Coarctation of the Aorta in Children

What is Coarctation of the Aorta in Children?

Coarctation of the aorta in children is a limited narrowing of the aortic lumen, which causes hypertension in the vessels of the upper extremities, hypoperfusion of the abdominal cavity and lower extremities, and left ventricular hypertrophy. Symptoms depend on how narrow the lumen is. In mild cases, chest pain, headache, coolness of the arms and legs, and weakness may occur. In the most severe cases, there is a chance of fulminant heart failure and shock. A soft noise is recorded above the narrowing point when listening.

Complications of aortic coarctation:

  • stroke
  • severe arterial hypertension
  • left ventricular heart failure with the development of cardiac asthma and pulmonary edema
  • subarachnoid hemorrhage
  • expanded aneurysm breaks
  • hypertonic nephroangiosclerosis
  • infectious endocarditis (difficult to treat with antibacterial drugs)

Causes of Coarctation of the Aorta in Children

Coarctation of the aorta is caused by a violation of the process of aortic formation, when the fetus still develops in the womb. Coarctation in most cases is located at the level of the ductus arteriosus. It is believed that some part of the duct tissue moves to the aorta, thereby involving the aortic wall in its closure process, which subsequently leads to its narrowing.

Children with Shereshevsky-Turner syndrome are genetically predisposed to the disease in question. This is a chromosomal disease that is caused by the presence of only one sex X chromosome and the absence of a second.

Pathogenesis during Coarctation of the Aorta in Children

With coarctation of the aorta, the presence of a mechanical obstruction (called a sluice) in the bloodstream in the aorta leads to the formation of two different circulatory regimes. Above the airlock, blood pressure is higher than normal, an expansion of the vascular bed is observed; the left ventricle hypertrophies due to systolic overload. Below the gateway, the pressure is below normal, blood flow compensation is carried out due to the development of multiple bypass paths of blood supply. The degree of impaired blood dynamics depends on the length and severity of the gateway and on the type of coarctation.

There is an adult and children’s type of coarctation. In adults, the ductus arteriosus is closed. In the vessels of the upper body, the pressure is increased – both systolic and diastolic. The work of the left ventricle sharply increases, the mass of circulating blood increases. In arteries below the belt, pressure is lowered, pressure is reduced, which causes the renal mechanism to increase blood pressure. Because of this, the pressure in the upper torso rises even more.

The children’s type is characterized by an open arterial duct. In the vessels of a large circle of blood circulation, blood pressure can be slightly increased, and bypass circulation is poorly developed. In post-ductal coarctation of the aorta, blood is released under high pressure from the aorta through the open arterial duct into the left pulmonary artery, which can early lead to the development of pulmonary hypertension. In preductal coarctation of the aorta, blood is discharged from the pulmonary trunk to the descending aorta through the open arterial duct. With a child’s type of coarctation, a child may have heart failure at a very young age.

Symptoms of Coarctation of the Aorta in Children

As noted above, there are two types of coarctation of the aorta in children:

  • adult
  • children’s

The children’s type is considered less favorable than the adult, because with it early high pulmonary hypertension forms. Above the narrowing point, pressure rises, which affects the physique of the baby. The body becomes “athletic” – the shoulder girdle is developed more than the lower part of the body. Complaints of nosebleeds and headache appear.

The development of morphological changes in the coronary arteries, disorders of cerebral circulation or hemorrhage in the brain, and secondary fibroelastosis of the endo- and myocardium of the left ventricle are likely. If the newborn has a significant narrowing, there may be circulatory shock with renal failure and metabolic acidosis. Symptoms in such cases resemble those with systemic diseases, for example, with sepsis.

If the narrowing is poorly expressed, then in the first year of life, symptoms may not appear. Nonspecific symptoms of aortic coarctation in children are also distinguished:

  • chest pain
  • headache
  • lameness during physical exertion>
  • weakness

They can be aggravated with the growing up of the child. Often there is hypertension. In rare cases, there may be a rupture of the cerebral aneurysm, which results in subarachnoid or intracranial hemorrhage.

Diagnosis of Coarctation of the Aorta in Children

To diagnose coarctation of the aorta in children, the following instrumental methods are used:

  • ECG
  • phonocardiography
  • echocardiography
  • radiography of the heart and lungs
  • cardiac catheterization
  • aortography

ECG is a method that stands for electrocardiography. An ECG in older children shows isolated signs of increased muscle mass in the left ventricle. Phonocardiography is a method of recording vibrations and sound signals that blood vessels and the heart emit while carrying out their activities. One of the signs of aortic coarctation in children, determined using this method, is an increase in tone II on the aorta.

Echocardiography is a method that is otherwise called ultrasound of the heart; it makes it possible to fix a narrowing of the aorta in the area of ​​its isthmus. Using this method, both direct and indirect signs of aortic coarctation in children are detected. X-ray of the chest organs makes it possible to determine that the apex of the heart is elevated, the ascending aorta is dilated, and other signs of the disease in question.

Cardiac catheterization allows you to measure pressure in the aorta and record the difference in systolic blood pressure above and below the blood stream at the site of aortic narrowing. Aortography shows the level and degree of narrowing of the aortic lumen.

Treatment of Coarctation of the Aorta in Children

With coarctation of the aorta in children, treatment may consist in surgical intervention. An indication for this method is the difference in systolic pressure measured on the arms and legs, exceeding 50 mm RT. Art. For infants, the indication for surgery will be severe arterial hypertension and decompensation of cardiac activity.

If the disease is detected within a few months after birth, but the course is favorable, then the operation is delayed until the child is five to six years old. The operation can be carried out to the patient at an older age, but its results will not be so good due to the possibility of preserving arterial hypertension.

Today, several methods of surgical treatment of aortic coarctation are relevant – resection and reconstruction of the aorta:

  1. Aortic surgery using vascular prostheses
    This method of treatment is used if the aorta is narrowed over a large area, because of which there is no way to match the ends of normal sites.
  2. Resection of coarctation of the aorta with the imposition of anastomosis (anastomosis) end to end
    This treatment method is relevant if the site is narrowed to a small extent
  3. Shunting of a pathologically narrowed section of the aorta
    A vascular prosthesis is made from a special material. Its edges are sutured below and above the narrowing area to create a path for the flow of blood.
  4. Aortoplasty using the left subclavian artery of the operated child
  5. Balloon angioplasty and stenting of aortic coarctation
    The method is relevant if the child has had surgery before, but a second narrowing has occurred. A balloon is inserted into the aorta, which swells, eliminating the narrowing. In some cases, it may be necessary to install stents that support the required diameter of the aorta.

Prognosis for coarctation of the aorta in children

The prognosis is different depending on the measure of narrowing of the aorta in the child. If coarctation of the aorta is insignificant, then the child has no obstacles to a normal lifestyle, and life expectancy is not shortened – on average it is the same as in people without the diagnosis in question.

If coarctation of the aorta is significant, then life expectancy, if surgery is not performed, will be on average from 30 to 35 years. Patients die from bacterial endocarditis, severe heart failure. Sudden death may occur due to rupture of the aorta or its aneurysm and stroke.

Prevention of Aortic Coarctation in Children

Before conceiving a child, the father and mother must give up such bad habits as alcohol, smoking and drugs.