Tetralogy of Fallot prognosis after surgery. Long-term prognosis for tetralogy of fallot How is it diagnosed?

One of the most severe congenital heart defects in children belonging to the blue type is tetralogy of Fallot. This anomaly becomes a frequent cause of death of a child in infancy or significantly shortens his life. On average, children with unoperated tetralogy of Fallot survive only up to 12-15 years, and less than 5% of patients survive until the age of 40. With such a heart defect, the child may lag behind in physical or mental development. And the cause of death of such patients is ischemic stroke, provoked by vascular thrombosis, or brain abscess.

Tetralogy of Fallot is a complex congenital heart defect and is accompanied by the following four characteristic morphological signs: extensive ventricular septal defect, stenosis (narrowing of the lumen) of the outflow tract of the right ventricle, unnatural location of the aorta and myocardial hypertrophy of the walls of the right ventricle. This anomaly of cardiac development received its name from the French pathologist E.L.A. Fallot, who first described its anatomical and morphological characteristics in detail in 1888.

The severity and nature of the symptoms of tetralogy of Fallot depends on many morphological features present in each specific clinical case, and the severity of such a defect is determined by the measure of stenosis of the outflow tract of the right ventricle, the mouth of the pulmonary artery and the size of the defect in the septum of the cardiac ventricles. The greater the degree of these anatomical anomalies, the more severe the clinical manifestations and course of the defect.

Cardiac surgery is indicated for all children with tetralogy of Fallot, which in many cases may consist of more than one operation. As a rule, one of these interventions is palliative, and the second involves radical surgical correction of the existing anomalies.

In this article, we will introduce you to the presumed causes, forms, symptoms, methods of diagnosis and surgical correction of tetralogy of Fallot in children. This information will help you understand the danger and essence of this anomaly, and you can ask your doctor any questions you have.

Causes

Some viral infections suffered by a pregnant woman mainly in the first trimester can cause the development of congenital heart disease in the fetus

Anatomical abnormalities in the structure of the heart are formed in the fetus at 2-8 weeks of embryogenesis. The reasons for changes in normal cardiogenesis can be factors affecting the body of a pregnant woman, which cause the development of other congenital defects:

taking certain medications;
heredity;
past infections;
bad habits;
work in hazardous industries;
unfavorable environment;
severe chronic diseases.

Tetralogy of Fallot often accompanies such a congenital pathology as Amsterdam dwarfism syndrome.

The formation of the tetralogy of Fallot occurs as follows:

due to improper rotation of the conus arteriosus, the aortic valve moves to the right of the pulmonary valve;
the aorta is located above the septum of the cardiac ventricles;
due to the “rider aorta,” the pulmonary trunk shifts and becomes more elongated and narrowed;
due to the rotation of the conus arteriosus, its septum does not connect with the ventricular septum and a defect is formed in it, which subsequently leads to the expansion of this chamber of the heart.

Varieties

Depending on the nature of the stenosis of the right ventricular outflow tract, four types of tetralogy of Fallot are distinguished:

embryological - obstruction is caused by an incorrect location of the conical septum downwards and/or forward and to the left, the fibrous ring of the pulmonary valve is almost unchanged or is moderately hypoplastic, and the area of maximum narrowing coincides with the level of the demarcating muscular ring;
hypertrophic - obstruction is caused not only by displacement of the conical septum downward and/or forward and to the left, but also by pronounced hypotrophy of its proximal part, and the area of maximum narrowing coincides with the level of the demarcating muscle ring and the opening of the outflow tract of the right ventricle;
tubular - obstruction is provoked by an uneven distribution of the common arterial trunk and because of this, the pulmonary cone is shortened, narrowed and hypoplastic (with this type of defect, pulmonary valve stenosis and hypoplasia of the fibrous ring may be present);
multicomponent - obstruction is caused by a high departure of the septal-marginal trabecula of the moderator cord or excessive elongation of the conical septum.

Depending on the characteristics of circulatory disorders, tetralogy of Fallot can occur in the following forms:

with atresia (abnormal overlap) of the pulmonary artery;
with cyanosis and varying degrees of narrowing of the pulmonary artery mouth;
without cyanosis.

Hemodynamic disorders

Blood circulation in tetralogy of Fallot is altered due to stenosis of the right ventricular outflow tract and the absence of part of the septum between the ventricles. The severity of such violations is determined by the size of the defects.

With significant narrowing of the pulmonary artery and a large size of the ventricular septal defect, a smaller amount of blood enters the pulmonary bed, and a larger amount enters the aorta. This process causes insufficient enrichment of arterial blood with oxygen and manifests itself as cyanosis. A large septal defect causes a comparison of pressure levels in both cardiac ventricles, and when the mouth of the pulmonary artery is completely blocked from the aorta, blood enters the pulmonary circulation through the ductus arteriosus or other bypass routes.

With moderate narrowing of the pulmonary artery, blood discharge due to high peripheral resistance occurs from left to right and cyanosis does not appear. However, over time, due to the progression of stenosis, the blood discharge becomes cross, and then right-left. As a result, the patient develops cyanosis.

Symptoms

Before birth, tetralogy of Fallot does not manifest itself in any way, and in the future the severity of its symptoms will depend on the size and nature of the anatomical abnormalities.

The main first sign of tetralogy of Fallot is cyanosis, and depending on the time of its occurrence, five clinical forms of this heart defect are distinguished:

early cyanotic – cyanosis appears in the first two to three months of a child’s life;
classic - cyanosis first appears at 2-3 years of age;
severe – the defect is accompanied by the occurrence of cyanotic crises;
late cyanotic - cyanosis first appears at 6-10 years of age;
acyanotic – cyanosis does not appear.

In severe forms of the defect, cyanosis first appears at 2-3 months and is maximally manifested by the first year of the child’s life. Blue skin and shortness of breath occur after any physical activity: feeding, changing clothes, crying, overheating, straining, active play, walking, etc. The child feels weak, dizzy and the pulse quickens. When starting to walk, to alleviate this condition, such children often squat down, since in this position their well-being improves.

In severe forms of the defect, cyanotic crises may appear in the child by the age of 2-5 years. They develop suddenly and are manifested by the following symptoms:

general anxiety;
dyspnea;
increased manifestations of cyanosis;
severe weakness;
increased heart rate;
loss of consciousness.

Over time, such crises appear more and more often. In severe cases, such attacks can end with the onset of hypoxic coma, respiratory arrest and the appearance of convulsions.

Children with tetralogy of Fallot often suffer from acute respiratory viral infections, various inflammatory diseases of the upper respiratory tract and pneumonia. They are often adynamic and retarded in development, and the degree of such deviations depends on the severity of cyanosis.

At an older age, children experience deformation of the fingers and nail plates of the “drumstick” and “watch glass” type.

With the acyanotic form of tetralogy of Fallot, children usually rarely squat, develop well and experience early childhood without problems. After this, they undergo radical cardiac surgery as planned (usually at the age of 5-8 years).

When examining a child with tetralogy of Fallot and listening to heart sounds, the following are revealed:

heart hump (not always);
rough systolic murmur to the left of the sternum in the II-III intercostal space;
weakened II tone in the projection of the pulmonary artery.

Diagnostics

A doctor can suspect tetralogy of Fallot in a child by finding bluish skin in combination with other relevant clinical manifestations (shortness of breath, fatigue, etc.)

A doctor can suspect the presence of tetralogy of Fallot in a child by the bluish color of the skin, a tendency to squat, and characteristic heart murmurs.

