Tetrada Fallot - what is this? Diagnosis and treatment

The tetralogy of Fallot is a congenital heart disease characterized by several anomalies. If there is such a disease, the functioning of the cardiovascular system changes, which is fraught with more dangerous consequences. For example, in newborns, this pathology is most often accompanied by cyanosis, shortness of breath and severe developmental delay. That is why parents are so concerned about questions about what a disease is, what factors influence it, what symptoms it accompanies. After all, early diagnosis and appropriate treatment is the only thing that can provide a normal life for the child in the future.

What is a tetralogy of Fallot?

The tetralogy of Fallot( ICD) is a congenital, complex heart disease. The morphological basis of the pathology consists of four signs( which, in fact, is evidenced by the term "tetrad").These are:

• obstruction of the outlet of the right ventricle;
• aortic displacement;
• hypertrophy of the right ventricle;
• extensive defect of interventricular septum.

This pathology was first described in 1888 by the French pathologist Phallo, whose name was subsequently named. According to statistics, this disease is about 7-10% of congenital malformations. By the way, sometimes the tetralogy of Fallot is combined with other anomalies, for example, the open arterial duct, stenosis of the branches of the pulmonary artery, the right-handed arch of the aorta, etc.

The main causes of the development of the disease

The tetralogy of Fallot is a pathology that is formed as a result of a violation of the bookmarking process anddevelopment of the cardiovascular system at about 2-8 weeks of embryonic development. It is worth saying that the causes of this anomaly are not fully understood. On the other hand, researchers managed to find out some of the risk factors.

For example, the pathology can be formed if in the early stages of pregnancy the woman suffered an infectious disease( rubella, measles, scarlet fever and some others).Violation of the laying and development of fetal organs can be associated with the use of certain medicines, including hormonal drugs, hypnotics and sedatives. Taking drugs, alcohol also affects the formation of congenital heart disease( CHD).

The tetralogy of Fallot is sometimes diagnosed in children with other anomalies, for example, the so-called Amsterdam dwarfism. This is a serious disease that is accompanied by a number of disorders, including deformities of the auricles, hypertrichosis, myopia, optic nerve atrophy, deformation of the spine, etc.

As a rule, the development of this heart defect begins from the moment when for some reason the arterial cone rotatescounterclock-wise. As a result of incorrect rotation, the aortic valve is displaced, and the aorta itself is located on the interventricular septum. As the fetus develops, the pulmonary trunk lengthens, narrows and shifts due to the incorrect location of the aorta.

Classification of the disease

The tetralogy of Fallot in children can be represented by four types, depending on the obstruction of the right ventricular outflow tract:

• Embryological, or the first type of tetralogy of Fallot, is a form of pathology in which the displacement of the conical septum occurs to the left and forward. The fibrinous ring is not deformed, and the maximum stenosis zone is located in the area of ​​the muscular ring.
• The second type, or hypertrophic tetralogy of Fallot, is a kind of pathology, in which not only the displacement of the septum( as described in the previous case) is observed, but also its pronounced hypertrophy. The zone of maximum development of stenosis is observed at the level of the outlet of the right ventricle.
• The third type of pathology, also referred to in medicine as also tubular, is characterized by an incorrectly formed division of the common arterial trunk. As a result of such a violation of the normal structure, there is a change in the pulmonary cone - it becomes narrowed and shortened. Also for this type of disease is characterized by stenosis of the pulmonary artery and hypoplasia of the fibrinous ring.
• The multicomponent, fourth form of the tetralogy of Fallot, as a rule, is accompanied by a considerable elongation of the conical septum.

Features of hemodynamics in this disease

What changes are accompanied by heart disease? The tetralogy of Fallot in any case entails hemodynamic disturbances, the degree of which is determined by the form of obstruction of the excretory duct of the right ventricle, as well as the presence of changes in the interventricular septum.

