D tms simple after surgery. Transposition of the great vessels in newborns

Transposition of the great vessels (hereinafter referred to as TMS, TMA) in newborns is of two types. The first is an anomaly in which the aorta begins from the anatomically right ventricle, and the pulmonary artery (hereinafter PA) begins from the anatomically left ventricle. The defect is characterized only by abnormal spatial relationships of the great vessels. The atria, atrioventricular valves, and ventricles are formed and positioned correctly.

The second, more rare case is when, simultaneously with the “confused” arteries, the atria, ventricles and valves are also out of place. This sounds worse, but in fact it is a much more favorable picture, because with such a TMA hemodynamics are practically not impaired.

Let's consider both options and talk about the diagnosis, anatomy, the danger of these defects, as well as when and how exactly they are treated.

Corrected transposition of the great vessels (ICD-10 code - Q20.5)– it’s innate heart defect, which is manifested by inconsistency (discordance) between the atria and ventricles, as well as the ventricles and pericardial arteries.

Despite the inconsistent communication between the chambers, the blood flow is physiological in nature - arterial blood enters the aorta, and venous blood enters the pulmonary trunk. The right atrium is connected to the ventricle through a valve, which is anatomically mitral, and the right ventricle has the structure of the left. From it, blood enters the lungs through the pulmonary artery.

From the lungs, the pulmonary veins connect to the left atrium. Between it and the ventricle there is a valve that repeats the structure of the tricuspid, and the ventricle itself is anatomically represented as right, not left. From it arterial blood enters the aorta.

Differences from the uncorrected form:

There is no isolation of the blood circulation from each other;
The aorta and pulmonary trunk do not intersect, but run parallel;
Simultaneous crossing of the ventricles is observed;
Characteristic disturbances in the structure of conductive fibers and the development in patients various types arrhythmias.

The frequency of occurrence is 0.5% of all birth defects.

Hemodynamics

An isolated defect does not lead to hemodynamic impairment, since organs receive oxygen in the right amount, A venous drainage has no obstacles. The essence of the defect is expressed not in the reverse structure of the heart valves and ventricles, but in the incorrect distribution of intracardiac load.

The right ventricle, which is anatomically left, begins to work with redoubled force. At the same time, the coronary arteries are not able to provide adequate blood flow (the right ventricular artery is much smaller than the left), which leads to its gradual ischemia and the development of angina.

Parallel development of prolapse is also characteristic mitral valve, which is anatomically tricuspid and not adapted to withstand high pressure.

Is it dangerous for newborns?

Since separation of the blood circulation does not occur, the disease in most cases is diagnosed on later(in the first and second decades of life). Average age detection – 12.5 years. In some patients, the defect remains undetected throughout their lives.

The condition of patients worsens with the development of arrhythmia and cardiac ischemia. Arrhythmias accompany the disease in 60% of cases (paroxysmal tachycardia, atrial fibrillation, blockades) and are often the first reason to see a doctor. In another group of patients, due to the fact that the right ventricle does the work of the left and experiences great overload, heart pain occurs like angina pectoris.

The timing will depend on the presence of concomitant defects and complications. Patients with additional defects (septal defects) are characterized by vivid symptoms and early detection of the disease; treatment is required in the first 28 days of life. In other patients, due to satisfactory general condition and a small number of complaints, treatment is carried out as planned.

Treatment is different, since the corrected form has its own characteristics and is accompanied by arrhythmia and ischemic painful attacks. The corrected form of therapy is complemented by the treatment of these complications.

What is an uncorrected (full) TMA?

Complete transposition of the great vessels (ICD-10 code - Q20.3)- This is a critical blue-type congenital heart disease, which is characterized by inverse connection between the ventricles and the pericardial arteries.

With a defect, complete delineation of the blood circulation occurs due to the inverse arrangement of large arterial trunks. The right ventricle is connected to the aorta, the left - to the pulmonary artery. Venous blood, bypassing the lungs, enters the internal organs from the right ventricle and returns through the vena cava. The lungs receive arterial blood from the left ventricle, which returns to it, bypassing organs and tissues. Venous blood does not become arterial, while arterial blood gradually becomes oversaturated with oxygen.

Synonyms: uncorrected TMS, cyanotic TMS, transposition main arteries, TMA.

Depending on the combination with other anomalies, TMS is distinguished, having:

intact septum between the ventricles;
(hereinafter referred to as VSD);
combination and VSD.

Frequency of occurrence of the defect: 5-7% of all congenital heart defects. It happens 3 times more often in boys than in girls. This CHD was first described in 1797 by M. Baillie, and the definition was first given by Abbott.

Anatomy

The aorta is located in front and most often to the right of pulmonary artery and starts from the right ventricle. The PA is located behind the aorta, starting from the left ventricle. Both main vessels run parallel to each other (normally they cross).

There is often an anomalous origin of the coronary vessels. The vena cava approach the right atrium, the pulmonary veins approach the left (as normal).

Hemodynamics

The blood circulation circles are separated:

Flows from the right ventricle into the aorta deoxygenated blood . It circulates through the systemic circulation and comes through the vena cava to the right atrium, from where it again enters the right ventricle.
Arterial blood comes from the left ventricle to the PA. It circulates in the pulmonary circulation and through the pulmonary veins through the left atrium again enters the left ventricle. That is, oxygenated blood constantly circulates in the lungs.

Mixing of blood from 2 circles of circulation and, as a consequence, compatibility with life with such hemodynamics is possible only if there are messages at the level of any parts of the heart or extracardially (outside the heart).