To clarify the diagnosis and obtain a complete clinical picture of this congenital anomaly, the following types of studies are prescribed:

chest x-ray – moderate increase in heart size, unclear pulmonary pattern, shoe-shaped heart;
ECG – deviation of the electrical axis of the heart to the right, signs of right ventricular myocardial hypertrophy, incomplete blockade of the right bundle branch;
phonocardiography – a typical picture of murmurs and changes in heart sounds;
Echo-CG – pulmonary artery stenosis, ventricular septal defect, atypical location of the aorta, right ventricular myocardial hypertrophy;
catheterization of the heart chambers - increased pressure in the right ventricle, communication between the ventricles through an existing defect, low oxygenation of arterial blood;
pulmonary arteriography and aortography – the presence of collateral blood flow, pulmonary artery stenosis, patent ductus arteriosus.

If necessary, the examination of the child can be supplemented with MRI and MSCT of the heart, selective coronary angiography and ventriculography.

Treatment

All children with tetralogy of Fallot are indicated for surgical correction of the defect. The method of cardiac surgery and the timing of its implementation depend on the anatomical variant of the anomaly, the severity of its manifestations and the age of the patient.

Before surgery, children are recommended to undergo gentle treatment and drug therapy aimed at relieving cyanotic crises. For this purpose, intravenous infusions of solutions of Eufillin, Reopoliglucin, Glucose and Sodium Bicarbonate are prescribed. To compensate for the lack of oxygen, oxygen therapy is performed.

If drug correction is ineffective, emergency surgery to apply an aortopulmonary shunt is indicated. Such palliative interventions of the anastomosing type include:

intrapericardial anastomosis of the ascending aorta and the right pulmonary artery;
Blalock-Taussig subclavian-pulmonary anastomosis;
anastomosis between the descending aorta and the left pulmonary artery;
imposition of a central aortopulmonary anastomosis with a prosthesis made of biological or synthetic material, etc.

To reduce the manifestations of arterial hypoxemia, the following operations can be performed:

balloon valvuloplasty;
open infundibuloplasty.

Radical corrective operations for tetralogy of Fallot are usually performed before the age of 6 months or up to 3 years, and for the anticyanotic form - at 5-8 years. In the process of such interventions, stenosis of the outflow tract of the right ventricle and a septal defect between the cardiac ventricles are eliminated.

With adequate cardiac surgical corrections, hemodynamics are stabilized and all symptoms of tetralogy of Fallot are eliminated. For six months after the operation, children are recommended to undergo clinical observation by a cardiac surgeon and cardiologist, refuse to attend kindergarten or school, take a gentle regimen with limited physical activity, antibiotic prophylaxis of endocarditis before dental and surgical procedures, and take medications prescribed by a doctor.

Over time, blood circulation in operated patients is completely stabilized, medications are stopped, but observation by a cardiologist remains relevant. Given the fact of the anatomical severity of tetralogy of Fallot and the difficulty of performing cardiac surgical correction, such children are recommended to always limit physical activity in the future. When choosing a profession, this factor must be taken into account.

Timely radical operations to correct tetralogy of Fallot usually give a good prognosis, and patients adapt well socially, become able to work and normally tolerate physical activity adequate for their condition. When such interventions are performed at a later age, long-term results worsen.

Tetralogy of Fallot is a dangerous and complex congenital heart defect, and when such an anomaly is detected, the child’s parents should always understand that only timely cardiac surgery can save the baby’s health and life. In severe forms, two operations have to be performed - palliative and radical corrective. After timely surgical treatment, the prognosis for survival becomes favorable, and children can lead a normal lifestyle, but with some limitation in physical activity.

Channel One, program “Live Healthy!” with Elena Malysheva, in the “About Medicine” section, a conversation about the tetralogy of Fallot (see from 32:35 min.):

Watch this video on YouTube

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Diagnosis and treatment methods, prognosis for recovery.

Tetralogy of Fallot is a severe congenital heart defect with four (tetralogy) characteristic defects:

Strong displacement of the aorta to the right (normally the aorta departs from the left ventricle, with tetralogy of Fallot - completely or partially - from the right ventricle).
Maximum stenosis (narrowing) of the pulmonary artery trunk (normally, blood from the right ventricle passes through it into the lungs and is saturated with oxygen).
Absence of the interventricular septum.
Dilatation (increase in volume) of the right ventricle.

What happens during pathology? Due to defects:

venous and arterial blood mixes in the ventricles and enters the systemic circulation, insufficiently oxygenated;
increases oxygen starvation of tissues and organs, displacement of the aorta and narrowing of the pulmonary trunk (the stronger the stenosis, the less blood is saturated with oxygen in the lungs and the more remains in the ventricle, exacerbating congestion);
serious disturbances in the large (from the left ventricle to the aorta) and small (from the right ventricle to the pulmonary artery) circle quickly lead to the development of chronic heart failure.

As a result, the sick child develops characteristic cyanosis (blueness initially of the limbs, nasolabial triangle, and then all skin), shortness of breath, ischemia of the brain and the whole body.

Cyanosis in a child

Tetralogy of Fallot is a congenital defect; all defects appear during the period of intrauterine formation of the organ and appear immediately after the birth of the child. The average life expectancy is no more than 10–12 years; after surgery, the prognosis improves depending on how severe the malformations of the organ are. Only about 5% of children with the pathology grow up and live to be 40 years old, so it is usually considered a childhood pathology.

If we talk about the differences between childhood and adult manifestations of tetralogy of Fallot, they do not exist; at any age, the development of chronic heart failure can lead to loss of ability to work and severe disability.

The pathology is considered one of the most severe congenital heart defects; it is dangerous due to the rapid development of complications of heart failure and ischemia of organs and tissues. During the first two years, more than 50% of children die from stroke (acute oxygen deficiency of cerebral vessels), brain abscess (purulent inflammation), and attacks of acute heart failure. An unoperated defect leads to a serious delay in the development of the child.

It is impossible to completely cure heart disease; surgical methods can only improve the patient’s prognosis and prolong life. At the same time, there is a direct dependence on the timing of the operation - the earlier it is performed (preferably in the first year of life), the greater the chances of a positive result.

Surgical correction of tetralogy of Fallot is performed by cardiac surgeons, and patients are monitored before and after surgery by the attending cardiologist.

Reasons for appearance

Since the heart begins and forms in the first trimester, the influence of any toxins during the 2nd–8th week of pregnancy is considered especially dangerous for the appearance of tetralogy of Fallot. Most often they become:

medications (hormonal, sedatives, sleeping pills, antibiotics, etc.);
infectious diseases (rubella, measles, scarlet fever);
harmful industrial and household chemical compounds (salts of heavy metals, pesticides and fertilizers);
toxic effects of alcohol, drugs and nicotine.

The risk of a defect increases in families where close relatives had children with intrauterine cardiac anomalies.

Symptoms of pathology

Tetralogy of Fallot is a very severe, life-threatening heart defect; it is quickly complicated by the appearance of pronounced signs of heart failure and cerebrovascular accidents, greatly worsening the prognosis and complicating the patient’s life. From early childhood, any, even elementary, physical emotional activity ends in attacks of shortness of breath, pronounced cyanosis (cyanosis), weakness, dizziness, and fainting.

In the future, attacks can end in respiratory arrest, convulsions, hypoxic coma (due to lack of oxygen in the blood), and in the future, partial or complete disability. The characteristic posture of patients is squatting, tense, in order to alleviate the condition after exercise.

After the operation, the patient’s well-being improves, but physical activity is limited so that it does not provoke the development of shortness of breath and other symptoms of heart failure.

The main signs of the defect are caused by disturbances in the enrichment of blood with oxygen, which is why it is called “blue”.

