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 the pathology is localized outside the borders of the myocardium. Most often, coarctation of the aorta in newborns is diagnosed, although sometimes the disease is already found in adult patients. Most often, the disease is eliminated by surgical intervention.
Of course, this diagnosis is not entirely clear to patients and causes them to panic. Therefore, it is worth learning more about what this disease is and what complications a sick person may encounter.
Coarctation of the aorta: what is it?
To begin with, it’s worth understanding the meaning of the term. Coarctation of the aorta is a congenital malformation accompanied by 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 a person, and a violation of blood flow in this area adversely affects the work of not only the heart and blood vessels, but also the entire body.
As a rule, a narrowing is formed in the place where the aortic arch passes into its descending part. This pattern is understandable, because there is normally a slight physiological narrowing. By the way, for the first time this ailment was described in 1791 by D. Morgagni (Italian pathologist). According to statistics, the frequency of pathology is up to 15% of all congenital malformations. An interesting fact is that in boys the disease is observed 3-5 times more often than in female patients.
The main types of pathology
Depending on the features, coarctation of the aorta can be of two types:
- “Adult” coarctation is characterized by a narrowing of the aortic lumen below the place where the left subclavian artery departs from it; while there is a closure of the arterial duct;
- the infantile type of pathology is accompanied by aortic hypoplasia at the same location of the subclavian artery, but the duct remains open.
Anatomical features of pathology
Congenital coarctation of the aorta may be accompanied by various concomitant pathologies. Depending on this, defect is usually divided into three groups.
The pathology can be isolated - while there are no other defects in the development of the cardiovascular system. Often, coarctation of the aorta in children can be combined with other pathologies - it can be aneurysm, aortic stenosis, a defect in the septum between the ventricles and atria, and transposition of the great vessels.
The third group is coarctation, in which the opening of the ductus arteriosus is observed. In such cases, the pathology may be:
- postductal (the narrowing is located below the place where the open arterial duct departs;
- juxtaductal (coarctation is just at the level of the open duct);
- preductal (arterial duct opens below the site of narrowing).
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 you know, during the period of 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 in a child, a small part of the tissues of this duct moves to the aorta, therefore, 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 patients with Shereshevsky-Turner chromosome syndrome (the presence of only one sex chromosome) are most prone to this disease. Almost every tenth child with this diagnosis has this defect.
Coarctation of the aorta (ICD) does not have to be congenital. Rare cases are known in which narrowing of the vessel occurred already in adulthood. In such cases, the causes of coarctation were injuries and atherosclerotic lesions of the aorta, inflammatory diseases of the vascular walls of an unclear etiology (Takayasu syndrome).
Hemodynamic impairment in coarctation
Of course, aortic narrowing affects the functioning of the entire cardiovascular system, even if there are no concomitant defects. The presence of coarctation leads to the formation of two different modes of blood flow.
Above the narrowing point, blood pressure is increased, which leads to an expansion of the lumen of all vessels. Hypertrophy of the left ventricle develops due to systolic overload. But the situation is opposite below the coarctation region - blood flow is slowed down, blood pressure is lowered. As the child’s body develops, compensatory mechanisms are activated - multiple collateral vessels develop, which provide a bypass blood flow.
If we are talking about an adult type of defect, then patients have hypertrophy of the left ventricle, severe hypertension and an increase in the volume of circulating blood. In infantile coarctation, when the arterial duct is open, the above disorders are not so pronounced. But there are other pathologies. For example, with the postductive type of pathology, blood from the aorta under high pressure is supplied directly to the pulmonary artery, which leads to an increase in pressure in the vessels of the pulmonary circulation.
In any case, there is a serious violation of hemodynamics, which in no case should be ignored, because the consequences can be dire.
What are the symptoms of 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. A slight degree of coarctation may not appear at all.
In more severe cases, symptoms are noticeable in the first year of life. Toddlers often suffer from pneumonia and constant relapses. Children have pale skin and breathing problems - severe shortness of breath appears during eating, playing games, or even at rest. Often, children with this diagnosis physically develop more slowly than their peers.
It is possible to suspect the presence of a defect while listening to heart sounds, as well as when determining the pulse in the vessels of the upper and lower extremities. In arteries on the elbow bends, a pulsating pulse can be seen, while on the femoral vessels the pulse is felt very weakly.
In preductal coarctation, heterogeneous cyanosis is also observed - in a child, the skin on the legs acquires a bluish tint, while the skin in the upper body retains a natural color.
It often happens that pathology is diagnosed at an older age - in school children, 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, you can observe a disproportionate development of the muscles of the upper and lower body. Symptoms include weakness in the legs, frequent cramps, coldness of the feet. In women, menstrual disorders, and sometimes infertility, can be observed.
Coarctation of the aorta: diagnosis
The doctor may suspect the presence of a defect while listening to heart sounds. Further, other diagnostic measures are carried out that help not only establish the fax of the presence of coarctation, but also detect other defects and determine the degree of damage to the cardiovascular system:
- One of the simplest and most affordable methods is electrocardiography. This is not the most informative technique, since with a moderate narrowing of the aorta, the patient's electrocardiogram may look completely normal. In children in the first year of life during the procedure, you can notice a shift in the electrical axis of the heart. In older children, 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 an incomplete blockade of the left leg of the bundle of His is observed .