This explains why Possible fetal survival prenatal period . During this period, there are temporary structural features: the oval window between the atria, the ductus arteriosus between the PA and the aorta, and gas exchange takes place in the placenta. Therefore, the existence of the defect does not greatly affect the development of the fetus.

After birth, the baby loses its placenta, and the fetal (only the fetus has) communications are closed. And then several options for the development of pathology are possible:

The movement of blood at the level of any communication always goes in 2 directions, otherwise one of the circles would be completely empty.

Useful video about hemodynamics during transposition of the great arteries:

How dangerous is this?

This defect is critical and incompatible with life. After birth, the child develops deep hypoxia, accompanied by overflow of the small circle. Most newborns die in the first or second month.

Life expectancy increases slightly if the defect is accompanied by the presence of a hole in the heart septum - this allows the blood circulation to communicate with each other. Such a defect is necessary for the continuation of life during the period of preparation for surgery, but if left untreated, the defect quickly leads to heart failure.

Natural course

TMS of any type - critical condition requiring intervention in early childhood. In the absence of surgery 30% of children die within the first week, 50% in the first month, 70% within six months, 90% before the age of 1 year. Survival is determined by the type of defect.

Causes of death: heart failure, hypoxia, acidosis, concomitant pathology (ARVI, pneumonia, sepsis).

When is treatment required?

The timing of treatment will depend on whether the child has a hole between the chambers of the heart. If a septal defect is present, surgery is performed within the first 28 days after birth. If there is no defect, surgery is planned in the first week of life. In some cases (if there is a highly specialized hospital and a surgeon narrow specialization) surgery can be performed on the fetus.

Causes and risk factors

The exact reason has not been established. Genetic inheritance is suspected, but the gene responsible has not yet been discovered. Sometimes the reason is spontaneous mutation when the pregnant woman has not been exposed to any external influences like x-rays, infectious diseases, medications.

Risk factors:

pregnant women over 40 years of age;
alcohol abuse during pregnancy;
infections during pregnancy;
diabetes;
hereditary burden.

The majority of patients are boys with large birth weights. TMA occurs most often in children with chromosomal abnormalities and Down syndrome. Less common are concomitant defects such as communication between the right atrium and the left ventricle.

Symptoms in children and adults

External signs:

Cyanosis of the skin and mucous membranes, which appears immediately or shortly after birth.
This sign is observed in 100% of patients, which is why the defect is also called “blue”.

The severity of cyanosis depends on the size of the shunt opening. When the baby cries, the cyanosis turns purple.
Shortness of breath in 100% of patients.
Normal or increased birth weight. However, by the age of 1-3 months, malnutrition develops due to difficulties in feeding such children, which are caused by hypoxemia and heart failure.
Delayed motor development.
Often there is a lag in mental development.
Repeated acute respiratory viral infections, pneumonia.

Signs revealed during physical examination:

wheezing in the lungs;
II tone is loud, unsplit;
in the absence of concomitant defects, no murmurs are heard in the heart area;
when VSD is present, audible systolic murmur medium strength along the lower half of the left edge of the sternum, caused by the discharge of blood through the VSD;
in the presence of PA stenosis, there is a systolic ejection murmur (at the base of the heart, quiet);
tachycardia;
increase in liver size.

Diagnostics

Laboratory data: blood gas examination revealed severe arterial hypoxemia. Data instrumental methods are presented below.

Differential diagnosis is carried out with other blue-type congenital heart defects.

How is it detected in the fetus: Ultrasound and ECG

Method	Determination time	results
Determining the thickness of the collar space	12-14 weeks (1st trimester)	Thickness more than 3.5 mm
First ultrasound screening	1st trimester	Disturbance of the embryonic anlage of the heart and large vessels
Second ultrasound screening	2nd trimester	Formed transposition of blood vessels, fetal growth restriction
Color Doppler mapping	2nd trimester	Disconnection of blood circulation, transposition of blood vessels
Ultrasound of the heart (fetal echocardiography)	2nd trimester	Separation of blood circulation, transposition of blood vessels, “egg-shaped” heart
Indirect electrocardiography	2nd trimester	Bias electrical axis heart to the left, signs of heart block

If the diagnosis is confirmed, a medical consultation is held. Further tactics:

Pregnant gets comprehensive information about the defect, treatment prospects and possible risks operations;
At the time of childbirth, a woman is hospitalized in a maternity hospital with intensive care and cardiovascular surgery departments;
After delivery is performed surgery.

Treatment

The disease always manifests itself during the neonatal period. The rate of deterioration of the child’s condition depends on the presence and size of accompanying defects that determine the communication between the two circles of circulation. Treatment is surgical only. When making a diagnosis, the indications are absolute.

Preoperative preparation

Obtaining saturation data arterial blood oxygen and its pH.
Measures to correct metabolic acidosis and hypoglycemia.
Intravenous infusion of prostaglandin E1 preparations. This prevents the ductus arteriosus from closing, and the possibility of mixing blood remains. The measure is only a short-term alternative to the Rashkind procedure.
For severe hypoxia - oxygen therapy.
Assessment of the condition of the kidneys, liver, intestines and brain.

Surgical methods can be divided into corrective and palliative.

Palliative operations

Palliative operations are intended to:

reduce hypoxemia by improving blood exchange between the right and left parts of the heart;
create good conditions for the work of the pulmonary circulation;
be technically simple and not create obstacles to corrective surgery in the future.

These requirements are satisfied various methods expansion or creation of an ASD. Of them the most common are the Rashkind operation and the Park method.

