Palliative surgery for complete transposition of the great vessels. How dangerous is this? After surgery of transposition of the great arteries

Congenital malpositioned vessels emanating from the ventricles of the heart pose a threat to life. Transposition great vessels- the most common congenital heart defect among other defects. Monitoring a pregnant woman can identify the problem before the baby is born, which will help plan its treatment.

Features of the disease

The correct structure ensures that the pulmonary trunk emerges from the ventricle on the right, which is responsible for venous blood. Through this main vessel, the blood is sent to the lungs, where it is enriched with oxygen. An artery emerges from the left ventricle. Left half is responsible for arterial blood, which is sent to the large circle to nourish tissues.

Transposition is a pathology when the great vessels have changed places. From the right ventricle, depleted blood enters the artery and systemic circle. And from the left ventricle, blood, intended to saturate the cells with oxygen, flows again through vicious circle into the lungs.

The result is two parallel circles, where the enriched blood cannot enter the large circle, and when it enters the lungs, it is not saturated with oxygen, because it has not given it to the tissues and has not become depleted. Venous blood in a large circle immediately creates oxygen starvation fabrics.

How are abnormal locations of blood vessels perceived? different periods life:

Baby in the womb. The baby in the womb does not experience discomfort when similar pathology, because his blood has not yet passed through a large circle and he is not vital during this period.
For a born child, it is important that at least some part has the opportunity venous blood receive oxygen. The situation is helped by the presence of other congenital anomalies. , when there is a hole in it and the blood of different ventricles can communicate with each other. Part of the venous blood moves to the left ventricle and is therefore drawn into the pulmonary circulation. It has the opportunity to receive oxygen by entering the lungs. Arterial blood, partially entering the right half of the heart, goes through the artery into a large circle and prevents tissue from entering critical condition due to hypoxia. Also, partial exchange of blood between the venous and arterial circulation can be carried out thanks to:
- not yet closed arterial duct,
- if the atrial septum has,
- oval window.

At this stage, specialists determine when it is best to make adjustments to the patient. They consider how long they can wait, usually no more than a month. Actions are taken to ensure that the small and large circles do not become completely isolated from each other. The child most often has a bluish skin color. Over time, the negative effects of oxygen starvation begin to accumulate and more and more symptoms of the disease appear.

An adult, if not corrected in early childhood birth defects caused by this type of defect cannot be viable. This is due to the fact that the results of the progression of the pathology accumulate in the body, leading to irreversible processes. There may be options if the anomaly is not very severe, but without any correction it is impossible to have a normal life expectancy.

Medical specialists talk in more detail about the features of the disease and the main method of combating transposition of the great vessels in the following video:

Forms and classification

Experts distinguish four types of violations.

Incomplete transposition. When the great vessels depart from one ventricle, for example: the right.
If the vessels, by an error of nature, have changed places, but one of them has an outlet from both ventricles.
Complete transposition of the great vessels. This is the name given to a defect when the main arteries, the artery and the pulmonary trunk, have swapped places. The result is two parallel circles of blood circulation. At the same time the blood venous circulation and the arterial circle do not communicate with each other.
A difficult case. It helps to hold out until correction, which makes it possible for the blood of the small and great circle. It persists from the perinatal period, and doctors try to delay its closure until a corrective procedure is performed.
Transposition of the highways, which has additional physiological defects. Cases are suitable here when the septum of the heart has a hole, which is a defect. However, this circumstance makes the newborn’s condition easier and makes it possible to survive until the corrective procedure.
Corrected form of transposition of the great vessels
With this pathology, nature seems to have made a double mistake. As in the first case, the main main vessels are mutually displaced in places. And the second anomaly is that the left and right ventricles are also in each other’s place. That is, the right ventricle is located on the left and vice versa.
This form makes the situation easier because it does not have a strong effect on blood circulation. But over time, the consequences of the pathology still accumulate, because the right and left ventricles are naturally created to bear different loads and it is difficult for them to replace each other.

Incorrect location of the great vessels (diagram)

Causes

Incorrect location of the great vessels is formed during the perinatal life of the fetus during the period when the creation of the heart and vascular system occurs. This occurs in the first eight weeks. It is not known exactly why the anomalous failure occurs.

Factors contributing to improper intrauterine development of organs include:

exposure of the expectant mother to influences:
- contact with harmful chemicals,
- reception medicines without the consent of the doctor,
- exposure to ionizing radiation,
- living in places with unfavorable ecology,
- if a pregnant woman has been ill:
  - chicken pox,
  - ARVI,
  - measles,
  - herpes,
  - mumps,
  - syphilis,
  - rubella;
predisposition at the genetic level,
malnutrition or poor diet,
alcohol consumption,
diabetes mellitus in the expectant mother without proper control during pregnancy,
if pregnancy occurs after the age of forty years,
the disease occurs in children with other chromosomal disorders eg Down Syndrome.

Symptoms

Since patients do not survive without correction in early childhood, we can talk about symptoms of transposition of the great vessels in newborns:

the skin has a bluish tint,
enlarged liver,
dyspnea,
increased heart rate;
in the future, if the child was able to survive without correction of the defects:
- there is a delay in physical development,
- does not gain the required weight according to age,
- the chest enlarges,
- the heart is larger than normal,
- swelling.

Diagnostics

Transposition of the great vessels can be determined in the fetus during its intrauterine development. This will help you plan and prepare for help for your child. If the problem has not been identified, then at birth, cyanosis specialists suggest a heart defect.

To clarify the type of violation, there may be such procedures.

Echocardiography
Very informative and safe method. You can determine the incorrect location of blood vessels and other defects.
X-ray images
They make it possible to see the shape and size of the heart, some features of the blood vessels.
Catheterization
A catheter is inserted into the heart area through the vessels. With its help, you can examine in detail the internal structure of the heart chambers.
Angiography
One of the ways to examine blood vessels using a contrast agent.

The following video will tell you what transposition of the great vessels looks like:

Treatment

Chief and the only way treatment for transposition of the great vessels - surgery. There are several methods to support a newborn in the period before adjustment.

Therapeutic and medicinal methods

The therapeutic method will be needed during monitoring of the condition after surgery. The medicinal method is used as an auxiliary method. While preparing the newborn for adjustment, he may be prescribed to take prostaglandin E1. Goal: prevent the growth of the ductus arteriosus.

It exists in the child before birth, then it grows over. Keeping the duct open will help the child survive until surgery. The ability to communicate with the venous and arterial circulation will remain.

Now let's talk about surgery for transposition of the great vessels.

