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

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2018-10-08 05:00:38

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Coarctation of the aorta is a heart defect, which is accompanied by narrowing of the lumen of the major vessel in the human body. In fact, it is not heart disease, as the pathology is localized outside the boundaries of the myocardium. This files most often diagnosed coarctation of the aorta in neonates, although sometimes the disease is found in adult patients. Most often disease removed by surgery.

Of Course, this diagnosis is not entirely clear to patients and makes them panic. So you should learn more about what is this disease and what complications may encounter a sick person.

Coarctation of the aorta: what is it?

coarctation of the aorta is a

To get started is to understand the meaning of the term. Coarctation of the aorta is a congenital defect, which is accompanied by a narrowing of the aorta. The structure of the heart patients are quite normal. However, the aorta is the largest vessel of the person and a violation of blood flow in this region has negative effects on not only the heart and blood vessels, but also the entire body.

Usually, the narrowing is formed in the place where the aortic arch becomes the descending part of it. Such a pattern is understandable, because here the norm is a small physiological narrowing. By the way, for the first time this disease was described in 1791 D. Morgagni (Italian pathologist). According to statistics, the frequency of pathology in up to 15% of all birth defects. Interesting fact that in boys, the disease occurs in 3-5 times more often than in patients of the female sex.

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Main types of pathology

coarctation of the aorta in children

Depending on the features, coarctation of the aorta may be of two types:

  • “adult” coarctation is characterised by narrowing of the lumen of the aorta below the place where it leaves the left subclavian artery; in this case there is closure of the ductus arteriosus;
  • Infantile type of pathology is accompanied by hypoplasia of the aorta in the same place of origin of the subclavian artery, but the duct remains open.

Anatomical features of the pathology of

Congenital coarctation of the aorta may be accompanied by different comorbidities. Depending on this defect can be divided into three groups.

The disease can be isolated — there are no other defects in the development of the cardiovascular system. Often coarctation of the aorta in children may be combined with other abnormalities — it could be an aneurysm, aortic stenosis, defect of the septum between the ventricles and Atria, transposition of the great vessels.

The Third group — coarctation, in which there is the opening of the ductus arteriosus. In such cases, the pathology may be:

  • Postductal (a narrowing is below the place where leaves patent ductus arteriosus;
  • Juxtaductal (coarctation is located just at the level of an open duct);
  • Productline (arterial duct opens below the narrowing).

Of Course, the symptoms largely depends on the type and characteristics of the coarctation.

What are the causes of defect?

coarctation of the aorta in newborns

Most Often develops coarctation of the aorta in the fetus. Why is this happening? As you know, in the period of intrauterine development in the child's body is functioning arterial duct that connects the aorta and the left pulmonary artery. This structure is only needed temporarily. After the birth and the beginning of pulmonary respiration, the arterial duct closes.

There is a theory that for whatever reasons the child a small portion of the tissue of this duct is moved into the aorta, so when you close the duct in the process involved and the aortic wall, leading to its narrowing.

The Reasons for this process, alas, unknown. Scientists only found out that the disease most likely patients with a chromosomal syndrome Shereshevsky-Turner (having only one sex chromosome). Almost every tenth child with this diagnosis is detected the fault.

Coarctation of the aorta (ICD) does not have to be innate. Known rare cases in which the narrowing occurred in adulthood. In such cases, the causes of coarctation was injury and atherosclerotic lesions of the aorta, inflammatory disease of the vascular walls of unknown etiology (Takayasu's syndrome).

Violation of hemodynamics in coarctation

Of Course, aortic stenosis affects the entire cardiovascular system, even if the attendant evils is not available. The presence of coarctation leads to the formation of two different modes of blood flow.

Above the narrowing of the arterial pressure is increased, which leads to the extension of the lumen of all vessels. Due to systolic overload develops left ventricular hypertrophy. But below the area of coarctation, the situation is the opposite — blood flow is slowed, blood pressure is lowered. With the development of child's organism activate compensatory mechanisms — develop multiple collateral vessels that provide a bypass blood flow.