In cases where the child has an ASD of sufficient size, the defect can be corrected without palliative interventions. In other cases, corrective surgery is usually preceded by palliative interventions.

Operation Rashkind

In patients without ASD or VSD, surgery should be performed immediately upon admission to the cardiac surgery center. The increase in blood oxygenation obtained with this procedure gives freedom to choose the timing of corrective surgery within 7-20 days after birth.

Progress of the operation:

A folded balloon is inserted through the femoral and inferior vena cava into the right atrium.
It is pushed through the foramen ovale into the left atrium, where it is filled with a liquid radiopaque substance and sharply returned in a straightened form to the right atrium under X-ray or echoscopic control. In this case, the valve of the oval hole is torn off.

The advantage of the procedure is that there is no dissection chest, which usually causes the development of adhesions in this area, and this complicates the subsequent corrective surgery (thoracotomy and isolation of the heart are difficult).

Park equipment

If the child is more than 30 days old the desired effect from the Rashkind operation is often not achieved due to the fact that the valve oval window tightly fixed to the septum, and also due to the greater strength of the interatrial septum. In these cases, the Park technique is used.

Using a blade built into the end of the catheter, the septum between the atria is cut, and then dilation is performed using a balloon.

Complete arterial correction

Corrective operations should radically correct impaired hemodynamics and eliminate compensating and associated defects. The main such interventions include arterial switching and intraatrial correction.

Arterial switching

The bottom line: true anatomical correction of TMS. Optimal time implementation: first month of life.

Progress of the operation:

After putting the patient under anesthesia and dissecting the chest, they begin artificial circulation, which simultaneously cools the blood.
When the temperature decreases, the metabolism slows down, and this protects the body from postoperative complications. The aorta and PA are cut.
Separated from the aorta coronary vessels and connect to the beginning of the PA, which will then become the beginning of a new aorta. The severed aorta is sutured here. A tube is then created from a piece of the patient's pericardium, which is sewn into the new LA and repairs it.

Main complications: supravalvular aortic stenosis, PA; failure aortic valve and/or aircraft valve; heart rhythm disturbances.

Methods of intra-atrial correction (Mustard and Senning)

They long time were the only methods of surgical treatment of transposition of the great arteries. Now these operations are used when it is not possible to perform a complete anatomical correction of the defect.

The bottom line: correction of hemodynamics, the defect itself is not anatomically corrected.

Progress of the operation: the right atrium is dissected, the interatrial septum is completely removed and patches from the patient’s tissue (part of the atrium wall, pericardium) are sewn inside the resulting cavity. As a result, blood flows through the vena cava into the left ventricle, pulmonary artery and lungs, and from the pulmonary veins into the right ventricle, aorta and systemic circle.

Additional corrective surgeries: VSD repair, correction of PA stenosis.

Useful video about TMA correction:

Prognosis and mortality after surgery, duration and quality of life

The prognosis after surgery for both defects is relatively favorable. Patients with complete transposition experience slower physical development, stunted growth, decreased immunity, and a tendency to infectious diseases despite the therapy.

Life expectancy, depending on the usefulness of the operation, may not be reduced, but more often there is a decrease in it by 10-15 years. Patients who live into adulthood and old age adhere to individual medical recommendations for life.

In people with a corrected form, life expectancy is not changed. Patients from this group live to adulthood and old age (70 years or more). The quality of life changes slightly - those operated on are registered with a cardiologist and undergo courses of treatment for arrhythmia, angina pectoris and other concomitant diseases.

Mortality during operations:

Rashkind operation – 9%;
Operation Park – 13%;
Operation Mustard – 25%;
Arterial switching - 10%.

Immediate and long-term consequences of correction

Immediate consequences:

Damage coronary arteries;
Myocardial fiber ruptures and small focal infarctions;
Arrhythmia.

Long-term consequences:

Complete atrioventricular block;
Acute and chronic heart failure;
Atrial paroxysmal tachycardia;
Ventricular flutter and fibrillation;
Developmental delay;
Prolapse of the mitral valves.

Most common reasons negative consequences are:

Traumatic injuries of coronary vessels;
Incomplete elimination of concomitant pathology - septal defect, mitral insufficiency;
Conductive rupture nerve fibers(His bundles, Purkinje fibers).

Observation

Operated patients are monitored for life. Interval - 6-12 months. The goal is timely detection of complications. In the first six months after surgery or if complications arise in long term prevention of bacterial endocarditis is carried out.

Transposition of the great vessels is characterized by rapid development severe complications, critically disrupting work of cardio-vascular system. Without treatment, children die early age. That's why it is necessary to take urgent conservative and surgical measures to correct TMS taking into account its type.

The most common severe disease encountered in pediatrics is transposition of the great vessels. This disease is more common in male infants, and in its most severe form is fatal to a newborn if he is not provided with immediate help.

Transposition of the great vessels is a complex condition in which the child’s aorta comes out of the right cardiac ventricle and transports blood of the venous type, after passing through vascular system returning to right half. The pulmonary artery carries life-giving fluid from the left side to the lungs and returns it to the same side. It turns out that both working circles of blood circulation are not connected, and the blood of the systemic circle remains unsaturated with oxygen, venous.

In this condition, the child's survival is impossible. Complete transposition of the great vessels in children requires immediate surgical intervention.

A newborn can be saved by the presence of an opening in the right and left halves - an open oval window. This defect is often combined with a heart defect. But one such hole is not enough to completely compensate for the lack of oxygen, so the heart literally strains to increase the release of blood, which ultimately causes.