If the stenosis of the pulmonary artery is significant and a large septal defect occurs, a larger volume of blood from both ventricles enters the aorta. But in the pulmonary artery is much less than normal, which provokes the development of arterial hypoxemia. In the extreme form of this pathology, the blood enters the pulmonary circulation from the aorta, either through the collaterals, or through the open arterial duct.

If the obstruction in the patient is moderate, then left-right venting of the blood develops - in this case the acyanotic form of the disease is observed. Nevertheless, the disease is constantly progressing - first the collection of blood becomes cross, and then right-left, while the disease is transformed from white to blue( cyanotic).

What are the symptoms associated with the disease?

The clinical picture for this pathology can be different, since it largely depends on the form of the disease. For example, sometimes a tetralogy of Fallot in a newborn is accompanied by a pronounced cyanosis. In this case, the baby may suffer from shortness of breath during feeding. Also in the first months of life a low weight gain is observed. On the other hand, if the stenosis of the pulmonary artery in the child is minimal, then at rest, cyanosis is not observed, and therefore diagnose the disease by external signs is not always possible quickly.

Quite often, seizures of hypoxia develop against the background of motor activity. This condition is accompanied by deep and very frequent breaths. The kid starts screaming, becoming extremely restless. Cyanosis gradually builds up. Typically, this pattern is observed in young children, with the symptoms most pronounced at the age of two to four months.

If the tetralogy of Fallot in a newborn is accompanied by severe seizures, it can lead to general lethargy and seizures. Sometimes the pathology leads to a lethal outcome. Older children sometimes instinctively squat during a physically active game, which presumably reduces the systemic venous return and increases oxygen saturation in the arterial blood.

The main symptoms of the pathology are attacks of cyanosis and dyspnea, which are often accompanied by loss of consciousness. The degree of oxygen deficiency and the severity of seizures directly depend on the severity of pulmonary artery stenosis.

It is also worth noting that in the clinical picture, as a rule, several stages of the relative well-being of the child can be identified, as well as a few particularly difficult periods:

• From birth to 6 months. The fact is that during this period children are not too active physically, and therefore cyanosis and hypoxia are moderately expressed. Naturally, this does not always happen, some children suffer practically from the first days of life.
• The most difficult stage( from a medical point of view) is observed from six months to two years. The attack develops with spasm of the lead out section of the right ventricle. As a result of spasm all the venous blood is discharged into the aorta, which leads to the development of more pronounced brain hypoxia. As a rule, the onset of a child begins suddenly - it becomes restless. Cyanosis and dyspnea are observed, sometimes up to loss of consciousness. Cramps often appear. This process in most cases occurs against a background of emotional or physical stress.

In the future, the clinical picture changes slightly. At an older age, cyanosis is constantly growing( sometimes quickly, sometimes slowly), but the seizures almost completely disappear. Dyspnea and tachycardia decrease, and in the lungs a collateral circulation circuit is formed.

Modern diagnostic methods

Naturally, only a qualified doctor can make a similar diagnosis. The Fallot Notebook is a pathology that requires the collection of a complete history and a series of studies that not only confirm the presence of a congenital malformation, but also help determine the form of the ailment, the degree of its severity, and the overall health of the child.

In the presence of symptoms, such studies are carried out:

• Primary examination includes an electrocardiogram. During the procedure, it can be noted signs of overload of certain parts of the heart, as well as hypertrophy. For example, the results may demonstrate a syndrome of weakness of the sinus node or an AV blockade.
• The tetralogy of Fallot is a pathology that can be seen during chest X-ray. For example, this defect is accompanied by the bulging of the left and right ventricles, and a noticeable impoverishment of the pulmonary pattern is observed. In newborns, the heart on the roentgenogram in shape often resembles a slipper.
• Quite an informative method is EchoCG.During this study, you can accurately assess the size of the right ventricular cavity, as well as determine the type of pathology, evaluate the degree of narrowing of the outlet duct. With the same method, heart valves, in particular, the aortic valve, are examined, the general condition of its valves is examined, and the diameter of the fibrous rings is measured. The fact is that if you do not detect aortic stenosis and perform aortic treatment, after the end of therapy, complications, in particular, pulmonary edema, may appear.