If we are talking about adult type of defect, patients present left ventricular hypertrophy, severe hypertension and increased blood volume. In infantileof coarctation when the ductus arteriosus is open, the above disorders are expressed not so clearly. But there are other pathology. For example, when postlactational the type of pathology of blood from the aorta under high pressure is supplied directly into the pulmonary artery, which leads to increased pressure in the blood vessels of the pulmonary circulation.

In any case there is a serious violation of hemodynamics, which in any case should not be ignored, because the consequences can be dire.

What symptoms accompany the disease?

coarctation of the aorta in neonates surgery

Signs of coarctation of the aorta is largely dependent on the degree of narrowing of the vessel, and the presence or absence of attendant evils. A slight degree of coarctation may not be evident.

In more severe cases, the symptoms are noticeable the first year of life. Kids often suffer from pneumonia and constant relapses. Children have pale skin and breathing problems — severe shortness of breath appears during eating, playing or even at rest. Often kids with this diagnosis develop physically slower than their peers.

To Suspect the presence of the defect while listening to the heart tones, as well as in the determination of the pulse in the vessels of the upper and lower extremities. In the arteries at the elbow, you notice the tense ripple, while on the femoral vessels, the pulse is very weak.

When productline of coarctation is observed inhomogeneous cyanosis — the baby skin on the feet becomes bluish tint, while skin in the upper part of the body retain their natural color.

Often it happens that pathology already diagnosed at an older age — from school-age children, adolescents, and adult patients. The 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 inspection it is possible to observe a disproportionate development of muscles of the upper and lower parts of the body. Symptoms include weakness in the legs, frequent cramps, chilliness of the feet. Women can be observed disorders of the menstrual cycle, and sometimes infertility.

Coarctation of the aorta: diagnosis

coarctation of the aorta diagnosis

To Suspect the presence of the defect, a doctor may while listening to the heart tones. Further, there are other diagnostic measures that help not only to set the Fax machine to the presence of coarctation, but also to detect other defects and to determine the degree of damage of the cardiovascular system:

  • One of the simplest methods is electrocardiography. It's not the most informative method since with moderate aortic stenosis electrocardiogram of the patient might look absolutely normal. The children in the first year of life during the procedure you may notice the offset of the electrical axis of the heart. The guys are older, it is already possible to detect signs of left ventricular hypertrophy. In adult patients, the electrical axis of the heart shifts to the left, and sometimes there is incomplete blockade of the left bundle branch block.
  • Often patients are prescribed the phonocardiography. This procedure allows you to record audible signals and vibrations generated during operation of the heart and blood vessels. In the presence of coarctation is possible to notice the strengthening of the second tone on the aorta, as well as the appearance of systolic murmur at the back and in the second intercostal space at the edge of the sternum (the right and left sides).
  • Informative is ultrasound of the heart, which can detect narrowing of the aorta. Also the specialist can see an increase in mass of the heart. Doppler echocardiography is used to identify the characteristic symptoms of the pathology, in particular, the difference in blood pressure in areas above and below the narrowing, the presence of turbulent systolic flow.
  • Additionally performed x-rays of the lungs and heart. As a rule, the heart size remains normal, but you can see a significant expansion of the ascending aorta. Pulmonary drawing can be enhanced in the arterial line, although this has not been the case always.
  • Aortography — a procedure which involves the insertion into the aorta of a special contrast medium with subsequent monitoring of its distribution on the vessel. This study allows to accurately determine the extent and degree of narrowing of the aorta.
  • Catheterization — a rather complicated procedure. However, by introducing a special catheter into the cavity of the vessel can accurately measure blood pressure.

Only after a complete picture, the doctor can give a prognosis and to select appropriate methods of eliminating this defect.