Operation

The first surgical intervention, which in most cases is performed on newborns as quickly as possible, is the Rashkind procedure. It involves inserting a catheter with a balloon into the heart area under the supervision of equipment.
The balloon in the oval window inflates, thereby expanding it. Closed operation (palliative).
The operation to correct defects is a radical intervention using artificial circulatory support (Jatenay's operation). The purpose of the procedure is to completely correct natural defects. The best time to carry it out is the first month of life.
If you are late in turning to specialists for help, then sometimes the operation to move the vessels cannot be performed. This is due to the fact that the ventricles have adjusted and adapted to the existing load and may not be able to withstand the change. This usually applies to children aged over a year old and up to two years. But specialists have options on how to help them too. Surgical intervention is performed to redirect blood flows so that as a result, arterial blood circulates in the large circle, and venous blood circulates in the small circle.

The following video will tell you more about how the operation is performed if a child has a transposition of the great vessels:

Disease prevention

Before conceiving a child, you need to seriously prepare, have your health examined, and follow the recommendations of specialists. During pregnancy, situations that are harmful should be avoided:

be in places with unfavorable ecology,
do not come into contact with chemicals,
not be exposed to vibration or ionizing radiation;
If you need to take pills, consult a specialist;
take precautions to avoid infectious diseases.

But if a pathology occurs, then best option– its detection before the birth of the child. Therefore, you should be monitored while carrying a child.

Complications

The longer a child lives without adjustment, the more the body adapts to the situation. The left ventricle gets used to the reduced load, and the right to the increased load. U healthy person the load is distributed in reverse.

The reduced load allows the ventricle to reduce wall thickness. If adjustments are made late, the left ventricle may not be able to cope with the new load after the procedure.

Without surgery, oxygen starvation of tissues increases, creating new diseases and shortening life span. Very rarely there are complications after surgery: narrowing of the pulmonary artery. This happens if during the procedure artificial materials were used for prosthetics or due to sutures.

In order to avoid this, many clinics use:

a special technique for sewing elements, taking into account the further growth of the highway;
natural materials are used for prosthetics.

Forecast

After performing corrective surgery, positive results occur in 90% of cases. Such patients require long-term observation by specialists after the procedure. They are advised not to expose themselves to significant physical exertion.

Without qualified assistance Newborns with naturally displaced vessels die in the first month of life, up to 50%. The remaining patients for the most part live no longer than a year due to hypoxia, which progresses.

Transposition of the great vessels: the essence of congenital heart disease, causes, treatment, prognosis

Transposition of the great vessels (GV) is a severe cardiac anomaly when the aorta emerges from the right ventricle (RV) and the pulmonary trunk from the left. TMS accounts for up to 15-20% of all congenital heart defects (CHD); among patients there are three times more boys. TMS is among the most common forms CHD along with, (VSD), etc.

When the great arteries are transposed, oxygen enrichment does not occur arterial blood, since it moves in a vicious circle, bypassing the lungs. Little patient becomes cyanotic immediately after birth, on the face obvious signs heart failure. TMS – “blue” defect with severe tissue hypoxia, demanding surgical treatment in the first days and weeks of life.

Causes of TMS

The exact reasons for the appearance of pathology in a particular baby are usually impossible to establish, because the mother could have been exposed to a variety of adverse effects during pregnancy. The following may play a role in the occurrence of this anomaly:

Viral diseases during pregnancy (rubella, chickenpox, herpes, respiratory infections);
Heavy ;
Ionizing radiation;
Alcohol consumption medicines with teratogenic or mutagenic effects;
Concomitant pathology in a pregnant woman (diabetes, for example);
The mother is over 35 years old, especially if this is the first pregnancy.

It has been noted that TMS occurs more often in children with Down syndrome, the causes of which are chromosomal abnormalities caused by the reasons listed above, among others. Children with TMS may also be diagnosed with defects of other organs.

There may be an influence of heredity, although the exact gene responsible for abnormal heart development has not yet been found. In some cases, the cause is a spontaneous mutation, while the mother denies the possibility of external influence in the form of x-rays, drugs or infections.

The laying of organs and systems occurs in the first two months of embryo development, therefore during this period it is necessary to protect the very sensitive embryo from all kinds of toxic factors. If the heart begins to form incorrectly, then it will not change, and signs of the defect will appear immediately after birth.

Blood movement during TMS

I would like to dwell in more detail on how blood moves through the cavities of the heart and vessels during their transposition, because without understanding these mechanisms it is difficult to imagine the essence of the defect and its manifestations.

Features of blood flow during TMS are determined by the presence of two closed, unconnected blood circulation circles. From a biology course, everyone knows that the heart “pumps” blood in two circles. These streams are separate, but represent a single whole. Venous blood leaves the pancreas into the lungs, returning as arterial blood enriched with oxygen to the left atrium. From the LV, arterial blood with oxygen enters the aorta and goes to the organs and tissues.

With TMS, the aorta begins not in the left, but in the right ventricle, and the pulmonary trunk arises from the left. Thus, we get two circles, one of which “drives” venous blood through the organs, and the second one sends it to the lungs and, in fact, receives it back. In this situation, there can be no talk of adequate exchange, since oxygenated blood does not reach other organs besides the lungs. This type of defect is called complete TMS.

Complete transposition in the fetus is quite difficult to detect. On an ultrasound, the heart will appear normal, four-chambered, with two vessels branching off from it. Diagnostic criterion The defect in this case may be the parallel course of the main arteries, which normally intersect, as well as visualization of a large vessel that originates in the left ventricle and is divided into 2 branches - the pulmonary arteries.

It is clear that blood circulation is impaired to critical level, and it’s impossible to do without at least some opportunity to send arterial blood to the organs. To help the sick little heart Strange as it may sound, other UPS may come. In particular, the ventricles will benefit. The presence of such additional communication routes makes it possible to connect both circles and ensure, albeit minimal, delivery of oxygen to the tissues. Additional pathways provide vital activity before surgery and are present in 80% of patients with TMS.

blood flow paths that are pathological for an adult partially compensate for the defect and are present in most patients

Of no small importance in relation to the clinic and prognosis is the state of the pulmonary blood flow, the presence or absence of its blood overload. From this position It is customary to distinguish types of TMS:

With overload or normal pressure in the lungs;
With reduced pulmonary circulation.

In nine out of ten patients, there is an overload of the small circle with “excess” blood. The reasons for this may be defects in the partitions, open ductus arteriosus, the presence of additional communication routes. Depletion of the small circle occurs when the LV outlet is narrowed, which occurs in isolated form or in combination with a ventricular septal defect.

