Coarctation of the aorta is what? Coarctation of the aorta in children

Coarctation of the aorta is a heart defect, which is accompanied by a narrowing of the lumen of the largest vessel in the human body. In fact, this is not a heart disease, since pathology is localized outside the boundaries of the myocardium. Most often, coarctation of the aorta in newborns is diagnosed, although sometimes the ailment is detected already in adult patients. Most often, the ailment is eliminated by surgical intervention.

Of course, this diagnosis is not entirely understandable to patients and causes them to panic. Therefore, it is worthwhile to find out more about what this disease is and what complications a sick person may face.

Coarctation of the aorta: what is it?

To begin with it is necessary to understand the meaning of the term. Coarctation of the aorta is a congenital disorder that is accompanied by a narrowing of the aorta. In this case, the structure of the heart in patients is quite normal. Nevertheless, the aorta is the largest vessel of man, and the disturbance of blood flow in this area negatively affects the work not only of the heart and blood vessels, but of the whole organism.

As a rule, constriction is formed in the place where the aortic arch passes into its downward part. This pattern is understandable, because there is a normal physiological contraction in the norm. By the way, for the first time this affliction was described in 1791 by D. Morgani (Italian pathologist). According to statistical data, the frequency of pathology is up to 15% of all congenital malformations. It is also interesting that in boys the disease is observed 3-5 times more often than in female patients.

The main types of pathology

Depending on the characteristics, coarctation of the aorta can be of two types:

• "Adult" coarctation is characterized by a narrowing of the aortic lumen below the point where the left subclavian artery departs from it ; With the closure of the arterial duct;
• The infantile type of pathology is accompanied by aortic hypoplasia at the same site of the subclavian artery, but the duct remains open.

Anatomical features of pathology

Congenital coarctation of the aorta can be accompanied by various concomitant pathologies. Depending on this, vice is divided into three groups.

Pathology can be isolated - while there are no other vices in the development of the cardiovascular system. Often coarctation of the aorta in children can be combined with other pathologies - it can be an aneurysm, aortic stenosis, a defect of the septum between the ventricles and atria, a transposition of the main vessels.

The third group is coarctation, in which the opening of the arterial duct is observed. In such cases, the pathology may be:

• Post-ductal (the constriction is located below the point where the open arterial duct departs;
• Yukstaductal (coarctation is located just at the level of the open duct);
• Preductal (arterial duct opens below the site of constriction).

Of course, the symptomatology largely depends on the type and characteristics of coarctation.

What are the causes of the development of vice?

Most often, coarctation of the aorta in the fetus develops. Why is this happening? As is known, during the intrauterine development in the child's body, the arterial duct functions, which connects the aorta and the left pulmonary artery. This structure is only needed temporarily. After birth and the onset of pulmonary respiration, the duct closes.

There is a theory that for one reason or another the child has a small part of the tissues of this duct moving into the aorta, so when the duct is closed, the aortic wall is also involved in the process, which leads to its narrowing.

The reasons for this process, alas, are unknown. Scientists only managed to find out that the patients with chromosomal syndrome of Shereshevsky-Turner (the presence of only one sex chromosome) are most likely to this disease. Practically every tenth child with this diagnosis shows this defect.

Coarctation of the aorta (ICD) does not have to be congenital. There are rare cases in which narrowing of the vessel occurred already in adulthood. In such cases the causes of coarctation were traumas and atherosclerotic lesions of the aorta, inflammatory diseases of the vascular walls of an unclear etiology (Takayasu's syndrome).

Disturbance of hemodynamics during coarctation

Of course, the narrowing of the aorta affects the work of the entire cardiovascular system, even if there are no concomitant defects. The presence of coarctation leads to the formation of two different regimes of blood flow.

Above the site of narrowing, blood pressure is increased, which leads to an expansion of the lumen of all vessels. Because of systolic overload, hypertrophy of the left ventricle develops. And here below the coarctation area the situation is the opposite - the blood flow is slowed down, blood pressure is lowered. With the development of the child's organism, compensatory mechanisms are activated - multiple collateral vessels develop that provide bypass blood flow.

