Congenital aortic isthmus stenosis mild .

One. There is no problem with the diagnosis (if the ultrasound is accurate).

Two. Coarctation of the aorta can be intervened**, but if your pressure difference is too large and usually more than 20 mmHg, surgery should be considered.

Three. In general, coarctation of the aorta requires surgery**.

In recent years, there have been cases of severe coarctation of the aorta, and the use of stent implantation has also been good.

In principle, once coarctation of the aorta is diagnosed, surgery should be considered to relieve the aortic obstruction. Surgery** is the fundamental way to completely remove coarctation of the aorta. It is generally believed that a pressure gradient of more than 20 mmHg between the two ends of the coarctation is considered to be an indication for surgery.

Congenital subaortic stenosis accounts for 3% to 8% of cases of congenital left ventricular outflow tract obstruction, second only to aortic stenosis, and has two common types: localized subvalvular stenosis, which accounts for 70%, and includes subdiaphragmatic stenosis, which is stenosis caused by a fibrous diaphragm under the aortic valve, and fibromyobular septal stenosis (limited stenosis caused by fibrous septum with fibromuscular thickening). Diffuse subvalvular aortic stenosis is a tubular stenosis caused by diffuse thickening of the left ventricular outflow tract muscle, also known as tunneled subvalvular stenosis.

The disease is often combined with other malformations such as patent ductus arteriosus, coarctation of the aorta, aortic arch disconnection, ventricular septal defect and aortic valve malformation, and aortic stenosis is the most common, accounting for 50% to 60%.

The incidence is roughly equal for men and women, and the symptoms are similar to those of aortic stenosis, but few symptoms occur in infants and young children, and the symptoms are mostly in children, but some are as late as 20 or 30 years of age. Chest pain and syncope are common symptoms after exercise, and sudden death in infancy and early childhood can occur in patients with aortic and branch stenosis; Sudden death can occur in all age groups if there is stenosis of the ascending aorta and its branches. Both supravalvular and valvular stenosis are progressive.

Death is usually caused by adulthood, and hypercalcemia in infants and young children can also lead to death. Supraaortic localized stenosis may be familial, Williams syndrome, with a characteristic facial appearance, wide anteriorly, a complete face, a sunken nasal bridge, upturned nostrils, thick lips, a pointed mandible, developmental delay, and intellectual disability. Young children often present with a heart murmur similar in nature to aortic stenosis, but without a click, high in position, conduction into the neck, and often palpable systolic tremor at the jugular vein notch.

Blood pressure in the right upper extremity is often higher than in the left upper extremity. Patients with this disease 2 3 combined with other vascular malformations, such as pulmonary artery stenosis, aortic stenosis, patent ductus arteriosus, mitral regurgitation, etc., should be paid attention to before surgery. Most children have high blood calcium, called hypercalcemia syndrome, which is related to abnormal vitamin D and cholesterol metabolism, no special face, and often has a family history.

A systolic pressure gradient of 50 mmHg across the stenosis segment, or left ventricular hypertrophy and strain on ECG, cardiac enlargement on plain x-ray, or clinical angina, syncope, and cardiac insufficiency, or other severe cardiac malformations are all indications for surgery. The surgical approach should be determined based on the length of ascending aorta stenosis and the type of pathology. In 1961, Kirklin performed the first enlarged aortic plasty with a "teardrop" mesh ** with limited supravalvular stenosis, and achieved satisfactory results.

In 1977, Doty applied a "breeches"-shaped patch, and the incision was extended to the right coronary sinus and the non-coronary sinus in order to relieve the stenosis more completely. Brom's proposed transection of the ascending aorta, resection of stenosis, and three-sinus mesh widening of the aortoplasty, as well as the modification method proposed by Myers et al., are all improvements based on the above principles, but further long-term follow-up is still needed to determine which of the various methods is the most ideal. Surgical modalities for diffuse supravalvular stenosis depend on the extent of the lesion and include aortic mesh widening plasty and left ventricular apical to descending aortic valve duct diversion.

In recent years, it has been found that some patients with congenital aortic stenosis are not very satisfied with the release of left ventricular outflow tract obstruction after surgery. Some scholars advocate ROSS surgery, which uses the autologous pulmonary artery to widen the aortic root and left ventricular outflow tract, and at the same time performs coronary artery grafting to replace the reactivated pulmonary artery with the same artery, which may have a better long-term effect on relieving outflow obstruction. In addition to observing whether the outflow tract obstruction is satisfactory to be relieved, the means of concomitant aortic regurgitation should also be understood, which can be judged by checking the arterial pressure in addition to echocardiography.

Once progressive stenosis or severe insufficiency is found at follow-up, reoperation will be indicated, and re-stenosis or aortic replacement will be required.

Congenital supravalvular aortic stenosis is less common than aortic and subvalvular stenosis. It is associated with dysplasia of the aortic sac and is associated with a range of specific presentations. Williams reported in 1961 that patients with supravalvular aortic stenosis often have mental retardation and special faces, which was later confirmed by some disciplines, so it is called Williams syndrome.

**, the surgical method should be determined according to the length of ascending aorta stenosis and the type of pathology.

Varies depending on the degree of stenosis of the aortic ostium. Mild stenosis, good prognosis, and survival into old age, but can be complicated by subacute bacterial endocarditis. Severe stenosis worsens with age, and death from myocardial hypoxia and ventricular fibrillation is common in childhood.

Deaths due to left ventricular failure are rare. Severe stenosis of the aortic ostium in infancy results in death from heart failure.

The disease** is not clear, but scholars have found that it has a tendency to occur in families. Some cases have idiopathic hypercalcemia in infancy and early childhood, so some scholars have speculated that it is related to vitamin metabolism defects (inherited genes).