An anatomically more complex defect is corrected transposition of the great vessels. Both chambers and vessels are “confused” in the heart, but this makes it possible to compensate for disturbances in blood flow and bring it to an acceptable level. With corrected TMS, both ventricles with the vessels extending from them change places: the left atrium passes into the right ventricle, followed by the aorta, and from the right atrium the blood moves into the LV and pulmonary trunk. Such “confusion,” however, ensures the movement of fluid in the right direction and enrichment of tissues with oxygen.

Complete TMS (left) and corrected defect (right), photo: vps-transpl.ru

In the case of a corrected defect, the blood will move in a physiological direction, so the presence of additional communication between the atria or ventricles is not required, and if it is present, it will play a negative role, leading to hemodynamic disorders.

Video: TMS – medical animation (eng)

Manifestations of TMS

During intrauterine development, this heart defect does not manifest itself in any way, because in the fetus the pulmonary circuit does not work. After birth, when the baby’s heart begins to pump blood to the lungs on its own, TMS also manifests itself in full. If the transposition is corrected, then the clinical picture is poor; if the defect is complete, its signs will not be long in coming.

The degree of impairment with complete TMS depends on the communication pathways and their size. The more blood mixes in the heart in newborns, the more oxygen the tissues will receive. The optimal option is when there are sufficient holes in the septa, and the pulmonary artery is somewhat narrowed, which prevents volume overload of the pulmonary circle. Complete transposition without additional anomalies is incompatible with life.

Babies with transposition of the main vessels are born at term, with normal weight or even large ones, and already in the first hours of life signs of congenital heart disease are noticeable:

Strong throughout the body;
Dyspnea;
Increased heart rate.

The heart increases in size;
Fluid appears in the cavities (ascites, hydrothorax);
The liver enlarges;
Swelling occurs.

Other signs of cardiac dysfunction are also noteworthy. The so-called “heart hump” (deformation of the chest) is caused by an enlargement of the heart, the nail phalanges of the fingers thicken, the baby lags behind in development, and does not gain weight well. Certain difficulties arise during feeding, since it is difficult for a child to suckle at the breast with severe shortness of breath. Any movement and even crying can be an impossible task for such a baby.

If an excess amount of blood enters the lungs, there is a tendency to infectious and inflammatory processes and frequent pneumonia.

The corrected form of TMS proceeds much more favorably. In the absence of other cardiac defects, clinical transposition may not occur at all, because the blood moves correctly. The child will grow and develop correctly according to his age, and the defect can be detected accidentally by the presence of tachycardia, heart murmur, or conduction disturbances.

If the corrected transposition is combined with other disorders, then the symptoms will be determined by them. For example, if there is a hole in the interventricular septum, shortness of breath will appear, the pulse will increase, and signs of heart failure will appear in the form of edema and enlarged liver. Such children suffer from pneumonia.

Methods for correcting TMS

Given the availability anatomical changes hearts, only possible option treatment of the defect becomes surgical operation, and the sooner it is performed, the less irreversible consequences will bring disease.

Emergency intervention is indicated for patients with complete TMS, and before the operation, prostaglandin drugs are prescribed to prevent the closure of the ductus arteriosus, which allows the blood to “mix”.

In the first days of a baby’s life, it is possible to perform operations to ensure the connection of the blood circulation. If there are holes in the partitions, they are expanded; if there are no defects, they are created. Rashkind's operation is performed endovascularly, without penetration into the chest cavity and consists of introducing a special balloon that expands oval window. This intervention provides only a temporary effect for several weeks, during which the issue of radical treatment must be decided.

The most correct and effective treatment is considered to be an operation in which the aorta returns to the left ventricle and the pulmonary trunk to the right, as they would be normally. The intervention is carried out openly, under general anesthesia, the duration is from one and a half to two hours or more, depending on the complexity of the defect.

example of operation for TMS

After the baby is under anesthesia, the surgeon cuts through the chest tissue and reaches the heart. At this point, artificial blood flow is established, when the device plays the role of the heart, and the blood is additionally cooled to prevent complications.

Having opened the way to the main arteries and the heart, the doctor cuts off both vessels slightly above their attachment, approximately in the middle of their length. At the mouth pulmonary artery The coronary ones are sutured, then the aorta is “returned” here. The pulmonary artery is fixed to the portion of the aorta remaining at the exit from the right ventricle using a fragment of the pericardium.

The result of the operation is normal location vascular tracts, when the aorta leaves the left ventricle, the coronary arteries of the heart also begin from it, and the pulmonary trunk originates in right half organ.

The optimal period for treatment is considered to be the first month of life. Of course, you can live longer waiting for her, but then the intervention itself will become inappropriate. As you know, the left ventricle is thicker than the right and is designed for greater pressure load. With a defect, it atrophies, as the blood is pushed into a small circle. If the operation is performed later than expected, the left ventricle will not be ready for the fact that it will have to pump blood into the systemic circulation.

When time is lost and it is no longer possible to restore the anatomy of the heart, there is another way to adjust the blood flow. This is the so-called intra-atrial correction, which has been used for more than 25 years and has proven itself as effective way TMS treatment. It is indicated for children who did not undergo the above-described operation on time.

The essence of intra-atrial correction is to dissect the right atrium, remove its septum and sew in a “patch” that directs venous blood from the systemic circle to the left ventricle, from where it goes to the lungs, while the pulmonary veins return oxygenated blood to the “right” heart and then - into an abnormally located aorta. Thus, without changing the location of the main arteries, blood movement in the desired direction is achieved.

Prognosis and treatment results

When a baby is born with transposition of blood vessels, his parents are very concerned about not only the operation, but also what will happen after, how the child will develop and what awaits him in the future. With timely surgical treatment the prognosis is quite favorable: up to 90% or more of patients live a normal life, periodically visiting a cardiologist and undergoing a minimum of examinations to monitor the functioning of the organ.

With complex defects, the situation may be worse, but most patients still have an acceptable quality of life. After intra-atrial correction surgery, about half of the patients do not experience restrictions in life, and its duration is quite long. The other half may suffer from arrhythmias and heart failure, which is why it is recommended to limit physical activity, and women are warned about the risks of pregnancy and childbirth.

Today, TMS is a completely curable anomaly, and hundreds of children and adults who have successfully undergone surgery are proof of this. Much depends on the parents, their faith in success and desire to help their baby.

Or for short TMS- this is one of the types birth defects heart development, in which the great vessels ( aorta and) leave the heart in the wrong order. This malformation can be detected already at the very early stages development of pregnancy, so quite often young mothers learn about this problem long before giving birth. In this article we will try to explain what transposition of the great vessels is, can a child be born and live with such a diagnosis, and what should be done in this case?