Characteristic symptoms of tetralogy of Fallot:

Shortness of breath that appears and increases after any activity (crying, sucking)

Severe weakness (caused by the most basic actions)

Loss of consciousness (the last two symptoms are due to progressive cerebral ischemia)

A complication of the pathology is cyanotic attacks, the appearance of which indicates severe hypoxia (oxygen deficiency) and greatly worsens the patient’s prognosis. They usually appear between 2 and 5 years of age and are accompanied by the following symptoms:

Breathing and pulse suddenly increase (from 80 beats per minute).
Shortness of breath increases.
The child is worried.
Cyanosis noticeably intensifies to a purple hue.
Severe weakness appears.
The attack may result in loss of consciousness, convulsions, respiratory arrest, coma, stroke or sudden death.

Due to oxygen deficiency, children with congenital defects lag behind in development, are less able to master various skills (cannot hold their head up, etc.), and often get sick.

Diagnostics

Over time, patients develop characteristic external signs that can be used to make a primary diagnosis:

the most characteristic indicator is acrocyanosis (cyanosis of the peripheral parts - hands, feet, tips of the ears, fingers, nose, and then the whole body);
thickening of the fingertips in the form of “drumsticks” and deformation of the nails in the form of “hour glasses” (convex, round);
retardation in physical development, loss of body weight;
flattened chest (a chest hump appears less frequently).

When listening to the heart, a rough “buzzing” or “scraping” sound is detected.

Tetralogy of Fallot in children is confirmed using hardware methods:

Ultrasound, which is used to determine changes in the size of the chambers of the heart (dilatation of the right ventricle).
The ECG shows incomplete blockade of the conductive branches of the (right) bundle of His and hypertrophy (enlargement, thickening) of the right ventricular myocardium.
Using radiography, a characteristic pattern of the lungs (due to lack of blood supply, they appear translucent) and the heart (increase in size and shape in the form of a boot or shoe, with a raised top of the heart) is recorded.
Dopplerography allows you to determine the direction of blood flow and the diameter of blood vessels.

In a general blood test (instead of the norm), the number of red blood cells (erythrocytes) is doubled. This is explained by the fact that the body tries to compensate for the lack of oxygen by increasing cells that can satisfy this need.

Treatment methods

It is impossible to completely cure the pathology:

in 30% of cases, disorders are combined with other congenital anomalies, which complicates prognosis and treatment;
in 65% of cases, hemodynamic (blood flow) disturbances are so pronounced that surgical treatment improves the patient’s condition for a while, prolonging the period and improving the quality of life, but the pathology gradually progresses, leading to the development of chronic heart failure.

The following treatment methods are used:

Drug therapy (emergency care for cyanotic attacks).
Palliative intervention (preparation for radical surgery, temporary elimination of critical hemodynamic disorders).
Radical correction (restoration of the interventricular septum, relocation of the aortic mouth, expansion of pulmonary artery stenosis, etc.).

Life expectancy and further prognosis depend entirely on how timely the surgery was performed.

Drug therapy

Drug therapy is used as emergency treatment for a cyanotic attack:

oxygen inhalations are used to restore oxygen saturation of tissues and blood;
sodium bicarbonate solution is administered to relieve acidosis (accumulation of metabolic products);
to facilitate the breathing process, use bronchial and antispasmodics (aminophylline);
to prevent shock due to gas exchange disorders, adhesion (clumping of red blood cells) and the formation of blood clots, a plasma substitute solution (reopolyglucin) is administered intravenously.

After the onset of seizures, the child’s prognosis worsens; surgical correction of the defect is needed in the near future.

Temporary elimination of hemodynamic disturbances

Temporary correction or palliative methods are used immediately after birth or at an early age, these include the creation of various anastomoses (connections, paths) between the vessels.

Mild stenosis of the pulmonary artery (closing the lumen up to 50%) is eliminated using catheter balloon vulvoplasty (a tip with a balloon at the end is brought through the vessels using a catheter to the desired hole and inflated several times, expanding the lumen).

Full operation

The optimal time frame for a full-fledged surgical operation to eliminate tetralogy of Fallot is up to 3 years. After this period, predicting the further development and life span of the child becomes increasingly difficult: symptoms of heart failure, cerebral ischemia and oxygen starvation of body tissues increase and increase the chances of developing fatal complications.

Method of operation: radical correction of congenital defect of Fallot.

Goal: restore hemodynamics and gas exchange in tissues and organs, eliminate symptoms of heart failure and cerebral ischemia, improve the condition, quality of life and prognosis of the patient.

How it is performed: The vascular system is connected to a heart-lung machine; the heart does not work at the time of the operation; it is cooled with special solutions:

if necessary, preliminary palliative anastomoses are eliminated;
move the aortic mouth into the left ventricle;
apply a patch to the interventricular septum;
expand the pulmonary artery stenosis, cutting the fibrous ring;
The trunk of the pulmonary artery is expanded by applying a longitudinal flap.

A child with such a defect must be operated on in any case; in most cases, treatment methods and surgery are carried out according to an individually developed plan (depending on the child’s condition).

Emergency intervention is necessary if:

Cyanotic attacks appear or become more frequent, ending in fainting, convulsions and loss of consciousness.
Signs of heart failure have increased (shortness of breath at rest).
The general health and condition of the child noticeably deteriorated.
There is a serious lag in physical and mental development.

Possible complications after surgery

The most common postoperative complications (in 20%) of cases can be:

thrombosis of artificial anastomoses;
attack of acute heart failure;
pulmonary hypertension (increased pressure in the pulmonary artery);
atrioventricular block (impaired conduction between the ventricles and atria);
aneurysm (protrusion) of the right ventricle;
various arrhythmias.

Prognosis for recovery

Tetralogy of Fallot is not uncommon; it is a common congenital heart defect; it is diagnosed in 6–5 newborns. With the development of modern cardiac surgery, pathology has ceased to be a death sentence, but it is still impossible to cure it completely. More than 25% of newborns die during the first weeks of life without waiting for surgery, less than 5% survive to 40 years.

The operation greatly improves the prognosis: children operated on in the first year of life are allowed moderate physical activity, they are able to work and lead an active social life (80%). However, in the long term, hemodynamic disorders still lead to complete or partial disability.

The pathology is complicated by the fact that in 30% it is combined with other intrauterine developmental defects (patent duct of Botall) and genetic disorders (mental retardation, congenital dwarfism, Down syndrome), such a complex worsens the prognosis and shortens the patient’s life.

Patients with a congenital defect of this type are registered with a cardiologist for life and require regular examination and prevention of infective endocarditis after any surgical or dental procedures.

Tetralogy of Fallot: symptoms, diagnosis, correction, prognosis

About 100 years ago, the diagnosis of “tetralogy of Fallot” sounded like a death sentence. The complexity of this defect, of course, allowed for the possibility of surgical treatment, but the operation was carried out for a long time only to alleviate the patient’s suffering, since it could not eliminate the cause of the disease. Medical science moved forward, the best minds, developing new methods, never ceased to hope that the disease could be dealt with. And they were not mistaken - thanks to the efforts of people who dedicated their lives to the fight against heart defects, it became possible to treat, prolong life and improve its quality even with such ailments as tetralogy of Fallot. Now new technologies in cardiac surgery make it possible to successfully correct the course of this pathology with the only condition that the operation will be performed in infancy or early childhood.

The very name of the disease says that its appearance is due not to one, but to four defects that determine a person’s condition: tetralogy of Fallot is a congenital heart defect that combines 4 anomalies:

A defect in the septum between the ventricles of the heart, usually the membranous part of the septum is missing. The length of this defect is quite large.
Increased volume of the right ventricle.
Narrowing of the lumen of the pulmonary trunk.
Displacement of the aorta to the right (dextroposition), to the point where it partially or even completely moves away from the right ventricle.