The hole in the interventricular septum partially compensates for the lack of oxygen, but it also provides only an incomplete supply of oxygen to the blood. As a result, in the presence of such serious disorders, the newborn immediately suffers from severe cyanosis, he needs urgent help, because the condition is urgent and can quickly lead to death.

Causes

Can cause transposition of the great vessels in newborns following reasons:

burdened heredity;
late pregnancy, at 35–45 years;
negative environmental conditions;
the use of medications that have a toxic effect on the fetus;
infectious diseases that occurred during pregnancy: ARVI, chickenpox, rubella, measles, mumps, syphilis and others;
diabetes mellitus and other endocrine pathologies;
nutritional disorders and general lack of vitamins during pregnancy;
work of a pregnant woman with toxic substances.

Availability bad habits in the expectant mother it causes colossal harm to the fetus and can manifest itself not only as heart defects, but also other developmental disorders, defects and deformities. The danger comes from the use of alcohol, drugs, and uncontrolled use of medicines and smoking, especially on early stages, since the pathology is formed in the 2nd month of intrauterine life of the fetus.

Classification

Among all congenital heart diseases, transposition of the great vessels accounts for 7 to 15% of all occurring and recorded cases. Based on the presence of accompanying communication routes, the vice is divided into the following types:

TMS combined with hypervolemia or full blood flow in the lungs:

simple transposition (with a hole between the atria or an open “oval window”);
with a hole in the septum between the ventricles of the heart;
with an unclosed ductus arteriosus and the presence of additional channels.

TMS with reduced pulmonary blood flow:

with stenosis of the left ventricular outflow tract;
complex transposition (also with left ventricular stenosis of the heart and a window in the septum between the right and left ventricles).

Features of hemodynamics

When assessing hemodynamics, the following forms of TMS are distinguished:

Full. With it, venous blood from the right ventricle enters the aorta, into the systemic circulation and returns to the right half of the heart. The left ventricle drives arterial blood into the pulmonary artery, into the pulmonary circulation, and then the blood returns to left half hearts.

Corrected. In this condition there is ventricular inversion.

Characteristic symptoms

During intrauterine development In the fetus, the disease does not manifest itself in any way, because the pulmonary circulation is not yet involved, and the blood flow is through the oval window and the ductus arteriosus. A child with such a heart defect is born at term and has normal weight.

But with complete transposition of the great vessels in children, survival is impossible, so death without immediate intervention is inevitable. In the presence of so-called compensatory shunts, that is, holes, venous and arterial blood gets the opportunity to mix and saturate the heart a little with oxygen.

Only with corrected transposition of the great vessels obvious signs is not available, and the baby grows normally until a certain time.

Typical indicators of defects in infants are following signs:

general cyanosis;
rapid pulse;
dyspnea.

If holes are present, cyanosis is limited to the torso, face and neck. In the future, the following manifestations of the disease may occur:

Heart failure, manifested by enlargement of the heart and liver, the appearance of swelling, and rarely ascites.
Even normal-weight newborns experience weight loss in the first three months. Children with signs of the disease are lagging behind in physical and/or mental development and often suffer from viral diseases, which can be complicated by pneumonia.

One of the very serious cardiac pathologies, which also develops even before birth, is transposition of the great vessels. Such an abnormal disorder of the heart structure is extremely severe and requires urgent surgical intervention. Otherwise, the survival rate of patients with this diagnosis is extremely low. What is transposition of the great vessels (TMS), how it manifests itself and how the pathology is treated, we will learn from the material below.

What is transposition of the great vessels?

Transposition of the great vessels is complex congenital pathology heart disease, in which the location of the main cardiac vessels is anatomically incorrect. In this case, the aorta branches from the right heart chamber, and the pulmonary artery from the left. That is, the vessels abnormally changed their location to the exact opposite. With such localization of the main cardiac vessels, serious violation blood circulation in the body. That is, the pulmonary artery transports blood to the lung area, where it is saturated with oxygen. But then, due to an anomaly, the same blood returns to the right ventricle, when it should have been sent to the left chamber of the heart. In turn, the aorta incorrectly transports blood, which again returns to the left chamber. As a result, there is a complete local (separate) blood supply to the entire body and separately to the lungs. Similar condition represents very serious threat for the life of a newborn, while the fetus in the womb can still develop quite normally with such an anomaly. The disease code according to ICD is Q20.3.

Important: according to statistics, almost 50% of newborns with this diagnosis do not even survive to 2 months. More than 60% of young patients do not survive to one year. On average, in the absence of timely surgical intervention, newborns live 3–20 months.

Causes of pathology

Transposition of the great vessels in newborns develops, as mentioned above, exclusively in utero (embryonic). This happens in the first 8 weeks of gestation. The reasons for this abnormal embryogenesis are:

genetic predisposition;
transferred expectant mother viral infections (chickenpox, ARVI, measles, rubella, herpes, mumps, syphilis, etc.);
exposure to radiation on mother and fetus;
taking a certain group of medications;
lack of vitamins in the body of a pregnant woman;
long-term toxicosis;
history of diabetes mellitus in a pregnant woman;
alcohol abuse;
late birth (after 35 years).

Important: TMS is often diagnosed in babies with Down syndrome.