Transposition of the great vessels There are several types: simple, TMS in combination with cardiac septal defects and corrected TMS. Critical defects requiring treatment in the first days of life include simple TMS.

At TMS the vessels completely change their places, that is aorta departs from the right ventricle of the heart, and from the left ventricle. In this case both circles blood circulation(large and pulmonary) turn out to be completely isolated from each other. It turns out that the blood of the pulmonary circle is constantly enriched with oxygen, but does not enter the systemic circle. And blood from the systemic circulation, poor in oxygen, cannot enter the lungs. In such a situation, the child's life would be impossible.

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Still, with TMS there is blood between the circles blood circulation may mix. In particular, blood can enter the pulmonary (pulmonary) circulation through. This vessel functions in all children during intrauterine development, and closes after birth. There are drugs that can support ductus arteriosus open. Therefore, it is very important that the child simple TMS received these medications. This will stabilize the child's condition before surgery.

Before the birth of the child, at the stage intrauterine development, such congenital heart disease does not interfere with the life and development of the fetus, since in the fetus the pulmonary circulation practically does not function, all blood circulates only in a large circle through and patent ductus arteriosus. Therefore, children are born full-term, completely normal, but immediately very cyanotic.

After birth and the first breath, the situation changes dramatically - the pulmonary circulation begins to function separately, and the size becomes insufficient to mix blood in the large and small circles. In addition, every hour it becomes less and less, which aggravates the situation. Therefore, children with simple transposition of the great vessels are in a life-threatening situation and require urgent care immediately after birth.

Of course, if TMS is combined with other heart defects, for example or VSD, then this situation is more beneficial for the newborn, since these defects allow the flow of blood to flow from one circle to another, so the threat to their life becomes less severe, and surgeons have time to choose the right tactics surgical treatment. However, in any case, with complete transposition of the great vessels, surgery is necessary, the only question is when to do it - immediately after childbirth, or you can wait.

Therefore, if during pregnancy you find out that the fetus may have transposition of the great vessels, then the most important thing that needs to be done is to find a specialized maternity hospital that cooperates with the cardiac center so that the child can have surgery immediately after. There are similar maternity hospitals in major major cities such as Moscow, St. Petersburg, Novosibirsk, Tomsk, Penza, Samara.

However, it is important to understand that radical correction of full TMS is a rather complex operation that is successfully performed only in some cities of our country. In all other cardiac centers, they can only perform the Rashkind procedure - this is an auxiliary operation that eliminates a critical situation and allows a child with TMS to live for several more weeks. However, for complete correction of TMS, the child still needs to be transported to one of the cities where the leading ones of our country are located. We can recommend Moscow, Novosibirsk, Tomsk, Samara, Penza.

Remember that the most difficult surgery for complete correction of TMS must be performed within the first month of life! Later it becomes problematic to perform a radical correction, because the left ventricle “gets unaccustomed” to working as a systemic one and after radical surgery may not be able to cope with the load. Therefore, if the timing of radical correction is missed, the child first undergoes an auxiliary operation, which prepares the left ventricle for radical correction.

Another subspecies transposition of the great vessels is corrected TMS. In this case, nature seemed to have made a mistake twice: it was not the aorta and the pulmonary artery that were confused, but the ventricles of the heart. At corrected TMS venous blood enters the left ventricle, but the pulmonary artery departs from it, and arterial blood from the lungs returns to the right ventricle, but the aorta departs from it. That is, blood circulation does not suffer. The child looks absolutely healthy. Problems arise later. The fact is that the right ventricle is not adapted to work in the systemic circulation. Right ventricular function deteriorates over time

Since both circulation circles function normally, a life-threatening situation does not arise in this case and children can live without surgery for a very long time. On our forum of parents of children with congenital heart disease there are participants who are over 30 years old and have corrected TMS. However, such children may have some problems. For example, the fact that the right ventricle of the heart is not anatomically designed to supply blood to the systemic circle leads to the fact that these children still lag behind in development compared to healthy children, although only slightly. Also corrected TMS often combined with other heart anomalies (ventricular septal defects, etc.)

Transposition of the great arteries, or d-transposition, is the most common cause of cyanosis in newborns and the leading cause of death in children with cyanotic heart defects in the first year of life. Previously, this defect was fatal, but now, thanks to the advent of palliative and radical operations, the prognosis for it has significantly improved.

Morphology and hemodynamics

When the great arteries are transposed, the systemic venous return from the right atrium enters the right ventricle, and from there it is ejected into the aorta arising from the right ventricle. The pulmonary venous return passes through the left atrium and left ventricle and returns to the lungs through the pulmonary trunk arising from the left ventricle. Normally, two circles of blood circulation are connected in series, but here they are separated. Life with such blood circulation is possible only if there are communications between the circles, allowing oxygenated blood from the lungs to flow into the arteries of the systemic circle, and blood from the veins of the systemic circle to flow into the pulmonary arteries. In more than half of patients with transposition of the great arteries, the interventricular septum is intact and intracardiac shunt occurs only through a dilated foramen ovale or, less commonly, through an atrial septal defect such as ostium secundum. Cyanosis is very pronounced. With a large ventricular septal defect, the blood mixes better, so SaO 2 is higher in these patients. A patent ductus arteriosus can be found in approximately half of newborns with transposition of the great arteries, but in most cases it closes and obliterates soon after birth. In rare cases, the ductus arteriosus remains wide open; this condition is dangerous and requires timely diagnosis and treatment. In addition, there may be varying severity of left ventricular outflow tract obstruction caused by a subvalvular membrane or fibromuscular cord. Common AV canal, atresia of AV valves, severe pulmonary valve stenosis and atresia, coarctation of the aorta and right-sided arc aortas with transposition of the great arteries are rare.

Transposition of the great arteries leads to severe hypoxemia, metabolic acidosis and heart failure. SaO 2 depends on the discharge of oxygenated blood from the vessels of the small circle into the vessels of the systemic circle and venous blood in the opposite direction. The magnitude of the discharge, in turn, depends on the size of the messages that provide it: the oval window, atrial septal defect such as ostium secundum, ventricular septal defect, patent ductus arteriosus, dilated bronchial arteries. A noticeable effect on hemodynamics, especially with a large ventricular septal defect, is exerted by obstruction of the outflow tract of the left (functionally right) ventricle and an increase in pulmonary vascular resistance; with high resistance to blood flow from the left ventricle, pulmonary blood flow decreases, the return of oxygenated blood from the pulmonary veins decreases, and SaO 2 falls. The disconnection of the two circulations, as a rule, leads to an increase in cardiac output of both ventricles, followed by dilatation of the heart cavities and heart failure. This is further aggravated by the fact that oxygen-poor blood from the veins of the systemic circle enters the coronary arteries.