Basically, tetralogy of Fallot is associated with childhood, this is understandable: the disease is congenital, and life expectancy depends on the degree of heart failure, which is formed as a result of pathological changes. It is not a fact that a person can expect to live happily ever after - such “blue” people do not live to an old age, and, moreover, they often die during the infancy period if surgical intervention is postponed for some reason. In addition, the tetralogy of Fallot may be accompanied by a fifth anomaly of cardiac development, which turns it into a pentade of Fallot - atrial septal defect.

Circulatory disorders with tetralogy of Fallot

Tetralogy of Fallot belongs to the so-called “blue” or cyanotic defects. A defect in the septum between the ventricles of the heart leads to a change in blood flow, resulting in blood entering the systemic circulation that does not bring enough oxygen to the tissues and they, in turn, begin to experience starvation.

Due to increasing hypoxia, the patient’s skin acquires a cyanotic (bluish) tint, which is why this defect is called “blue”. The situation with tetralogy of Fallot is aggravated by the presence of narrowing in the area of the pulmonary trunk. This leads to the fact that a sufficient volume of venous blood cannot escape through the narrowed opening of the pulmonary artery into the lungs, so a significant amount of it remains in the right ventricle and in the venous part of the systemic circulation (this is why patients turn blue). This mechanism of venous stagnation, in addition to reducing blood oxygenation in the lungs, contributes to the fairly rapid progression of CHF (chronic heart failure), which manifests itself:

cyanosis in tetralogy of Fallot

Worsening cyanosis;
Violation of metabolism in tissues;
Accumulation of fluid in cavities;
Presence of edema.

In order to prevent such a development of events, the patient is indicated for cardiac surgery (radical or palliative surgery).

Symptoms of the disease

Due to the fact that the disease manifests itself quite early, in the article we will focus on childhood, starting from birth. The main manifestations of tetralogy of Fallot are caused by an increase in CHF, although in such babies the development of acute heart failure (arrhythmia, shortness of breath, anxiety, breast refusal) cannot be excluded. The appearance of the child largely depends on the severity of the narrowing of the pulmonary trunk, as well as on the extent of the defect in the septum. The greater these disturbances, the faster the clinical picture develops. The appearance of the child largely depends on the severity of the narrowing of the pulmonary trunk, as well as on the extent of the defect in the septum. The greater these disturbances, the faster the clinical picture develops.

On average, the first manifestations begin at 4 weeks of a child’s life. Main symptoms:

A bluish coloration of the skin of a child first appears when crying or sucking, then cyanosis can persist even at rest. At first, only the nasolabial triangle, fingertips, and ears appear blue (acrocyanosis), then, as hypoxia progresses, total cyanosis may develop.
The child is lagging behind in physical development (later he begins to hold his head up, sit up, and crawl).
Thickening of the terminal phalanges of the fingers in the form of “drumsticks”.
Nails become flattened and round.
The chest is flattened, and in rare cases, a “heart hump” forms.
Decreased muscle mass.
Irregular growth of teeth (wide gaps between teeth), caries develops quickly.
Spinal deformity (scoliosis).
Flat feet develops.
A characteristic feature is the appearance of cyanotic attacks, during which the child experiences:
breathing becomes more frequent (up to 80 breaths per minute) and deeper;
the skin becomes bluish-purple;
pupils dilate sharply;
shortness of breath appears;
characterized by weakness, up to loss of consciousness as a result of the development of hypoxic coma;
Muscle cramps may occur.

typical areas of cyanosis

Older children tend to squat during attacks, as this position makes their condition a little easier. On average, such an attack lasts from 20 seconds to 5 minutes. However, after it, children complain of severe weakness. In severe cases, such an attack can lead to a stroke or even death.

Algorithm of actions when an attack occurs

You need to help the child squat down or take a “knee-elbow” position. This position helps reduce venous blood flow from the lower body to the heart, and therefore reduces the load on the heart muscle.
Oxygen supply through an oxygen mask at a rate of 6-7 l/min.
Intravenous administration of beta blockers (for example, Propranolol at 0.01 mg/kg body weight) eliminates tachycardia.
The administration of opioid analgesics (Morphine) helps to reduce the sensitivity of the respiratory center to hypoxia and reduce the frequency of respiratory movements.
If the attack does not stop within 30 minutes, emergency surgery may be required.

Important! During an attack, drugs that increase heart contractions (cardiotonics, cardiac glycosides) should not be used! The action of these drugs leads to an increase in the contractility of the right ventricle, which entails additional blood discharge through the defect in the septum. This means that venous blood, which contains practically no oxygen, enters the systemic circulation, which leads to an increase in hypoxia. This is how a “vicious circle” arises.

What tests are used to diagnose Tetralogy of Fallot?

When listening to the heart, the following is revealed: a weakening of the second tone; a rough, “scraping” noise is detected in the second intercostal space on the left.
Electrocardiography data can reveal ECG signs of enlargement of the right chambers of the heart, as well as a shift of the heart axis to the right.
The most informative is an ultrasound of the heart, which can reveal a defect in the interventricular septum and displacement of the aorta. Thanks to Doppler ultrasound, it is possible to study in detail the blood flow in the heart: the discharge of blood from the right ventricle to the left, as well as the difficulty of blood flow into the pulmonary trunk.

ECG fragment with tetralogy of Fallot

Treatment

If a patient has tetralogy of Fallot, it is important to remember one simple rule: surgery is indicated for all (without exception!) patients with this heart defect.

The main treatment method for this heart defect is surgical. The most optimal age for surgery is considered to be 3-5 months. It is best to perform surgery as planned.

There may be situations where emergency surgery may be required at an earlier age:

Frequent attacks.
The appearance of bluish skin, shortness of breath, increased heart rate at rest.
Marked retardation of physical development.

Usually, a so-called palliative operation is performed as an emergency. During this time, an artificial shunt (connection) is not created between the aorta and the pulmonary trunk. This intervention allows the patient to temporarily gain strength before undergoing a complex, multicomponent and lengthy operation aimed at eliminating all defects in tetralogy of Fallot.

How is the operation performed?

Considering the combination of four anomalies in this heart defect, surgical intervention for this pathology is particularly difficult in cardiac surgery.

Under general anesthesia, a dissection of the chest along the anterior line is performed.
After providing access to the heart, a heart-lung machine is connected.
An incision is made into the heart muscle from the right ventricle so as not to touch the coronary arteries.
From the cavity of the right ventricle, access to the pulmonary trunk is made, the narrowed opening is dissected, and the valves are repaired.
The next step is to close the ventricular septal defect using synthetic hypoallergenic (Dacron) or biological (from the tissue of the heart sac - pericardium) material. This part of the operation is quite complex, since the anatomical defect of the septum is located close to the heart pacemaker.
After successful completion of the previous stages, the wall of the right ventricle is sutured and blood circulation is restored.

This operation is performed exclusively in highly specialized cardiac surgery centers, where relevant experience has been accumulated in the management of such patients.

Possible complications and prognosis

The most common complications after surgery are:

Preservation of narrowing of the pulmonary trunk (with insufficient dissection of the valve).
When the fibers that conduct excitation in the heart muscle are injured, various arrhythmias may develop.

On average, postoperative mortality is up to 8-10%. But without surgical treatment, children's life expectancy does not exceed years. In 30% of cases, the death of a child occurs in infancy from heart failure, stroke, or increasing hypoxia.

However, with surgical treatment performed on children under 5 years of age, the vast majority of children (90%) upon re-examination at the age of 14 years do not reveal any signs of developmental lag from their peers.

Moreover, 80% of operated children lead a normal lifestyle, practically no different from their peers, except for restrictions on excessive physical activity. It has been proven that the earlier a radical operation is performed to eliminate this defect, the faster the child recovers and catches up with his peers in development.