Classification of transposition of the great vessels

Depending on the abnormal type of location of the main cardiac vessels TMS classified in cardiology into three types. The classification looks like this:

TMS is simple. In this case, the main vein and aorta completely changed their positions. And if during intrauterine development this anomaly does not affect the health of the fetus in any way, since the blood mixes through the arterial open duct, then in a newborn baby this very duct closes due to unnecessary reasons. As a result, the process of normal blood mixing is disrupted. If pathology is detected early in a child, the cardiologist prescribes a series of medical supplies, which do not allow the duct to close. Against the background of such therapy, urgent surgical intervention is indicated. This only chance to save a little patient. Otherwise death inevitable.
Simple TMS with defects (atrial and interventricular septum defective). In this case, a hole is formed in one of the named partitions in utero. At first glance, this is good sign, indicating that the small and large circles of blood circulation are still in interaction. However, this does not save the baby, but rather, on the contrary, it delays the moment of detection of cardiac pathology. So, if the hole is very small, then all the signs of pathology are present, and the diagnosis can be made before the situation becomes hopeless. If the hole does not have a small diameter, then the exchange of blood flow occurs to a degree sufficient to ensure the vital functions of the body. But at the same time, all vessels of the small circle suffer critically due to the developing arterial hypertension. Most often, in this case, neither an already made diagnosis nor possible surgical intervention can save the baby, since little patient at this point it is no longer operable.
TMS corrected. Here, the pathology is characterized by an abnormal location not of the vessels themselves, but of the heart chambers. That is, during intrauterine development, the right and left ventricles change places. With this structure, the systemic and pulmonary circulation occur relatively normally, although abnormally. But such patients often have obvious mental and mental retardation. physical development, since the right heart chamber is not intended to physiologically serve the systemic circulation.

Features of hemodynamics with different types of TMS

Regarding the movement of blood along abnormally located channels during different types TMS, it looks like this:

Corrected TMS. Abnormal blood circulation is somewhat modified. Namely, depleted venous blood is flowing through the pulmonary artery, and arterial blood moves through the aorta. In this case, the pathology will look more or less pronounced if the baby also has concomitant heart defects such as dysplasia of the interventricular or atrial septum, valve insufficiency, etc.
Against the background of simple TMS, blood flow from the right chamber of the heart moves into the aorta and then further into the systemic circle. Having passed the trajectory, the blood returns to the same cardiac chamber. Blood from the left ventricle enters the pulmonary artery and then further into the pulmonary circle. Later blood still returns to the left chamber of the heart. In this situation, oddly enough, additional heart defects (septal dysplasia, valve insufficiency, etc.) can save the situation. Against the background of such defects, the blood, although not enough, still mixes. If the baby does not have such defects, the baby dies a few hours after birth.

Symptoms

In newborns with complete TMS, the following signs and symptoms of pathology are noted immediately after birth:

cyanosis (blueness of the upper body);
enlarged liver and heart;
swelling of the body;
change in the shape of the phalanges of the fingers;
tachycardia and heart murmurs;
shortness of breath even at rest;
In rare cases, ascites is detected.

Corrected TMS in a patient may be characterized by the following signs and symptoms:

obvious developmental delay;
frequent pneumonia;
paroxysmal tachycardia plus heart murmurs;
atrioventricular block.

Diagnostics

To put accurate diagnosis, specialists use certain research methods. Early diagnosis Transposition of the great vessels includes the following techniques:

Initial examination of the patient and listening to the heart.
ECG to detect heart sounds and conduction of electrical impulses in the myocardium.
Echocardiography (ultrasound). Allows the doctor to assess the location of chambers and vessels, as well as determine their functionality.
Catheterization. Used to assess valve function and pressure in both ventricles.
Radiography. Allows for accurate assessment of heart parameters and location pulmonary trunk.
CT or MRI of the heart. In this case, the doctor receives a complete three-dimensional image of the organ.
Angiography. Here the location of all heart vessels and their performance are assessed.

Important: if a baby’s heart defect is diagnosed in a pregnant woman while she is still pregnant, the woman is offered an artificial termination of pregnancy. If a woman insists on further gestation and birth of the fetus, then the pregnant woman is transferred to a special maternity center, which has everything necessary equipment for immediate diagnosis and possibly surgery immediately after birth.

Treatment

Transposition pathology is treated exclusively operationally. And even then only a corrected form of the defect or a form in which the oval window is not closed (simple). Today, there are several types of surgical interventions, all of which are quite effective if performed in a timely manner. All types of operations can be divided into two types:

Corrective. During the intervention, the doctor completely copes with the anomaly, eliminating it by suturing the aorta and pulmonary artery. The first connects to the left heart chamber, the second to the right.
Palliative. In this case, the purpose of the operation is to significantly improve the functioning of the pulmonary circulation. To do this, an artificial window-tunnel is formed in the atrium zone. After such an operation, the right chamber of the heart will direct blood to the lungs and further into the systemic circulation.

The following palliative operations are mainly used:

Closed atrial balloon septostomy. It is indicated only for babies in the first month of life from birth, since their atrial septum remains elastic, which allows it to be ruptured by a balloon quite easily. Later, the septum thickens, making it difficult for the surgeon to perform the procedure using a catheter balloon.
Operation Park-Rushkind. Its greatest effectiveness is observed if the patient is 2 or more months old. Here, a special catheter with a thin blade is used to create a hole in the atrial septum. Using a blade, a cut is made in the septum and then the hole is inflated using a balloon.
Blalock-Hanlon operation. It is used if the first two types of interventions are ineffective.

The types of operations that can be used to correct hemodynamics include the following:

Operation Jaten. Here the surgeon performs anatomical displacement of all vascular main tracks(arteries) and at the same time in parallel swaps the mouths of the coronary arteries at the pulmonary trunk.
Mustard's operation and Senning's operation. Here the doctor uses special patches that are installed after effectively cutting the septum. Such patches change the direction of blood flow according to the anatomical norm. That is, now blood will flow from the pulmonary trunk into the right chamber, and from the vena cava into the left ventricle.