If we are talking about an adult type of blemish, then patients have hypertrophy of the left ventricle, severe hypertension and an increase in the volume of circulating blood. With infantile coarctation, when the arterial duct is open, the above disorders are not so clearly expressed. But there are other pathologies. For example, in the post-pathological type of pathology, blood from the aorta under high pressure is fed directly into the pulmonary artery, which leads to increased pressure in the vessels of the small circle of the circulation.

In any case, there is a serious violation of hemodynamics, which in no case should not be ignored, because the consequences can be terrible.

What symptoms are accompanied by the disease?

Signs of coarctation of the aorta largely depend on the degree of narrowing of the vessel, as well as on the presence or absence of concomitant defects. An easy degree of coarctation may not appear at all.

In more severe cases, the symptoms are noticeable already in the first year of life. Babies often suffer from pneumonia and recurrence. Children have pale skin and trouble breathing - pronounced dyspnea appears during eating, playing games or even at rest. Often children with this diagnosis develop physically more slowly than their peers.

It is possible to suspect the presence of a defect while listening to the heart sounds, and also when determining the pulse in the vessels of the upper and lower extremities. In the arteries on the elbows you can see an intense pulsation, while on the femoral vessels the pulse is palpated very weakly.

With pre-coarctation, there is also a non-uniform cyanosis - in the child the skin on the legs acquires a cyanotic hue, while the skin in the upper part of the body retains a natural color.

Often it happens that pathology is diagnosed already at an older age - in children of school age, adolescents, adult patients. Symptoms in this case include high blood pressure. Patients complain of weakness, headaches, frequent dizziness, pain in the heart, nosebleeds and fatigue. Often during the examination, it is possible to observe a disproportional development of the musculature of the upper and lower parts of the body. Symptoms include weakness in the legs, frequent cramps, chilliness of the feet. Women can observe disorders of the menstrual cycle, and sometimes infertility.

Coarctation of the aorta: diagnosis

To suspect the presence of a vice doctor can during listening to heart sounds. Further, other diagnostic measures are carried out that help not only to establish a fax of the presence of coarctation, but also to detect other defects and determine the degree of damage to the cardiovascular system:

• One of the easiest and most accessible methods is electrocardiography. This is not the most informative technique, since with moderate aortic narrowing, the patient's electrocardiogram can look quite normal. In children in the first year of life during the procedure, you can see the displacement of the electrical axis of the heart. The older children can already detect signs of hypertrophy of the left ventricle. In adult patients, the electric axis of the heart is shifted to the left, and sometimes there is an incomplete blockage of the left branch of the bundle.
• Often patients are prescribed phonocardiography. This procedure allows you to record the sound signals and vibrations generated by the work of the heart and blood vessels. In the presence of coarctation, one can notice a strengthening of the second tone on the aorta, as well as the appearance of systolic noise on the back and in the second intercostal space near the edge of the sternum (from the right and left sides).
• Informative is the ultrasound of the heart, in which it is possible to detect narrowing of the aorta. Also, a specialist can see an increase in the mass of the heart. Doppler echocardiography is used to identify the characteristic symptoms of pathology, in particular, the difference in arterial pressure in areas above and below the narrowing, the presence of turbulent systolic flow.
• In addition, chest radiography is performed. As a rule, the heart size remains normal, but you can notice a significant expansion of the ascending aorta. Pulmonary pattern can be strengthened along the arterial channel, although this is not always the case.
• Aortography is a procedure that involves the introduction of a special contrast agent into the aorta, followed by monitoring its distribution along the vessel. This study allows you to accurately determine the degree and level of constriction of the aorta.
• Cardiac catheterization is a rather complicated procedure. Nevertheless, by introducing a special catheter into the cavity of the vessel, blood pressure can be accurately measured.

Only after receiving a complete picture the doctor can give a forecast and choose the appropriate methods for eliminating this blemish.

Is surgery necessary?