Damage to the pulmonary vessels with transposition of the great arteries and a large ventricular septal defect is more common and progresses much faster (according to both morphological studies and cardiac catheterization) than with a ventricular septal defect with normal origin of the great arteries. Severe pulmonary vascular obstruction is found in approximately 75% of children with transposition of the great arteries and a large ventricular septal defect older than 1 year. Obstructive damage to the pulmonary vessels does not develop with concomitant stenosis of the pulmonary valve, with timely closure of the ventricular septal defect and early surgical narrowing of the pulmonary trunk. Morphological examination reveals moderate pulmonary vascular damage in many children with ventricular septal defect already at the age of 3-4 months. Therefore, narrowing of the pulmonary trunk or radical correction of the defect should be carried out in more timely manner. early age. Even with an intact interventricular septum, 5% of children who did not die in the first months of life show significant damage to the pulmonary vessels.

Clinical manifestations

With an intact ventricular septum, the condition becomes critical in the first hours after birth, while with a large ventricular septal defect, cyanosis may be mild and symptoms of heart failure appear only several weeks after birth. A sign of poor blood mixing is cyanosis in appearance. healthy child; Nurses are often the first to notice it. Early diagnosis requires a high suspicion of this defect, since, with the exception of persistent cyanosis and progressive tachypnea in the first hours after birth, the child may appear healthy, and there may be no changes on the ECG and chest x-ray.

On auscultation, the second sound is loud, unsplit, best heard from above at the left edge of the sternum, however, with careful auscultation, you can often hear a slight splitting of the second sound with a quiet pulmonary component. With an intact interventricular septum in newborns, there is practically no noise, although in the middle, at the left edge of the sternum, a short mesosystolic murmur of II-III degree of loudness can be heard. In older children, a loud, rough systolic murmur may occur, indicating a ventricular septal defect or left ventricular outflow tract obstruction. In the first case, the murmur is pansystolic, best heard in the middle and below at the left edge of the sternum; and in the latter - decreasing, best heard in the middle at the left edge of the sternum, but carried towards the upper part of the right edge of the sternum.

With transposition of the great arteries and a large ventricular septal defect, severe heart failure and moderate cyanosis develop by the 3-4th week. Tachypnea and sweating increase. Cyanosis may increase, but due to good mixing of the blood it often remains relatively mild. There are congestive wheezing in the lungs and severe hepatomegaly.

In newborns, the ECG is not informative, since deviation of the electrical axis of the heart to the right and other signs of right ventricular hypertrophy are observed normally. However, the persistence of positive T waves in the right chest leads after 5 days indicates pathological hypertrophy right ventricle. Later infants with intact interventricular septum clear signs of hypertrophy of the right atrium and right ventricle appear. With a large ventricular septal defect, signs of left ventricular hypertrophy may also appear in the first months of life.

Diagnostics

Chest X-ray

Changes on the radiograph can range from gross to almost imperceptible. Immediately after birth, the heart shadow is not enlarged, but it increases in the first or second week after birth. The pulmonary vascular pattern is initially normal or only slightly enhanced; its noticeable enrichment appears later. An oval cardiac shadow with a narrow superior mediastinum and a small thymic shadow, classic for transposition of the great arteries, allows an immediate diagnosis, but in newborns it occurs only in a third of cases.

With a large ventricular septal defect, a large rounded heart shadow and a significantly enriched pulmonary vascular pattern are revealed soon after birth.

EchoCG

EchoCG with Doppler study is the main method for assessing the morphology of the defect and hemodynamic features. The aortic root is located in front and to the right of the pulmonary trunk, it arises from the right ventricle, and the pulmonary trunk, located behind and to the left, from the left; the morphology of the ventricles corresponds to their location. Doppler studies clarify the direction and magnitude of intracardiac discharge at different levels; assess the pressure in the ventricles.

Cardiac catheterization

Cardiac catheterization in newborns is performed for therapeutic purposes - to perform balloon atrial septostomy. It is often carried out under the control of echocardiography directly at the patient's bedside. Diagnostic catheterization is performed in a cardiac catheterization laboratory. SO 2 in the pulmonary artery is higher than in the aorta. With an intact interventricular septum, the pressure in the right and left ventricles immediately after birth may be the same; however, within a few days, left ventricular pressure drops to a factor of 2 or more compared to right ventricular pressure (unless there is left ventricular outflow tract obstruction). Before balloon atrial septostomy, pressure in the left atrium is often higher than in the right atrium.

Right ventriculography shows a high anterior position of the aorta arising from the right ventricle. The presence of a ventricular septal defect and the patency of the ductus arteriosus are determined. With left ventriculography, the pulmonary trunk extending from the left ventricle is filled; the integrity of the interventricular septum and the presence of left ventricular outflow tract obstruction can be assessed.

Treatment

After stabilization of the condition using balloon atrial septostomy, alprostadil infusion, tracheal intubation with transfer to mechanical ventilation and correction of metabolic disorders, the patient is examined for several days. It is necessary to identify and characterize all associated defects, as well as clarify the course coronary arteries. In the absence of pulmonary valve stenosis or subvalvular obstruction of the right ventricular outflow tract, an arterial switch operation is performed. This operation is best method treatment of transposition of the great arteries. After it, the blood is released into the vessels of the systemic circle by the left ventricle; it has been shown that in the medium term after surgery the normal function left ventricle and low incidence of postoperative arrhythmias. The operation consists of cutting the main arteries, suturing them to the desired semilunar valves and transplanting the ostia of the coronary arteries into the base of the pulmonary trunk (functioning after the operation as the aortic root). Since the left ventricle ejects blood into the pulmonary trunk, the resistance in which quickly decreases, the pressure in it drops and the mass of the myocardium decreases. Therefore, if the interventricular septum is intact or has a minor defect, the operation should be performed before the 8th week of life, before this has yet happened. Early postoperative mortality after a simultaneous operation to switch arteries into major centers less than 5%. Repeated operations (most often to eliminate postoperative pulmonary artery stenosis) are required in 5-10% of cases. When transposing the great arteries with a large ventricular septal defect, the main difficulties are associated with left ventricular failure, pulmonary hypertension and early defeat pulmonary vessels. In some cases, myocardial ischemia occurs due to impaired blood flow in the transplanted coronary arteries. Early surgery switching of arteries with closure of the ventricular septal defect has significantly improved the prognosis of this group of patients: the five-year survival rate reaches 90%.