Important: The effectiveness of corrective operations is approximately 80–90%. Only 10% of patients who undergo surgery still die. Survivors develop complications such as narrowing of the lumen of the mouths of the pulmonary or vena cava (gradual) or.

Forecast

As for the prognosis for TMS, then complete transposition main vessels, only 20% of babies have a chance of survival. Approximately 50% of children with this defect die before 2 months. Another 60% may not even survive 1 year.

With simple transposition of the great vessels, almost 70% of children have a chance of life if the operation is performed in a timely manner. The effectiveness of the operation is almost 90%.

Corrected TMS can also be corrected 96% of the time with surgery.

Important: all patients diagnosed with TMS and who have undergone surgery receive disability and are under the supervision of a cardiologist for life in an outpatient (day) hospital. Contraindicated physical exercise for life.

Prevention

Preventative measures for TMS should only be taken by a woman who is planning a pregnancy or is already pregnant. So, if the expectant mother has chronic diseases(diabetes mellitus, etc.), it is advisable to first consult with an endocrinologist and gynecologist about the risk of developing fetal abnormalities.

A pregnant woman should protect herself from viral infections and eat a properly balanced diet so that the body receives required amount vitamins and minerals for the fetus and mother. It is also advisable for a pregnant woman to avoid exposure to radiation and unauthorized use of any medications. In addition, it is extremely important to stop smoking and drinking alcohol.

It is worth understanding that TMS is most often a heart defect incompatible with life. Therefore, if even at the stage of pregnancy a pathology in the baby was identified, then, based on the conclusion of specialists, it is necessary to hospitalize the mother for further childbirth in a specialized perinatal center, where the baby will receive the necessary prompt assistance immediately after birth. If already in the maternity hospital a woman notices unusual symptoms (blueness of the baby’s body), then it is important to insist on thorough examination baby and undergoing emergency surgery. Only this can save the life of a newborn and relatively cure him.

Transposition of the great vessels is a congenital heart defect, one of the most severe and, unfortunately, the most common. According to statistics, it is 12-20% of congenital disorders. The only way to treat the disease is surgery.

The cause of the pathology has not been established.

Normal heart function

The human heart contains two ventricles and two atria. There is an opening between the ventricle and the atrium that is closed by a valve. Between the two halves of the organ there is a solid septum.

The heart works cyclically, each cycle includes three phases. In the first phase - atrial systole, blood is transferred to the ventricles. In the second phase - ventricular systole, blood is supplied to the aorta and pulmonary artery, when the pressure in the chambers becomes higher than in the vessels. In the third phase there is a general pause.

The right and left parts of the heart serve the pulmonary and systemic circulation, respectively. Blood flows from the right ventricle to the pulmonary arterial vessel, moves to the lungs, and then, enriched with oxygen, returns to the left atrium. From here it is transmitted to the left ventricle, which pushes oxygen-rich blood into the aorta.

The two circles of blood circulation are connected to each other only through the heart. However, the disease changes the picture.

TMS: description

During transposition the main blood vessels change places. The pulmonary artery moves blood to the lungs, the blood is saturated with oxygen, but ends up in the right atrium. The aorta from the left ventricle carries blood throughout the body, but the vein returns the blood to the left atrium, from where it is transferred to the left ventricle. As a result, the blood circulation of the lungs and the rest of the body are completely isolated from each other.

Obviously, this condition is life-threatening.

In the fetus, the blood vessels serving the lungs do not function. In a large circle, blood moves through the ductus arteriosus. Therefore, TMS does not pose an immediate threat to the fetus. But after birth, the situation of children with this pathology becomes critical.

The life expectancy of children with TMS is determined by the existence and size of the opening between the ventricles or atria. This is not enough for normal life, which causes the body to attempt to compensate for the condition by increasing the volume of blood pumped. But such a load quickly leads to heart failure.

The child's condition may even be satisfactory in the first days. The only obvious external sign in newborns is distinct blueness. skin- cyanosis. Then shortness of breath develops, the heart and liver enlarge, and edema appears.

X-rays show changes in the tissues of the lungs and heart. The origin of the aorta can be observed with angiography.

Classification of the disease

The disease comes in three main types. The most severe form is simple TMS, in which transposition of the vessels is not compensated for by additional heart defects.

Simple TMS - complete replacement of the main vessels, the small and large circle are completely isolated. The child is born full-term and normal, since during the intrauterine development of the fetus, blood was mixed through. After children are born, this duct closes because it is no longer needed.

With simple TMS, the duct remains the only way mixing of venous and arterial blood. A number of drugs have been developed to maintain the duct in an unclosed state in order to stabilize the position of a small patient.

In this case, urgent surgical intervention is the only chance for survival for the child.

Transposition of vessels with defects of the interventricular or atrial septum - an abnormal hole in the septum is added to the pathology. Through it, partial mixing of blood occurs, that is, the small and large circles still interact.

Unfortunately, this kind of compensation does not give anything good.

Its only advantage is that the position of children after birth remains stable for several weeks, not days, which makes it possible to accurately identify the picture of the pathology and develop an operation.

The size of the septal defect can vary. With a small diameter, the symptoms of the defect are somewhat smoothed out, but they are observed and allow a diagnosis to be made fairly quickly. But if blood exchange occurs in sufficient quantities for the child, then his condition seems quite prosperous.

Unfortunately, this is absolutely not true: the pressure in the ventricles is equalized due to the communicating opening, which becomes the cause. Lesions of the vessels of the small circle in children develop too quickly, and if their condition is critical, the child becomes inoperable.