Is registration of a disability group indicated for illness?

All patients before undergoing radical heart surgery, as well as 2 years after the operation, are required to register for disability, after which re-examination is carried out.

When determining the disability group, the following indicators are of great importance:

Are there any circulatory problems after surgery?
Does pulmonary artery stenosis persist?
The effectiveness of surgical treatment and whether there are complications after surgery.

Is it possible to diagnose tetralogy of Fallot in utero?

Diagnosis of this heart defect directly depends on the qualifications of the specialist performing ultrasound diagnostics during pregnancy, as well as on the level of the ultrasound machine.

When an expert-class ultrasound is performed by a high-category specialist, tetralogy of Fallot is detected in 95% of cases up to 22 weeks; in the third trimester of pregnancy, this defect is diagnosed in almost 100% of cases.

In addition, an important factor is genetic research, the so-called “genetic doubles and triples”, which are performed on all pregnant women at one week of pregnancy as screening. It has been proven that tetralogy of Fallot in 30% of cases is combined with other anomalies, most often chromosomal diseases (Down syndrome, Edwards syndrome, Patau syndrome, etc.).

What to do if this pathology is detected in the fetus during pregnancy?

If this heart defect is detected in combination with a serious chromosomal abnormality, accompanied by severe mental development disorders, the woman is offered termination of pregnancy for medical reasons.

If only a heart defect is detected, then a consultation gathers: obstetricians-gynecologists, cardiologists, cardiac surgeons, neonatologists, as well as a pregnant woman. At this consultation, the woman is explained in detail: why this pathology is dangerous for the child, what the consequences may be, as well as the possibilities and methods of surgical treatment.

Despite the multicomponent nature of tetralogy of Fallot, this heart defect is classified as operable, that is, it is subject to surgical correction. This disease is not a death sentence for a child. The modern level of medicine allows in 90% of cases to significantly improve the patient’s quality of life through a complex, multi-stage operation.

Currently, cardiac surgeons practically do not use palliative operations, which only temporarily improve the patient’s condition. The priority is radical surgery performed in early childhood (up to one year). This approach allows you to normalize overall physical development and avoid the formation of permanent deformities in the body, which significantly improves the quality of life.

Tetralogy of Fallot (TCF) in newborns and prognosis for children after surgery

In the group of congenital heart defects, tetralogy of Fallot ranks firmly in tenth place. The prevalence among “blue” defects is half. In medical reports and reference literature, the abbreviation CHD is often used, which is synonymous with the term “congenital heart disease.”

In the International Classification of Diseases ICD-10, it is included in the group of congenital anomalies under code Q21.3. An unusual combination of disturbances in the formation of the heart and main vessels was described in 1888 by A. Fallot as a separate syndrome. His name remains in the history of medicine.

What anomalies does the syndrome consist of, anatomical features

Tetralogy of Fallot includes a combination of four anomalies:

defect in the interventricular septum;
right-sided position of the aorta (as if “sitting astride” on both ventricles);
stenosis or complete fusion of the pulmonary artery, it lengthens and narrows due to rotation of the aortic arch;
pronounced right ventricular hypertrophy of the myocardium.

Among the combinations of defects with pulmonary artery stenosis and septal defects, there are 2 more forms, also described by Fallot.

The triad consists of:

holes in the interatrial septum;
pulmonary stenosis;
right ventricular hypertrophy.

Pentad - adds to the first option the damaged integrity of the interatrial septum.

In most cases, the aorta receives a large volume of blood from the right side of the heart without sufficient oxygen concentration. Hypoxia is formed according to the circulatory type. Cyanosis is detected in a newborn child or in the first years of a baby’s life.

As a result, the infundibulum of the right ventricle narrows, and a cavity is formed above it, similar to an additional third ventricle. The increased load on the right ventricle contributes to its hypertrophy to the thickness of the left.

The only compensatory mechanism in this situation can be considered the appearance of a significant collateral (auxiliary) network of veins and arteries supplying blood to the lungs. The open botal duct temporarily maintains and improves hemodynamics.

Tetralogy of Fallot is typically associated with other developmental anomalies:

non-closure of the ductus botallus;
accessory superior vena cava;
additional coronary arteries;
Dandy Walker syndrome (hydrocephalus and underdevelopment of the cerebellum);
in ¼ of patients the embryonic right aortic arch remains (Corvisart's disease);
congenital dwarfism and mental retardation in children (Cornelia de Lange syndrome);
defects of internal organs.

Causes

The causes of the anomaly are considered to be effects on the fetus in the early stages of pregnancy (from the second to the eighth week):

infectious diseases of the expectant mother (rubella, measles, influenza, scarlet fever);
taking alcohol or drugs;
treatment with hormonal drugs, sedatives and sleeping pills;
toxic effect of nicotine;
intoxication with industrial toxic substances in hazardous industries;
a hereditary predisposition is possible.

The use of pesticides in the garden without respiratory protection affects not only the health of the woman, but also her offspring

The important thing is that at a short period of time a woman may not notice pregnancy and independently provoke fetal pathology.

Types of tetralogy of Fallot

It is customary to distinguish 4 types of tetralogy of Fallot according to the characteristics of anatomical changes.

Embryological - narrowing is caused by anterior displacement of the septum to the left and low localization. Maximum stenosis coincides with the level of the anatomical demarcation muscle ring. In this case, the structures of the pulmonary valve are practically unchanged, moderate hypoplasia is possible.
Hypertrophic - pronounced hypertrophy of the exit zone from the right ventricle and the dividing muscle ring is added to the mechanism of the previous type.
Tubular - obstruction is caused by incorrect division in the embryonic period of the common arterial trunk, which is why the pulmonary cone (the future of the pulmonary artery) turns out to be underdeveloped, narrowed and short. At the same time, it is possible to change the valve apparatus.
Multicomponent - all of the listed factors are partially involved in the formation.

Features of hemodynamics

The severity of the defect is determined by the degree of narrowing of the diameter of the pulmonary artery. To diagnose and determine treatment tactics, it is important to distinguish three types of anomalies:

with complete fusion (atresia) of the arterial lumen: the most severe disorder, with a large interventricular foramen, the mixed blood of both ventricles is directed predominantly to the aorta, oxygen deficiency is pronounced, in the case of complete atresia, blood enters the lungs through the open ductus arteriosus or through collateral vessels;
acyanotic form: with moderate stenosis, the obstacle to the blood flow from the right ventricle can be overcome by a lower pressure than in the aorta, then the blood discharge will take a favorable path from the artery to the vein, the defect variant is called “white”, since cyanosis of the skin does not form;
cyanotic form with stenosis of varying degrees: caused by the progression of obstruction, discharge of blood from right to left; this causes a transition from the "white" form to the "blue" form.

Symptoms

The clinical picture appears:

significant cyanosis - located around the lips, in the upper half of the body, intensifies when the child cries, feeds, strains;
shortness of breath - has a paroxysmal nature associated with physical activity, the child takes the most comfortable “squatting” position, due to a temporary reflex additional spasm of the pulmonary artery and the cessation of blood oxygen saturation by 2 times;
fingers in the shape of “drumsticks”;
physical underdevelopment and weakness of children; running, outdoor games cause increased fatigue, dizziness;
convulsions - associated with hypoxia of brain structures, blood thickening, and a tendency to thrombosis of cerebral vessels.

The form of the disease depends on the age of the child, and it determines the adequacy of compensation; in a newborn, cyanosis is visible on the face, hands and feet

early manifestations in the form of cyanosis immediately after birth or in the first 12 months of life;
The classic course is considered to be the manifestation of cyanosis at two to three years of age;
severe form - paroxysmal clinical picture with shortness of breath and cyanosis;
late - cyanosis appears only by 6 or 10 years;
acyanotic form.