Transposition of the great arteries with obstruction of the left ventricular outflow tract

With transposition of the great arteries with an intact interventricular septum, there may be mild or moderate subvalvular obstruction of the left ventricular outflow tract. The causes of obstruction are myocardial hypertrophy (dynamic obstruction) or subvalvular membrane or fibromuscular cord (permanent obstruction). This cord often forms where the interventricular septum bulges into the cavity of the left ventricle (due to high pressure in the right) and approaches the septal leaflet of the mitral valve. Obstruction is usually mild; excision or anastomosis to correct the defect is required only in cases of severe stenosis.

In transposition of the great arteries with a ventricular septal defect and severe subvalvular obstruction of the left ventricular outflow tract clinical picture resembles tetralogy of Fallot. Severe cyanosis and cyanotic crises may occur from birth; The pulmonary vascular pattern on the radiograph is depleted. For less severe obstruction clinical manifestations At first they are not so pronounced, but with age they intensify. The location and severity of obstruction is assessed by echocardiography and left ventriculography.

If a newborn with transposition of the great arteries and a ventricular septal defect has severe obstruction of the left ventricular outflow tract or severe pulmonary valve stenosis or atresia, the safest option is to perform an anastomosis between the systemic and pulmonary circulation. It is very difficult to correct the defect and it is better to do it after 1-2 years. Surgical correction (Rastelli operation) consists of closing the ventricular septal defect with a patch so that the aorta is connected to the left ventricle. The right ventricle is then connected to the pulmonary artery using an external valve anastomosis; this allows one to bypass obstruction of the left ventricular outflow tract.

Transposition of the great vessels is one of the most common heart defects in infants and the most common cyanotic congenital heart defect. infancy(20-30 cases per 100,000 births). TMS occurs in 5-7% of children with congenital heart disease. Among patients with this defect, boys predominate, with a ratio of M/D = 1.5-3.2/1. Among patients with transposition of the great arteries, 10% have malformations of other organs. Less common is the so-called congenital corrected TMS, the clinical picture and treatment tactics of which differ significantly from TMS.

Morphology
The first anatomical description of TMS was given by M. Baillie in 1797, and the term “transposition” itself was introduced in 1814.

Farre as a characteristic of a defect in which the main arteries are discordant with the ventricles, and the atria and ventricles are concordant with each other. In other words, the morphological RA is connected to the morphological right ventricle, from which the aorta arises entirely or mostly, and the morphological left atrium is connected to the morphological left ventricle, from which the pulmonary artery arises. In the most common version of TMS, D-TMS, the aorta is located on the right and in front and the pulmonary artery is located on the left and behind the aorta.

The term “corrected transposition” means another type of defect in which atrioventricular and ventriculoarterial discordance is observed. With corrected transposition, the aorta is located to the left of the pulmonary artery.

Most authors call a defect with isolated ventriculoarterial discordance simple TMS, while the combination of TMS with other defects (usually VSD and pulmonary artery stenosis) is classified as complex forms of TMS. Among all cases of D-transposition of the great arteries, 50% occur with an intact IVS, another 25% with a VSD, and about 20% with a VSD and pulmonary artery stenosis. With so-called simple transposition, there are no additional cardiac abnormalities other than a patent foramen ovale and persistent ductus arteriosus. When TMS is combined with VSD (which occurs in 40-45% of patients), about a third of such patients have small interventricular defects that do not have serious hemodynamic significance.

VSDs are the most common associated cardiac anomalies. They can be small, large and can be localized in any part of the septum. Small membranous or muscular defects may close spontaneously over time. Sometimes there are VSDs of the atrioventricular canal type associated with a single AV valve. Sometimes there may also be a displacement of the tricuspid valve to the left with its location above the interventricular septum (straddling tricuspid valve) and hypoplasia of the pancreas.

Simultaneously with TMS, other additional defects may occur - most often PDA or coarctation of the aorta, coronary anomalies. For D-TMS with an intact VSD, coronary anomalies are more common than for D-TMS with a VSD. LV outflow tract obstruction occurs in less than 10% of cases with TMS with an intact IVS and is usually dynamic due to left ventricular displacement of the IVS because pressure in the right ventricle is higher than in the left. If the septum is displaced forward and to the right, then displacement of the pulmonary trunk is observed with its location above the IVS plus subaortic stenosis. In such cases, one should also expect the presence of anomalies in the structure of the aortic arch, such as hypoplasia, coarctation and other forms of its interruption.

Obstruction of the LV outflow tract occurs in 1/8-1/3 of cases and more often in combination with interventricular defect than with intact TMS. Rarely, obstruction may be caused by abnormalities such as the fibromuscular tunnel, fibrous membrane, and abnormal attachment of the atrioventricular valve leaflets.

Coronary anatomy
Although the anatomy of the epicardial branches of the coronary arteries varies, the two sinuses of Valsalva at the aortic root always face the pulmonary artery and give rise to the main coronary arteries; these are called coronary sinuses.

Because the great arteries are located side by side, the coronary sinuses are anterior and posterior, and the non-coronary sinus faces to the right. If (as usual) the aorta lies in front and to the right, then the coronary sinuses are located on the left in front and on the right behind. Most often (in 68% of cases), the left coronary artery arises from the coronary sinus, located in the left front, and gives rise to the left anterior descending and circumflex branches, and the right coronary artery arises from the coronary sinus on the right side. Often the circumflex branch is absent, but several branches depart from the left coronary artery, supplying the lateral and posterior surfaces of the LV. In 20% of cases, the circumflex branch originates from the right coronary artery (arising from the coronary sinus on the right posterior) and passes posterior to the pulmonary artery along the left atrioventricular groove. In this situation, the left anterior descending artery arises separately from the coronary sinus on the left anterior.

These two types of coronary anatomy occur in more than 90% of D-TMS cases. Other varieties include right single coronary artery (4.5%), left single coronary artery (1.5%), inverted coronary arteries (3%), and intramural coronary arteries (2%). For intramural coronary arteries of the mouth coronary arteries are located at the commissures, and there may be two orifices in the right sinus or a single orifice giving rise to the right and left coronary arteries.

Hemodynamic disorders
With TMS, the pulmonary and systemic circulations are separated (parallel circulation), and the newborn survives only during the period of functioning of the fetal communications (ductus arteriosus, patent foramen ovale). The main factors determining the degree of oxygen saturation of arterial blood are the number and size of communications between the systemic and pulmonary blood flow. The volume of blood flow in the lungs is much higher than normal due to these communications and low pulmonary vascular resistance. Therefore, systemic saturation is most dependent on the so-called effective pulmonary and systemic blood flow- the amount of desaturated blood from the systemic circulation, coming from the systemic circulation to the pulmonary circulation for oxygenation (effective pulmonary blood flow - EBC) and the volume of oxygenated blood that returns from the pulmonary circulation to the large circle for gas exchange at the capillary level (effective systemic blood flow - ESC). The volumes of ELC and ESC must be equivalent (intercirculatory mixing), otherwise the entire volume of blood will move into one of the circulation circles.