As a rule, the studies described above are sufficient to determine if a child has a malformation. The tetralogy of Fallot, however, is a diagnosis, which is sometimes very difficult to put, and a number of additional procedures may be required.

For example, coronary angiography is sometimes performed. This procedure is necessary in order to determine the exact localization of coronary and trunk vessels, to check the presence of any accompanying anomalies, and also to accurately determine the type of heart defect.

Catheterization is a more complicated procedure that involves the puncture of the heart cavities. This study is prescribed if it is necessary to measure the pressure in the right ventricle, determine the degree of oxygenation of the blood in the aorta.

The tetralogy of Fallot: treatment of

The treatment scheme largely depends on the form of the disease and the degree of its severity. Drug treatment helps to eliminate seizures and prevent the development of certain complications associated with oxygen starvation.

As a rule, newborns are prescribed prostaglandin infusions. This procedure sometimes helps to open the arterial duct again. If the baby has the first signs of an attack, then it is recommended to press his knees to his chest. The older children squat themselves - this position helps to avoid the appearance of a hypoxemia attack.

An intravenous fluid is recommended to supplement the volume of circulating blood. In some cases, small patients are prescribed morphine. If necessary, arterial pressure is increased with phenylephrine or ketamine( this substance, by the way, has a sedative effect).Prevent relapse sometimes helps propranolol.

Oxygen therapy is sometimes used to stop seizures, but it should be noted that the effect of such treatment is rather mild.

Palliative surgery

Naturally, various drugs can alleviate a child's condition. Nevertheless, drugs can not save the patient from such a dangerous ailment as the tetralogy of Fallot. Operation to date is the only way to eliminate the blemish. Only surgical intervention can give the baby a chance for a normal maturation and a full life.

To date, there are two main methods of surgical treatment. Palliative surgery consists in the imposition of different types of interarterial nodes, which allows the restoration of blood circulation. For example, the most popular procedure for today is the application of subclavian pulmonary anastomosis. During the procedure, the subclavian artery is connected with a single-sided pulmonary artery by means of a special implant.

Radical surgery as a method of treatment

Doctors recommend a radical operation whenever possible, as it gives much better results. Typically, this procedure is carried out at the age of 6 months to 3 years - it is during this period that it is most effective( if we consider the further process of growing up and developing a child).

A radical method of treatment is the complete correction of pathologically altered structures. With the help of a patch, surgeons close the defect of the interventricular septum, expand the stenotic portion of the pulmonary artery. Naturally, the procedure is complicated, and therefore only a qualified cardiac surgeon can conduct it.

Tetralogy of Fallot: outlook for patients

course, first of all parents interested prospects with similar diseases. The tetralogy of Fallot in children - the pathology is extremely serious, it can not be ignored in any case.

It should be understood that the course of the disease largely depends on the severity of pulmonary stenosis. If we are talking about statistics, then, unfortunately, almost a quarter of children die in the first year of life. Without surgical intervention, the average age of life of children with the disease is 12 years. Only about 5% live to be 40 years old with a diagnosis like the tetralogy of Fallot. Disability, developmental problems, constant control and poor quality of life - this is something with which, unfortunately, have to face adult patients.

There is, of course, an alternative. Do not forget about the surgical treatment, which is often prescribed to children diagnosed with the tetralogy of Fallot. After the operation, children, as a rule, quickly recover, develop normally, even able to withstand heavy physical exertion. A regularity is noted - surgical intervention at a later age does not give such good results in the future as an operation performed in infancy.

Patients with this disorder, even after completion of therapy should undergo regular check-ups at the heart surgeon and cardiologist. Before carrying out any surgical and dental procedures, patients should undergo special treatment to avoid the risk of endocarditis. Naturally, there are other requirements, limitations in the life of the patient, which the attending physician will necessarily tell.