An attack of shortness of breath can occur at rest: the child becomes restless, cyanosis and shortness of breath intensify, the heart rate increases, loss of consciousness with convulsions and subsequent focal manifestations in the form of incomplete paralysis of the limbs is possible.

Diagnostics

The diagnosis is made by observing the child and the presence of objective signs. Information from relatives about development and activity, attacks with loss of consciousness and cyanosis are taken into account.

When examined in children, attention is drawn to the cyanotic nature of the lips and the altered shape of the terminal phalanges of the fingers. Rarely does a “heart hump” form.

On percussion, the borders of the heart are not changed or expanded in both directions. During auscultation, a rough systolic murmur is heard to the left of the sternum in the fourth intercostal space due to the passage of blood flow through the hole in the interventricular septum. It is better to listen to the patient in the supine position.

On the radiograph, the contours of the cardiac shadow resemble a “shoe” directed to the left

Due to the absence of the pulmonary artery arch, retraction occurs in the place where the vessels are usually located. A depleted lung appears more transparent. Enlargement of the heart to a large size does not occur.

A general blood test determines the adaptive response to hypoxia in the form of an increase in the number of red blood cells and an increase in hemoglobin.

Ultrasound diagnostics using a conventional ultrasound machine or Doppler ultrasound allows you to accurately determine changes in the chambers of the heart, abnormal development of blood vessels, the direction and magnitude of blood flow.

The ECG shows signs of right-sided cardiac hypertrophy, possible right bundle branch block, and the electrical axis is significantly deviated to the right.

Probing of the cavities of the heart with measurement of pressure in the chambers and vessels is carried out in specialized clinics when deciding on surgical treatment.

Less commonly, coronary angiography and magnetic resonance imaging may be required.

In differential diagnosis, it is necessary to exclude a number of diseases:

transposition of the pulmonary artery causes a significant enlargement of the heart as the child grows;
fusion at the level of the tricuspid valve promotes hypertrophy not of the right, but of the left ventricle;
Eisenmenger's tetralogy - a defect accompanied not by fusion, but by dilation of the pulmonary artery; its pulsation and characteristic pattern of pulmonary fields are determined on an x-ray;
stenosis of the pulmonary artery lumen is not accompanied by a “shoe” picture.

Doppler ultrasound helps to distinguish atypical forms.

Treatment

Drug therapy for a patient with tetralogy of Fallot is carried out only for the purpose of preparing for surgery or in the postoperative period. The only goal is to support the myocardium, preventing possible thrombosis after attacks and impaired coronary and cerebral circulation.

inhalation of an oxygen-air mixture through nasal catheters or in an oxygen tent; newborns are kept in special intensive care units to reduce hypoxia;
A solution of Reopoliglyukin, Eufillin is administered intravenously (in the absence of tachycardia);
due to tissue acidosis, sodium bicarbonate solution is required.

It is impossible to treat a patient without surgical assistance

Operations can be:

emergency measure of temporary assistance;
shunt type for relieving blood flow along a new channel;
a radical choice with correction of the ventricular septal defect and the location of the aorta.

As an emergency, the creation of an artificial connection (anastomosis) between the aorta and pulmonary artery using a prosthesis is used.

It is used as the first stage of surgical intervention for newborns and young children. It is believed that such actions allow you to prepare the child and avoid complications during further treatment, reducing the risk to 5–7%.

It is necessary to decide on the final planned correction of the defect before the age of three years. Temporary anastomoses can be performed between the subclavian and pulmonary arteries.

Radical surgery includes plastic surgery of the right ventricular exit cone, elimination of a hole in the interventricular septum, and valvotomy (dissection of an overgrown pulmonary valve). It is performed on an open heart and requires the use of a heart-lung machine.

The first days after surgery already show improvement in hemodynamics

Can surgical complications develop?

There is a risk of complications after surgery. These include:

development of acute heart failure;
increased thrombus formation at the anastomosis site;
pulmonary hypertension;
arrhythmias or atrioventricular block;
infective endocarditis;
aneurysm of the wall of the right ventricle.

The success of surgical treatment depends on the timeliness and sufficient preparation of the patient and the experience of cardiac surgeons.

What is the prognosis of patients with the defect?

Children with tetralogy of Fallot, whose parents do not agree to surgery, grow up weak and cannot move enough or play with their peers. Frequent infectious diseases (influenza, ARVI, tonsillitis, sinusitis and other sinusitis, repeated inflammations in the lungs) are typical for them. The average survival age is 12 years.

In adulthood, tuberculosis is often associated. Against the background of the defect, the prognosis of any disease is unfavorable; all diseases are severe, with cardiac decompensation and thrombosis. The most common causes of death are ischemic stroke and brain abscesses. No more than 5% of people with this anomaly live to be 40 years old. As a rule, these are people with a high degree of disability who require outside care.

In severe forms of the defect, 25% of children without surgery die in the first year of life, half in the first month.

All patients are observed by a cardiologist and consulted by cardiac surgeons. Antibiotic prophylaxis is recommended for them annually, and it is recommended to maintain oral health.

In modern medicine, treatment of patients with tetralogy of Fallot is provided by cardiac surgery clinics. After the operation, the patient's cyanosis and asthma attacks disappear. Parents should listen to the opinions of experts. The missed deadline may not be forgiven by a grown-up child.

Rapid heartbeat is a very dangerous symptom! Tachycardia can lead to a heart attack

He can defeat her.

In 1888, the French pathologist Etienne-Louis Fallot was the first to speak in his writings about “cyanotic disease” - a congenital heart defect that combines several anatomical components and can only be detected after the death of the child. The modern view of the group of diseases that have been called tetralogy of Fallot, pentad of Fallot and triad of Fallot has, of course, changed. Surgical treatment prevents the death of the vast majority of newborns and older children, allowing them to live a full life.

All heart defects that are included in the group of Fallot's diseases belong to the so-called “blue” congenital heart disease. They have similar causes and symptoms, diagnostic methods and treatments.

Fallot's triad is a combined heart defect, accounting for up to 1.8% of all congenital heart defects and including three components:

right ventricular outflow tract obstruction or pulmonary valve stenosis;
atrial septal defect;
hypertrophy of the right ventricular myocardium.

Thus, three-component congenital heart disease is a complex disease that appears in a person from birth and causes serious hemodynamic disturbances. The main circulatory problems are caused by pulmonary stenosis. Interatrial communication is only of secondary importance, and hypertrophic processes in the right ventricle are a subsequent compensatory reaction to the ongoing changes.

If the stenosis is moderate, then against the background of an increase in systolic blood pressure in the right ventricle, compensation may be sufficient to maintain a normal level of blood flow. The discharge of blood from the left atrium to the right through the existing defect is insignificant, since the blood pressure of the right atrium is close to normal. With moderate stenosis, the child has no cyanosis (blueness of the skin), but with more severe stenosis, or with a long history of the disease without heart surgery, the signs of Fallot's triad become obvious.

An increase in the intensity of work of the right ventricle and its overload are accompanied by an increase in systolic and diastolic pressure. Against the backdrop of rising pressure in the right atrium, the direction of the blood shunt changes - venous blood is transferred to the left atrium from the right. At this stage of the pathology, characteristic symptoms appear - cyanosis, a drop in minute blood volume in the small circle and an increase in the same indicator in the large circle, the development of tricuspid insufficiency.

The difference between the more common heart defect - tetralogy of Fallot - and Fallot's triad is that its anatomical components are not three, but four:

valvular stenosis of the outgoing department of the right ventricle, or stenosis of the pulmonary valve;
ventricular septal defect, or ventriculoseptal defect (can be perimembranous, muscular, juxtaarterial);
dextraposition of the aorta, that is, its partial departure from the right ventricle;
right ventricular hypertrophy (develops with age).