Usually, the mixing of blood through the oval window and the closing ductus arteriosus is not enough for complete tissue oxygenation, so metabolic acidosis quickly develops and the child dies. If the patient lives due to septal defects or PDA, then severe pulmonary hypertension with obstructive damage to the pulmonary vessels quickly develops. Insufficiently high LV afterload leads to the progressive development of its secondary hypoplasia.

Time of onset of symptoms
The timing of the onset of symptoms depends on the degree of mixing of blood between parallel circles of the pulmonary and systemic blood flow. Typically, signs of TMS are visible from the first hours after birth (from the moment of constriction of the ductus arteriosus and closure of the oval window), but sometimes they appear after several days or weeks of life if large fetal shunts continue to function or there is a VSD.

Symptoms
Newborns with TMS are more likely to be born at normal birth weight. The neonatal picture of the defect varies from a deceptively safe appearance of an apparently healthy baby to acute total heart failure and cardiogenic shock. Parallel circulation is accompanied by severe hypoxemia, so the leading symptom of the defect is central cyanosis. Blue or purple coloration of the skin and mucous membranes suggests TMS. Not only the timing of the onset of cyanosis, but also its degree is closely related to morphological features defect and the degree of mixing of blood between two parallel circles of blood circulation. On initial physical examination after birth, the baby may appear generally healthy except for a single symptom, cyanosis.

In patients with an intact IVS (i.e. without VSD), cyanosis appears within the 1st hour of life in 56%, and by the end of the first day of life - in 92%. The condition worsens very quickly due to constriction of the ductus arteriosus, within 24-48 hours after birth, with increasing dyspnea and signs of multiple organ failure. PaO2 usually remains at a level of 25-40 mmHg. and almost does not increase when 100% oxygen is given. In the absence of an ASD and with a small size of the oval window, severe acidemia occurs. At the same time, most patients do not have a heart murmur, and the boundaries of the heart are not expanded until the 5-7th day of life. In a small proportion of patients, a weak systolic murmur may be heard at the left sternal border in the middle or upper third due to acceleration of blood flow in the LV outflow tract or closing of the ductus arteriosus. Even a chest x-ray and ECG performed in the first days of life in the maternity hospital may be normal. The defect can be recognized at this time by immediately performing echocardiography.

If a newborn has a large PDA or VSD, the diagnosis of TMS may not be made in time due to an apparently benign condition. Cyanosis in these cases is insignificant and appears only during crying, the boundaries of the heart in the 1st week of life are normal, and the noise may not be heard even if there are communications between the left and right sections due to the equality of pressures in them. In these cases, pronounced tachypnea with relatively weak cyanosis is noteworthy. Such classic signs of PDA as continuous systolo-diastolic murmur and racing pulse are observed in less than half of the patients in this group. When pulmonary vascular resistance decreases significantly, symptoms of severe heart failure increase. Newborns with TMS and a large PDA are at risk of developing necrotizing enterocolitis due to retrograde diastolic flow from the aorta to the pulmonary artery and ischemic damage intestines.

Diagnostics
On a frontal chest x-ray in the first days and even weeks of life in children with so-called simple TMS, the chest x-ray may appear normal or with a slight expansion of the cardiac shadow, although 1/3 of patients do not have cardiomegaly at all. The vascular pattern is not enhanced in 1/3-1/2 of patients and initially there is no oval shape of the heart, although the vascular bundle is narrowed. The right aortic arch is visible relatively rarely - in 4% of children with simple TMS and in 11% with TMS plus VSD.

After 1.5-3 weeks, in almost all patients without obstruction of the LV outflow tract, cardiomegaly progresses due to an increase in both ventricles and RA, which increases with each subsequent study. Characteristic features include an oval shape of the cardiac shadow in the form of an egg lying on its side and a narrowing of the shadow of the upper mediastinum (narrow vascular bundle). Signs of hypervolemia of the pulmonary circulation are clearly expressed.

In some patients, even after adequate balloon atrioseptostomy, there is no sharp increase in the vascular pattern of the lungs in the first 1-2 weeks of life and low arterial O2 saturation may persistently persist. This suggests the presence of persistent vasoconstriction arterial vessels pulmonary circulation, as a result of which an insufficient increase in the volume of pulmonary blood flow minimizes the effectiveness of atrioseptostomy. Previously, when surgical correction was delayed for months after balloon atrioseptostomy, some of these patients experienced a sharp deterioration in their condition with increasing cyanosis due to a progressive decrease in the volume of pulmonary blood flow due to dynamic narrowing of the LV outflow tract.

When TMS is combined with VSD, cardiomegaly and increased vascular pattern of the lungs are significantly pronounced compared to simple TMS. The vessels in the roots of the lungs are sharply dilated, and at the periphery of the pulmonary fields they often appear narrowed due to vasoconstriction. The left contour of the cardiac shadow may be deformed due to the imposition of the shadow of a significantly expanded pulmonary trunk.

The electrocardiogram usually shows electric axle the heart is deviated to the right, there are signs of hypertrophy of the RV and RA (Fig. 5.49). During the first days of life, the ECG may be normal, and after 5-7 days an increasing dynamic appears. pathological deviation the electrical axis of the heart to the right in patients with TMS without VSD. When TMS is combined with VSD, in 1/3 of patients the electrical axis of the heart is located normally. Biventricular hypertrophy is observed in 60-80% of children with TMS plus VSD. A deep Q wave occurs in V6 in 70% of patients with TMS and a large VSD, and in 44% of patients with an intact VSD. Signs of isolated left ventricular hypertrophy are very rare, with TMS in combination with a large interventricular defect, a tricuspid valve displaced to the left and RV hypoplasia.

Doppler echocardiogram visualizes the discordant origin of the main arteries from the ventricles from the epigastric (subcostal) access. In this case, the origin of the aorta from the RV, the parallel course of the aorta and pulmonary artery, the origin of the pulmonary artery from the LV and the branches of the pulmonary artery are visible. Additional signs in conventional transthoracic projections, the relative spatial arrangement of the aorta and pulmonary artery side by side, without the usual decussation, as well as the origin of the coronary arteries from the main vessel emanating from the pancreas are used. The pancreas and RA are significantly dilated. In the projection of the four chambers from the apex, the characteristics of the vessel located behind are specified, i.e. pulmonary artery with a characteristic division into right and left branch. Using Doppler sonography, the discharge of blood through the oval window or the ASD and ductus arteriosus, and significant regurgitation on the tricuspid valve are determined. Additionally, echocardiography clarifies the presence and localization of VSD, obstruction of the LV outflow tract (or pulmonary stenosis), as well as the presence of other additional anomalies (size of the PDA, presence of coarctation of the aorta, shape and functional state mitral and tricuspid valves).