What if the patient has coarctation of the aorta? Operation, of course, is the only effective remedy for the elimination of vice. But the decision to conduct a surgical intervention can be made only by doctors who are familiar with the history and clinical picture.

In some cases (if there is only a slight narrowing, which practically does not affect hemodynamics), the operation may not be required. The patient only undergoes periodic examinations.

If the difference in the systolic pressure of the arteries of the upper and lower extremities exceeds 50 mm Hg. Art., Doctors often suggest a surgical procedure. If coarctation of the aorta in newborns has been diagnosed, surgery (urgent) is performed if small patients have severe hypertension and cardiac decompensation. In those cases when the disease is relatively favorable and there is no great risk to the life of the child, the procedure can be postponed to five or six years of age.

Surgical treatment of heart disease

To date, there are several methods for eliminating this defect. The choice of surgical technique depends on the patient's condition, the form of the disease and the size of the coarctation.

• In some cases, doctors carry out a resection (excision) of the narrowed aorta, then re-connect the ends of the vessel, imposing an anastomosis. This procedure is possible only if coarctation has a small extent.
• In the event that the site of constriction is long, and anastomosis is not possible, prostheses are used. During the procedure, the surgeon removes the affected area of the aorta, after which the two ends of the vessel are connected with a special prosthesis made of synthetic materials.
• Aortoplasty is another kind of operation, only in this case, not a synthetic prosthesis, but part of the left subclavian artery of the patient itself, is used to restore the length of the aorta.
• Sometimes doctors decide to perform aortal bypass surgery. In such cases, a synthetic prosthesis is used, the edges of which are stitched above and below the narrowed portion of the vessel - thus creating a bypass path for blood flow.
• There is one more procedure, called balloon angioplasty. It is carried out in those cases, if after a previous surgical intervention again appeared narrowing of the vessel. During the procedure, the doctor introduces a special balloon through the peripheral vessels into the aortic lumen, which, when inflated, constricts the narrowing. In some cases, special rigid stents are additionally installed, which fix the dimensions of the aortic lumen.

This is how the correction of a malformation called "coarctation of the aorta" looks like. After surgery, the patient, of course, should adhere to certain recommendations. In particular, you need to eat properly, avoid excessive physical exertion and hypodynamia, carefully monitor your health and regularly undergo examinations with the treating cardiologist.

What complications are possible?

Coarctation of the aorta is a dangerous ailment, which in no case should not be ignored. In case of unfavorable course, the vice can lead to extremely dangerous complications:

• Because of the narrowing of the vessel in patients can develop severe hypertension.
• Often, this heart failure - coarctation of the aorta - is also accompanied by the formation of an aneurysm and its further rupture.
• Complications include also stroke and subarachnoidal outpouring of blood.
• Against the background of this disease patients often develop heart failure, which is accompanied by a so-called cardiac asthma and pulmonary edema.
• The narrowing of the aortic lumen affects the work of the entire vascular system. Because of severe hypertension, small arteries of the kidneys can be affected.
• Occasionally, patients develop bacterial endocarditis. A similar complication, as a rule, occurs in the event that coarctation of the aorta is associated with aortic valve pathologies. Unfortunately, in most cases, these bacterial lesions of the heart practically do not respond to antibiotic treatment.

As can be seen, coarctation of the aorta in children and adults can lead to death. That is why timely diagnostics and adequate therapy are so important.

Forecasts for patients with coarctation

Most often, coarctation of the aorta in newborns is diagnosed. What are the predictions for small patients and what should parents expect? In fact, everything depends on the degree of vasoconstriction. In some cases, the ailment requires immediate surgery. If it is a medium-severe degree of blemish, even if all the medical recommendations are followed, patients survive only up to 30-35 years, and the cause of death, as a rule, is a stroke or rupture of an aneurysm.

An easy degree of constriction sometimes does not require surgical intervention - the doctor only recommends periodically to undergo examinations to identify complications. In such cases, the presence of heart disease rarely affects the life span of the patient.