- Often patients are prescribed phonocardiography. This procedure allows you to record sound signals and vibrations generated during the work of the heart and blood vessels. In the presence of coarctation, one can notice an increase in the second tone on the aorta, as well as the appearance of systolic murmur on the back and in the second intercostal space at the edge of the sternum (on the right and left side).
- Informative is an ultrasound scan of the heart, in which aortic narrowing can be detected. Also, a specialist can see an increase in heart mass. Doppler echocardiography is used to identify characteristic symptoms of pathology, in particular, the difference in blood pressure in areas above and below the narrowing, the presence of turbulent systolic flow.
- An additional x-ray of the lungs and heart is performed. As a rule, the size of the heart remains normal, but you can notice a significant expansion of the ascending aorta. Pulmonary pattern can be increased along the arterial bed, although this is not always observed.
- Aortography is a procedure that involves the introduction of a special contrast agent into the aorta, followed by monitoring its distribution throughout the vessel. This study allows you to accurately determine the degree and level of aortic narrowing.
- 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 the full picture can the doctor give a prognosis and select the appropriate methods to eliminate this defect.
Is surgery necessary?
What to do if a patient has aortic coarctation? The operation, of course, is the only effective way to eliminate the defect. But the decision to conduct surgery can only be made by doctors who are familiar with the anamnesis and clinical picture.
In some cases (if there is only a slight narrowing, which practically does not affect hemodynamics), surgery may not be required. The patient only periodically undergoes examinations.
If the difference in systolic pressure of the arteries of the upper and lower extremities exceeds 50 mm RT. Art., then doctors most often suggest a surgical procedure. If coarctation of the aorta in newborns has been diagnosed, surgery (urgent) is performed if in small patients there is severe hypertension and decompensation of cardiac activity. In cases where the disease proceeds relatively favorably and there is no great risk to the life of the child, the procedure can be delayed until five or six years of age.
Surgical treatment of heart disease
To date, there are several methods to eliminate this defect. The choice of surgical technique depends on the condition of the patient, the form of the disease and the size of the coarctation.
- In some cases, doctors perform a resection (excision) of the narrowed section of the aorta, after which they reconnect the ends of the vessel, applying an anastomosis. Such a procedure is possible only if coarctation has a small extent.
- In the event that the narrowing section is long and it is not possible to apply an anastomosis, 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 using a special prosthesis made of synthetic materials.
- Aortoplasty is another type of surgery, only in this case, not a synthetic prosthesis is used to restore the length of the aorta, but part of the patient’s left subclavian artery.
- Sometimes doctors decide to have aortic bypass surgery. In such cases, a synthetic prosthesis is used, the edges of which are sutured above and below the narrowed portion of the vessel - thus creating a workaround for blood flow.
- There is another procedure called balloon angioplasty. It is carried out in those cases if, after a previous surgical intervention, a narrowing of the vessel again appeared. During the procedure, the doctor introduces a special balloon through the peripheral vessels into the lumen of the aorta, and when it is inflated, the narrowing disappears. In some cases, special rigid stents are additionally installed that fix the size of the aortic lumen.
This is exactly what the correction of the defect called “aortic coarctation” looks like. After the operation, the patient, of course, should adhere to certain recommendations. In particular, you need to eat right, avoid excessive physical exertion and physical inactivity, carefully monitor your well-being and regularly undergo examinations by a treating cardiologist.
What complications are possible?
Coarctation of the aorta is a dangerous ailment, which in no case should be ignored. With an unfavorable course, a defect can lead to extremely dangerous complications:
- Patients may develop severe hypertension due to narrowing of the vessel.
- Often this heart disease - aortic coarctation - is also accompanied by the formation of aneurysm and its further rupture.
- Complications include stroke and subarachnoid blood flow.
- Against the background of this disease, patients often develop heart failure, which is accompanied by so-called cardiac asthma and pulmonary edema.
- Narrowing the lumen of the aorta affects the work of the entire vascular system. Due to severe hyperemia, damage to the small arteries of the kidney is possible.
- Occasionally, patients develop bacterial endocarditis. A similar complication, as a rule, occurs if coarctation of the aorta is associated with pathologies of the aortic valve. Unfortunately, in most cases, such bacterial heart lesions are practically not amenable to antibiotic treatment.
As you can see, coarctation of the aorta in children and adults can be fatal. That is why timely diagnosis and adequate therapy are so important.
Predictions for patients with coarctation
Most often, coarctation of the aorta in newborns is diagnosed. What are the predictions for young patients and what should parents expect? In fact, it all depends on the degree of narrowing of the vessels. In some cases, the disease requires immediate surgical intervention. If we are talking about a moderate degree of defect, then even if all 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.
A slight degree of narrowing sometimes does not require surgical intervention - the doctor only recommends periodically undergoing examinations to identify complications. In such cases, the presence of heart disease rarely affects the life expectancy of the patient.