Do I Need surgery?

coarctation of the aorta ICD

What to do if the patient discovered coarctation of the aorta? The operation certainly is the only effective remedy of the defect. But the decision on surgical intervention can taken only doctors familiar with the history and clinical picture.

In some cases (if only a small contraction, which has virtually no effect on hemodynamics) operation may not be required. The patient only periodically pass check-UPS.

If the difference in systolic blood pressure of the arteries of the upper and lower extremities exceeding 50mm Hg. article, the doctors often offer to carry out a surgical procedure. If you have been diagnosed with coarctation of the aorta in newborns, surgery (urgent) is the case that in young patients there is hypertension and cardiac decompensation. In cases where the disease is relatively favorable and a large risk to the life of a child no, the procedure may be postponed up to five or six years of age.

Surgical treatment of heart disease

coarctation of the aorta surgery

Today, there are several methods of eliminating this defect. The choice of technique of surgery depends on the condition of the patient, form of the disease and the size of the coarctation.

  • In some cases, doctors perform the resection (removal) of the narrowed portion of the aorta, and then reunite the ends of the vessel, applying the anastomosis. Such a procedure is possible only if the coarctation is small in length.
  • In that case, if the constriction is long, and impose anastomosis is not possible, the use of dentures. During the procedure, the surgeon removes the diseased portion of the aorta, after which the two ends of the receptacle are joined using a special prosthesis made of synthetic materials.
  • Aortoplasty — another kind, in this case to restore the length of the aorta is not used synthetic prosthesis and of the left subclavian artery of the patient.
  • Sometimes doctors decide to conduct a bypass of the aorta. In such cases, a synthetic graft, which is sewn above and below the narrowed portion of the vessel — this creates a detour for blood flow.
  • There is another procedure called balloon angioplasty. It is carried out in cases, if after informed surgery again narrowing of the vessel. During the procedure, the doctor inserts through the peripheral vessels into the lumen of the aorta a special balloon, which, when blowing the narrowing disappears. In some cases, additionally, there are special rigid stents, which record the sizes of the aortic lumen.

This is the correction called “coarctation of the aorta”. After surgery, the patient, of course, is to follow certain guidelines. In particular, the need to eat right, avoid excessive physical exertion and inactivity, to carefully monitor the health and undergo regular checkups by a cardiologist.

What complications are possible?

Coarctation of the aorta — this is a dangerous disease, which in any case should not be ignored. When unfavorable course defect can lead to extremely dangerous complications:

  • Due to the narrowing of blood vessels in patients may develop severe hypertension.
  • Often, this heart defect - coarctation of the aorta - is also accompanied by the formation of the aneurysm and its subsequent rupture.
  • Complications include stroke and subarachnoid effusion of blood.
  • The background of this disease, patients often develop heart failure, which is accompanied by the so-called cardiac asthma and pulmonary edema.
  • Narrowing of the aorta affects the entire vascular system. The heavy gipertenzii perhaps the defeat of the small arteries of the kidney.
  • Occasionally, patients develop bacterial endocarditis. Such a complication usually found in that case, if coarctation of the aorta is associated with abnormalities of the aortic valve. Unfortunately, in most cases, these bacterial lesions of the heart are resistant to antibiotics.

As you can see, coarctation of the aorta in children and adults may lead to death. That is why it is important the timely diagnosis and adequate therapy.

The prognosis for patients with coarctation

Most Often diagnosed coarctation of the aorta in infants. What is the Outlook for young patients and what should parents expect? Actually it all depends on the degree of narrowing of the blood vessels. In some cases, the disease requires immediate surgery. If we are talking about a moderate degree of defect, even at observance of all medical recommendations patients only live up to 30-35 years, with cause of death usually becomes a stroke or aneurysm rupture.

Mild contractions sometimes requires surgery — the doctor only recommends that you periodically undergo examinations to detect complications. In such cases, the presence of heart disease rarely affects the duration of life of the patient.

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