Tetralogy of Fallot in a fetus can develop as a manifestation of various chromosomal abnormalities - Down syndrome, Edwards syndrome, Patau syndrome, Amsterdam dwarfism, etc. Among all congenital heart diseases, tetralogy of Fallot accounts for 6.5-10% of cases. The main hemodynamic disorders are associated with pulmonary stenosis and ventricular septal defect, which is always large. The latter fact determines the operation of two ventricles in the same mode. Venous blood is discharged into the aorta from the right ventricle through the defect, pulmonary blood flow decreases, and blood oxygen saturation is greatly reduced. A characteristic cyanosis of the skin occurs, due to which the disease is included in the group of “blue” defects.

Tetralogy of Fallot is often combined with other problems in the development of the cardiovascular system - anomalies of the coronary arteries, patent ductus arteriosus, partial abnormal drainage of the pulmonary veins, etc. There are four types of tetralogy of Fallot: embryological, hypertrophic, tubular, multicomponent. In addition, a combination of tetralogy of Fallot with an atrial septal defect is also recorded. This variant of the disease is called the pentade of Fallot (occurs in no more than 1% of cases of congenital heart disease).

The formation of the heart and coronary vessels of the fetus occurs in the first trimester of pregnancy (9-10 weeks). It is at this time that the impact of unfavorable factors on the mother’s body leads to disturbances in the development of the heart in the unborn child. The following factors can provoke the appearance of congenital heart disease, non-closure of septal septa and vascular anomalies:

uncorrected endocrine pathologies, especially hyperthyroidism and diabetes mellitus;
infections - rubella, herpes, influenza and others;
alcoholism and drug addiction;
taking drugs that have teratogenic properties (hypnotics, hormones, tetracyclines, etc.);
exposure to x-rays, receiving a dose of ionizing radiation;
severe vitamin deficiency;
work in hazardous production;
prolonged fetal hypoxia.

Researchers also do not rule out that unfavorable heredity (the presence of any heart defects in the mother or close relatives) may also play a certain role in the formation of the triad, tetrad, and pentad of Fallot. Gene mutations often take part in the formation of defects, as a result of which a child is born with various severe syndromes (more often with Down syndrome).

According to the degree of hemodynamic disturbances, there are two types of Fallot's defects:

acyanotic - the characteristic bluishness of the skin is absent, since the discharge of blood from the right heart to the left is moderate;
cyanotic - there is cyanosis of the skin and mucous membranes, which is caused by chronic hypoxia due to the discharge of blood from the right side of the heart to the left.

Thus, the main sign of pathology - cyanosis of the skin - can manifest itself in different ways, or be completely absent. Its degree varies from slight, when cyanosis is visible only in the area of the nasolabial triangle, to total cyanosis of the entire body skin and mucous membranes. Usually, with serious pulmonary artery stenosis, the baby is born with visually noticeable abnormalities - he is weakened, although he has normal weight, is inactive, there is shortness of breath, and rapid fatigue when sucking the breast.

Regarding the severity of cyanosis, several forms are distinguished during Fallot's defects:

early cyanotic - cyanosis appears from birth or from the first months after birth;
classic - cyanosis is noticeable by 2-3 years of life;
severe - accompanied by attacks of cyanosis and shortness of breath, which begin to develop by the age of 3-4 months;
late cyanotic - blueness of the skin is visible only 6-10 years after birth.

Cyanosis of the baby's skin may become more pronounced with straining, crying, physical activity, tension, bowel movements, and stress. In older children who have already learned to walk and run, any outdoor games or running can provoke tachycardia, chest pain, dizziness and even fainting. After exercise, children are forced to squat down to rest or lie on their sides. The most severe attacks can begin in a child by the age of 2-3 years. They develop suddenly and are accompanied by anxiety, restlessness, weakness, arrhythmia, shortness of breath and loss of consciousness. The attacks are associated with a sharp spasm of the right ventricle and the flow of all venous blood into the aorta through an existing defect in the interventricular septum, which leads to severe hypoxia. Complications of an attack can include convulsions, hemiparesis, apnea, and hypoxic coma.

However, in many children, due to the development of collateral circulation, severe attacks, on the contrary, disappear by the age of 5-6 years.

There are other characteristic symptoms of Fallot's defects that may occur in a child in the future:

strengthening the network of capillaries on the fingertips and on the nail bed;
thickening of the fingers, taking on the shape of drumsticks;
deformation of nails, their convexity;
inability to attend school, limited ability to work;
retardation in physical development;
motor impairment;
recurrent acute respiratory viral infections, sinusitis, tonsillitis, pneumonia;
often - pulmonary tuberculosis.

By the age of puberty, the condition of patients worsens, but remains stable for a long time. Some deterioration in well-being is observed when the weather changes or when there is excitement. Many people with Fallot's defects are able-bodied, but they usually do not survive adulthood. The most typical complications from which the patient dies:

cerebral hemorrhages; More information about operations for subarachnoid hemorrhage
thrombosis of cerebral vessels;
brain abscess;
septic endocarditis;
pulmonary hemorrhage;
rupture of collateral vessels;
massive intrapulmonary thrombosis.

During a physical examination of a patient with a triad, tetrad, or pentade of Fallot, the doctor notes the following signs:

unchanged area of the heart;
presence of systolic tremor;
the limits of relative cardiac dullness are normal;
the volume of tones is satisfactory;
there is a rough systolic murmur along the left edge of the sternum due to pulmonary artery stenosis (aphonic forms of heart disease are also possible);
the second tone over the pulmonary artery is weakened.

Blood tests for Fallot's defects reveal a sharp increase in the amount of hemoglobin, which reflects the body's compensatory reaction for “blue” defects. Also, young forms of red blood cells - reticulocytes - appear in the blood, the life cycle of platelets decreases - thrombocytopenia develops. When making a diagnosis, radiography plays an important role, which reveals increased transparency of the lung tissue against the background of depletion of the vascular bed. A network of hilar zone collaterals is often detected. The heart is of normal size, or is already slightly enlarged due to the growth of the right ventricle.

Final confirmation of the diagnosis is possible only after conducting a whole range of studies:

ECG - there are signs of hypertrophy and overload of the right ventricle, a shift of the heart axis to the left.
Ultrasound of the heart (ECHO) - a defect of the interatrial and interventricular septum and displacement of the aorta are detected. The addition of Doppler ultrasound will allow a detailed study of the cardiopulmonary blood flow and identify pathological blood discharge.
Angiocardiography or cardiac catheterization - during the examination, the catheter easily passes from the right ventricle into the aorta, but cannot penetrate the pulmonary artery.

In general, only with the help of angiography or MRI angiography can one accurately establish the diagnosis and its individual characteristics for such complex defects as triad, tetralogy, and pentade of Fallot. The pathology should be differentiated from isolated types of congenital heart defects, from the Eisenmenger complex.

In case of Fallot's triad, only when the pathology is complicated by irreversible pulmonary hypertension, surgical treatment is not indicated. In other cases, surgery is recommended, before which conservative treatment may be recommended:

sedatives;
cardiac glycosides;
beta blockers;
oxygen therapy.

Unlike other types of heart defects, which often require prior palliative surgery, such interventions are not practiced with Fallot's triad due to the risk of even more severe cardiac overload. Early radical correction of the defect in one or several stages is recommended - Broca's valvotomy, closure of the atrial septal defect, etc. After the operations, the pressure in the right ventricle drops, cyanosis decreases, and the volume of pulmonary circulation normalizes.

With tetralogy and pentade of Fallot, surgery is indicated for all patients without exception. The best treatment results are observed if the defect is corrected at the age of 3-6 months.