Laboratory data - when examining blood gases, PaO2 and Sp02 were reduced, the level of PaCO2 was increased, and the content of bicarbonate and pH was reduced. IN general analysis blood, increasing polycythemia due to an increase in the number of red blood cells, increased levels of hemoglobin and hematocrit.

TMS in combination with a large VSD
Newborns with this defect may initially have no symptoms other than mild cyanosis, usually occurring when crying or feeding. The murmur may initially be minimal or a systolic murmur of grade 3-4/6 according to Loude, as well as the third heart sound, gallop rhythm, splitting and strengthening of the second sound at the base of the heart can be heard. In such cases, the defect is most often recognized by symptoms of congestive heart failure, usually at 2-6 weeks of life. In addition to shortness of breath, severe sweating, fatigue during feeding, poor weight gain, gallop rhythm, “groaning” breathing, tachycardia, hyperexcitability, hepatomegaly, edema, and increasing cyanosis are observed.

If the VSD is also accompanied by pulmonary stenosis (obstruction of the LV outflow tract) or even pulmonary atresia, then pulmonary blood flow is reduced, and such patients experience severe cyanosis from birth in combination with clinical signs, as in tetralogy of Fallot with pulmonary atresia.
Damage to the central nervous system

Congenital anomalies of the central nervous system are rare in patients with TMS. Hypoxic-ischemic damage to the central nervous system can occur in patients with inadequate palliative correction or if it is not carried out. The most common onset of central nervous system damage is sudden onset of hemiparesis in a newborn or infant. The risk of such a complication is increased in children with hypochromic microcytic anemia in combination with severe hypoxemia. At older ages, disorders cerebral circulation usually occur against the background of severe polycythemia, which is caused by persistent hypoxemia.

Natural evolution of vice
Without treatment, 30% of patients with TMS die in the 1st week, 50% by the end of the 1st month, 70% within the first 6 months and 90% before 12 months of life.
Observation before surgery

Immediately after birth, a continuous intravenous infusion of prostaglandin E1 or E2 is started (starting rate 0.02-0.05 mcg/kg per minute, then the dose is titrated to effect), which is continued until palliative or radical surgical correction is performed. As a rule, due to severe heart failure (or apnea due to the administration of prostaglandin E1), respiratory support (ventilator support) is required. Additionally, diuretics and inotropes are prescribed (usually dopamine infusion ≥5 mcg/kg per minute).

Timing of surgical treatment
In the neonatal period, palliative or immediately radical surgery is required.

Types of surgical treatment
If arterial switching cannot be performed immediately after birth, then a palliative operation is performed - mini-invasive atrioseptostomy with a Rashkind balloon under echocardiographic or angiographic control, in which sufficient blood mixing is achieved when the defect size in the area of the fossa ovale is 0.7-0.8 cm.

Over the past four decades, radical operational TMS correction evolved from the previously used Senning or Mustard procedures with the creation of intra-atrial tunnels that direct blood from the veins of the pulmonary and systemic circulation to the ventricles corresponding to these circles, to the most physiological operation arterial switch(switch), in which the aorta and pulmonary artery are moved to their normal anatomical positions. Most technical difficult stage operation is the simultaneous movement of the coronary arteries. Arterial switching is best performed in the first days or weeks of life. Optimal time radical correction with D-TMS - the first 14 days of life. If for some reason the child is delivered later, pulmonary artery banding + systemic-pulmonary anastomosis is performed first. For an arterial switch operation after banding, an interval of 2 weeks is considered the most acceptable. There are criteria favorable for arterial switch surgery: 1) LV wall thickness - as normal for age; 2) ratio of LV pressure to RV pressure >70%; 3) values of LV volume and myocardial mass equal to age values. H. Yasui et al. (1989) found that in these cases the following conditions are quite safe for the arterial switch operation: 1) LV mass exceeding the norm by 60%; 2) left ventricular pressure >65 mm Hg; 3) LV/RV pressure ratio >0.8.

If TMS is combined with pulmonary stenosis and VSD, then, if necessary, in the first months of life, palliative operations(the type of which depends on the leading hemodynamic disorders), and the main interventions correcting the defect are carried out later. If severe cyanosis is observed, a balloon or open atrioseptostomy and systemic-pulmonary anastomosis are first required to improve blood mixing. Other patients have a more balanced combination of these defects, thanks to which they can feel well for many months without palliative interventions. The classic corrective procedure for these patients is then the Rastelli procedure, which directs blood flow from the LV through the intraventricular tunnel into the aorta and connects the RV to the pulmonary artery through a valve-containing conduit.

The result of radical surgical treatment
IN recent years in foreign cardiac surgery centers, early postoperative mortality ranges from 1.6 to 11-13% with D-TMS with an intact interventricular septum in the absence of other factors high risk. Factors increased risk postoperative mortality are: additional defects in the structure of the heart, congenital coronary anomalies, prematurity or low body weight at the time of surgery, long-term cardiopulmonary bypass during surgery (>150 min). The prognosis is especially aggravated by coronary anomalies such as the origin of all three coronary arteries from one sinus or the intramural course of the coronary arteries.

The incidence of such residual complications as supravalvular pulmonary stenosis, neoaortic insufficiency, and coronary artery strictures is quite low.
Postoperative follow-up

Early experience with arterial switch surgery was accompanied in some cases by supravalvular pulmonary artery stenosis, the incidence of which decreased after the introduction of a pericardial patch to fill tissue deficiency after dissection of the sinuses of Valsalva in the nonpulmonary artery.

Myocardial ischemia due to coronary insufficiency remains the most common cause of postoperative mortality, but in recent years it has become less common with the development of techniques for protecting the myocardium and relocating coronary arteries.

Most common cause reoperations after arterial switch is pulmonary stenosis, which can occur with an incidence of 7 to 21%. It is formed for various reasons, but most often due to inadequate growth of the pulmonary trunk, when the stenosis is localized in the suture area of the pancreas. Sometimes neoaortic regurgitation is observed (5-10%), which is mild and does not progress. Postoperative complications occur more often after intraatrial switch operations; these include atrial arrhythmias, ventricular dysfunction, and artificial atrial communication obstruction.