Corrected transposition of the great vessels - the location of the ventricles, rather than the arteries, changes: depleted venous blood ends up in the left ventricle, to which the pulmonary artery adjoins. Oxygenated blood is transferred to the right ventricle, from where it moves through the aorta to the systemic circle. That is, blood circulation, although according to an atypical pattern, is carried out. On the condition of the fetus and born child has no effect.

This condition is not a direct threat. But children with pathology usually exhibit some developmental delay, since the right ventricle is not designed to serve a large circle and its functionality is lower than that of the left.

Detection of pathology

The disease is detected in the early stages of fetal development, for example, using ultrasound. Due to the peculiarities of the blood supply to the fetus, the disease before birth has virtually no effect on development and does not manifest itself in any way. This asymptomaticity is main reason the defect is not detected until the birth of the children.

The following methods are used to diagnose newborns:

ECG - it is used to evaluate the electrical potential of the myocardium;
echocardia - is the main diagnostic method, as it gives the most full information about pathologies of the heart and main vessels;
radiography - allows you to determine the size of the heart and the location of the pulmonary trunk; with TMS they are noticeably different from normal;
catheterization - makes it possible to evaluate the operation of valves and pressure in the heart chambers;
angiography is the most exact method to determine the position of blood vessels;
CT heart. PET - prescribed to identify accompanying pathologies to develop optimal surgical intervention.

When a pathology is detected in the fetus, the question of terminating the pregnancy almost always arises. There are no other methods other than surgical intervention, and operations of this level are performed only in specialized clinics. Regular hospitals can only offer Rashkind surgery. This allows you to temporarily stabilize the condition of children with heart disease, but is not a cure.

If a pathology is detected in the fetus, and the mother insists on pregnancy, first of all, care must be taken to transfer it to a specialized maternity hospital, where it will be possible to immediately, immediately after birth, carry out the necessary diagnostics.

TMS treatment

The disease can only be cured by surgery. Best term according to surgeons - in the first two weeks of life. The more time passes between birth and surgery, the more the functioning of the heart, blood vessels and lungs is disrupted.

Operations for all types of TMS have been developed for a long time and are successfully carried out.

Palliative - a number of operational measures are carried out to improve the functioning of the small circle. An artificial tunnel is created between the atria. In this case, the right ventricle sends blood to both the lungs and the systemic circle.
Corrective - completely eliminate the disorder and accompanying anomalies: the pulmonary artery is sutured to the right ventricle, and the aorta to the left.

Patients receiving TMS should be under constant monitoring cardiologist even after the most successful operation. As children grow, complications may arise. Some restrictions, such as a ban on physical activity, must be observed throughout life.

Transposition of the great vessels is a severe and life-threatening heart defect. If you have the slightest doubt about the condition of the fetus, you should insist on a thorough examination using ultrasound. No less attention should be paid to the condition of the newborn, especially if cyanosis is observed. Only timely surgical intervention guarantees the child’s life.

With transposition of the great vessels, the aorta departs from the right ventricle, the pulmonary artery from the left ventricle. As a result, venous blood from the right ventricle enters the systemic circle, and through the venous vessels of the systemic circle it returns to the right side of the heart. Arterial blood from the left ventricle is sent through the pulmonary artery system to the pulmonary circulation, from where it returns to the left parts of the heart. Thus, there are two closed circles of blood circulation, in one venous blood circulates, in the other arterial blood. Venous blood circulates in the large circle, while oxygenated blood circulates in the small circle, which cannot be delivered to the organs and tissues of the body. Under such hemodynamic conditions, patients die in the first 3 months of life.

During intrauterine development, transposition of the great vessels does not lead to circulatory disturbances. Oxidized placental blood entering the right atrium is pumped by the right ventricle into the aorta and further through the vessels of the systemic circle. The other part of the placental blood passing through the oval window and the left atrium is pumped by the left ventricle into the pulmonary artery and through the ductus arteriosus into the descending aorta.

After birth, expansion of the lungs and inclusion of the pulmonary circulation in the blood, severe hemodynamic disorders occur. As long as the ductus arteriosus and the foramen ovale are open, since some of the blood passes from the venous to the arterial bed and back, this patient can be helped by surgical intervention. As soon as the physiological closure of the ductus arteriosus and foramen ovale occurs, and if there is no other way for blood to mix, for example, an atrial or ventricular septal defect, patients die quite quickly.

When transposition of the great vessels is combined with a ventricular septal defect, the necessary mixing of blood occurs in the ventricles. In this case systolic pressure both ventricles, aorta and pulmonary artery are located approximately at this level. The minute volume of the pulmonary circulation exceeds the minute volume of the systemic circulation. This is caused by different levels of arterial resistance in the greater and lesser circles. Naturally, more low level resistance of the vascular bed of the pulmonary artery causes a greater flow of blood into the pulmonary circle.

Thus, with transposition of the great vessels in combination with a ventricular septal defect, the magnitude of the minute volume of the greater and lesser circles is directly dependent on the level of arterial resistance of the vessels in both circles. In addition, with this combination of defects, compensatory mixing of blood in the ventricles is observed, which is more favorable for the patient. The degree of blood displacement is determined by an angiocardiographic study.

The anatomical location of the great vessels relative to the ventricles of the heart, from a physiological point of view, does not affect the dynamics of cardiac contraction. The dynamics of cardiac contraction in this pathology is determined by a large defect of the interventricular septum. This anomaly is characterized by an increase in the duration of the isometric contraction phase compared to the norm. This occurs due to the fact that the ventricles of the heart function from a hemodynamic point of view as a single cavity; they have to do more work than normal. And this, in turn, is reflected in systole, it lengthens.

