of patients have horse syndrome or mutations in the disease, and the rest are idiopathic. The proximal aorta and aortic roots widen, causing aortic regurgitation. The disease can be diagnosed according to clinical and examination.

Right aortic arch: Right aortic arch is a more common congenital vascular malformation. The aorta does not cross the left main bronchus from the left ventricle, but extends backwards across the right main bronchus, and descends from the descending aorta. The descending aorta descends along the right side of the spine until it approaches the left side. Patients are often accompanied by other congenital cardiovascular malformations such as tetralogy of Fallot, ventricular septal defect, pulmonary atresia, right atrioventricular atresia, permanent aortic stem and aortic dislocation.

Aortic sacral stenosis: Aortic stenosis is the rarest 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%.

Aortic sinus rupture: Aortic sinus rupture, also known as rupture of the sinus sinus, is due to the lack of normal elastic tissue and muscle tissue in the aortic sinus wall, which is impacted by high-pressure blood flow, gradually forming a cystic tumor, protruding outward It can eventually lead to rupture. This disease is a rare congenital heart disease. The incidence of this disease is high in adults, and the condition suddenly appears and develops rapidly. Clinical manifestations mainly include sudden and severe chest pain, palpitations, shortness of breath, and easily lead to heart failure. The curative effect of this disease is exact, the operative mortality is low, and the normal heart function can be completely restored after operation, but the mortality of severe heart failure is high and the postoperative recovery is slow. Therefore, early surgery should be performed.

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