The life expectancy of patients with transposition of the great vessels directly depends on the nature of intracardiac hemodynamics and the degree of mixing of venous and arterial blood.

THE MOST CHARACTERISTIC FUNCTIONAL DISORDERS FOR WHICH CLINICAL DIAGNOSIS OF HEART DEFECTS IS CARRIED OUT

Hemodynamic disorders		Pulmonary blood flow
basic	secondary 1
"pale"vices
A. With excess pulmonary blood flow
The release of blood under pressure from the left ventricle into the right and then into the pulmonary artery	Intense vortex blood flow in the right ventricle during the ejection phase	Excess
The discharge of blood from the left atrium to the right sections sharply increases the release of blood through the relatively narrow outflow tract of the right ventricle with a moderate increase in pressure in it.	Moderate vortex blood flow in the prevalvular section of the right ventricle or in the pulmonary artery during the ejection phase.	Excess	Overload and enlargement of the right heart
Continuous flow of blood under pressure from the aorta to the pulmonary artery.	Intense continuous vortex blood flow in the pulmonary artery	Excess	Overload and enlargement of the left ventricle. In pulmonary hypertension - right ventricular hypertrophy.

1 - Secondary violations are caused by the main ones, but it is from them that it becomes possible to decipher the primary violation.

	Hemodynamic disorders		Pulmonary blood flow	Load changes and heart size
	basic	secondary 1
B. Defects with gateway syndrome
Pulmonary stenosis	The ejection of blood from the right ventricle under pressure through a narrow opening into the pulmonary trunk.	Intense vortex blood flow during the ejection phase in the pulmonary artery (with prevalvular stenosis - in the outlet part of the right ventricle).	Not significantly changed
Aortic stenosis	Ejection of blood from the left ventricle under pressure through a narrow opening into the aorta	Intense vortex blood flow in the aorta during the expulsion phase.	Not significantly changed	Left ventricular overload.
Coarctation of the aorta	Blood flow to the lower half of the body is obstructed. Blood from the pool upper half bodies with high pressure is thrown into the descending aorta: a) through a narrowed lumen; b) through a mass of wide, sclerotic arterial collaterals.	Different levels of pressure and pulsation in the arteries of the upper and lower extremities. Vortex blood flow in the aorta below the narrowing. Vortex blood flow in collateral vessels.	Not significantly changed	Left ventricular overload
Mitral valve insufficiency	The ejection of blood from the left ventricle into the cavity of the left atrium.	Intense vortex blood flow in the left atrium during the ejection phase.	Stagnant overflow of the small circle	Enlargement and overload of the left atrium and left ventricle.

	Hemodynamic disorders			Pulmonary blood flow		Changes in load and heart size
	basic	secondary 1
Aortic insufficiency	Return of blood from the aorta to the left ventricle during the filling phase (diastole).	Vortex blood flow in the left ventricle during the filling phase (diastole).	Not significantly changed		Enlargement and overload of the left ventricle.
CYANOTIC DEFECTS
A. With reduced pulmonary blood flow
Tetralogy of Fallot	Unimpeded release of venous blood from the right ventricle through the VSD into the aorta and through the stenotic area into the pulmonary artery	Arterial hypoxemia syndrome. Vortex blood flow of varying severity in the prevalvular section of the right ventricle or in the pulmonary artery during the ejection phase.	Varying degrees of reduction in pulmonary blood flow		Right ventricular overload
Triad of Fallot	1. Venous blood discharge through the ASD into the left atrium. The ejection of blood from the right ventricle through a narrowed area under pressure into the pulmonary artery. 2. Discharge of arterial blood through the ASD into the right atrium, then into the right ventricle. Release of increased blood volume through the stenotic area into the pulmonary artery.	Arterial hypoxemia syndrome. Intense vortex blood flow in the pulmonary artery during the expulsion phase. Manifestation of stenosis and ASD	Varying degrees of decrease in pulmonary blood flow. Increased pulmonary blood flow.		Right ventricular overload. Overload and enlargement of the right ventricle.

	Hemodynamic disorders			Pulmonary blood flow		Changes in load and heart size
	basic	secondary"
Atresia of the right venous foramen	The discharge of all venous blood into the left atrium through the ASD. Discharge of blood from the left ventricle into the rudimentary right ventricle through a defect.	Arterial hypoxemia syndrome. Vortex blood flow in the cavity of the right ventricle.	Decreased pulmonary blood flow		Left ventricular overload.
B. With excess pulmonary blood flow
Position of mainline vessels	From the right ventricle, venous blood is ejected into the aorta, and from the left ventricle into the pulmonary artery. The child's life is possible only if there is a shunt that ensures blood mixing.	Arterial hypoxemia syndrome. Vortex blood flow corresponds to the level of blood shunting.	Excessive pulmonary blood flow, vascular changes similar to pulmonary hypertension		Overload of both ventricles is possible.
True common truncus arteriosus	The release of blood from both ventricles into the common arterial trunk. The pulmonary arteries are supplied by the aorta.	Pulmonary hypertension syndrome.	Excess pulmonary blood flow, pulmonary hypertension		Overload of any ventricle is possible.
There are many options for transposition of the great vessels. They differ both in the anatomical form of the main anomaly and in the variety of combinations. Possible combination with pulmonary stenosis, manifested by a decrease in pulmonary blood flow.