It will be necessary to urgently operate on a child at an early age if there is severe cyanosis or frequent seizures. It is possible to perform palliative operations, because radical interventions at an early age (up to 3 years) threaten various complications. Palliative operations are used:

Potts operation (creation of an anastomosis between the pulmonary vessels and the arterial system);
Cooley's operation (an anastomosis between the pulmonary artery and the ascending aorta);
Broca's operation (removal of pulmonary valve stenosis).

If you perform palliative operations and carry out correction in 2 stages, then the prognosis after surgery is much better, and postoperative mortality does not exceed 5-10%. After 4-6 months, radical surgical treatment should be done. It is very complex, multicomponent and lengthy and is carried out only in highly specialized cardiac centers. After dissection of the chest and connection to the heart-lung machine, the myocardium is cut from the right ventricle. From this cavity, instruments are brought to the pulmonary trunk, the stenotic opening is dissected, and valve repair is performed. Next, using a synthetic material, the defect in the interventricular or interatrial septum is closed. At the end of the operation, the wall of the right ventricle is sutured and blood circulation is restored.

It is prohibited to carry out surgical treatment for current endocarditis, myocardial contraction disorders, circulatory decompensation, and within 3 months after a stroke. After a successful operation and rehabilitation, heavy physical activity, military service, and professional sports are contraindicated for the patient. It is imperative to see a cardiologist and take medications to improve heart function for the rest of your life. The presence of chronic foci of infection in the body, including diseased teeth and tonsils, should not be allowed.

Among the preventive measures are those that will help prevent the impact of any teratogenic factors on the body of a pregnant woman and the fetus. The prognosis for triad, tetralogy, pentade of Fallot depends on the degree of pulmonary artery stenosis: with a mild form of the disease, people can live and remain active for a long time. A poor prognostic sign is the early appearance of cyanosis and shortness of breath. With severe pulmonary artery stenosis without surgery, patients die in childhood or adolescence. The success of the operation provides excellent long-term results and a significant increase in survival. Disability is issued for 2 years after the operation, and then re-examination is performed.

Have all your attempts to cure hypertension been unsuccessful?

Have you already thought about radical measures? This is understandable, because a strong heart is an indicator of health and a reason for pride. In addition, this is at least human longevity. And the fact that a person protected from cardiovascular diseases looks younger is an axiom that does not require proof.

The materials presented are general information and cannot replace medical advice.

Health

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5. Sokolov V.A. Damage control - a modern concept of treatment of patients with critical polytrauma. Vestnik Travmatologii i Ortopedii. 2005; 1: 81-4 (in Russian).

6. Cheatham M.L., Malbrain M.L.N.G., Kirkpatrick A. Results from the international conference of experts on intra-abdominal hypertension and abdominal compartment syndrome. II. Recommendations. Intens. Care Med. 2007; 33: 951-62.

7. Radzikhovskiy A.P., Bobrov O.E., Tkachenko A.A. Relaparotomy. Kiev; 2001: 10-35 (in Russian).

8. Savel"ev V.S., Gel"fand B.R., Filimonov M.N. Peritonitis. M.: Litterra; 2006 (in Russian).

9. Hensbroek P.B., Wind J., Dijkgraaf M.G.W., Busch O.R.C., Goslings J.C. Temporary closure of the open abdomen: A systematic review on delayed primary fascial closure in patients with open abdomen. World J. Surg. 2009; 33: 199-207.

10. Arvieux C., Cardin N., Chiche L. Damage control laparotomy for hemorrhagic abdominal trauma. A retrospective multicentric study about 109 cases. Annales de Chirurgie. 2003; 128 (3): 150-8.

11. Kriger A.G., Shurkalin B.K., Gorskiy V.A. Results and prospects for the treatment of common forms of peritonitis. Khirurgiya. 2001; 8: 8-12 (in Russian).

Received 03/27/2015

UDC 616.126.46-089:616.12-007-053.1-089.168

SUCCESSFUL TRICIPLE VALVE REPLACEMENT 47 YEARS AFTER RADICAL CORRECTION OF TETRALOGY OF FALLOT

T.Yu. Danilov, M.R. Chiaureli, N.A. Putyato, H.G. Isomadinov*

FGBNU Scientific Center for Cardiovascular Surgery named after. A.N. Bakulev" (director - academician of the Russian Academy of Sciences and Russian Academy of Medical Sciences L.A. Bockeria), 121552, Moscow, Russian Federation

Tetralogy of Fallot is one of the most common and studied congenital heart defects. Currently, surgical treatment of this defect is accompanied by low hospital mortality and good immediate results. However, in a significant number of patients, complications requiring repeated surgical intervention occur in the long term after radical surgery. The number of such patients increases every year, which is associated with an increase in the number of patients who have undergone radical correction of tetralogy of Fallot and with an increase in the period of observation of these patients. This report is devoted to the description of a clinical case of correction of tricuspid insufficiency that developed in a patient with tetralogy of Fallot 47 years after radical correction.

Key words: tetralogy of Fallot; repeated operations; tricuspid insufficiency.

For citation: Annals of Surgery. 2015; 2: 45-50.

SUCCESSFUL TRICUSPID VALVE REPLACEMENT 47 YEARS AFTER RADICAL CORRECTION OF TETRALOGY OF FALLOT

T.Yu. Danilov, M.R. Chiaureli, N.A. Putyato, Kh.G. Isomadinov

A.N. Bakoulev Scientific Center for Cardiovascular Surgery, 121552, Moscow, Russian Federation

Tetralogy of Fallot is one of the most widely studied congenital heart defects. Currently, surgical treatment of this defect is followed by low hospital mortality and good immediate results. However, a large number of patients long after total correction has

*Khairulo Gulomzhanovich Isomadinov, graduate student. Email: [email protected] 121552, Moscow, Rublevskoe highway, 135.

complications requiring repeat surgery. The number of such patients increases every year, which is associated with an increase in the contingent of patients subjected to total correction of tetralogy of Fallot and in the following period for these patients. This report is dedicated to the description of a clinical case of tricuspid insufficiency correction in a patient with tetralogy of Fallot, that took place 47 years after total correction.

Key words: tetralogy of Fallot; reoperations; tricuspid insufficiency

Citation: Annaly Khirurgii. 2015; 2: 45-50 (in Russian).

Surgical treatment of tetralogy of Fallot (TF) has a history of more than half a century. Radical correction of this defect was first performed by C.W. Lillechei and R.L. Varco in 1954 in Minnesota (USA), using the cross-circulation technique. A few months after this operation, J.W Kirklin at the Mayo Brothers Clinic (Rochester, USA) performed a radical correction of TF under conditions of extracorporeal circulation and oxygenation. The first radical correction of TF in our country under conditions of artificial circulation was performed by A.A. Vishnevsky in 1957

Currently, TF is one of the most studied congenital cardiac anomalies. Surgical treatment of this defect is accompanied by low hospital mortality and a low incidence of complications in the immediate postoperative period. However, in a significant number of patients, in the long term after radical intervention, complications associated with incomplete correction of the defect or resulting from the progression of pulmonary or tricuspid valve insufficiency are detected. The number of such patients increases every year, which is associated with an increase in the number of patients who underwent radical correction of TF and the period of observation of these patients. Typically, many of these patients require repeat surgical or endovascular treatment. This can be confirmed by the case in our clinical practice of the development of tricuspid insufficiency (TI) in a patient 47 years after radical correction of TF.

A 57-year-old patient was admitted to the clinic with complaints of shortness of breath and fatigue during physical activity, attacks of rapid heartbeat and interruptions in heart function, and swelling of the lower extremities.

From the anamnesis it is known that from birth the patient was diagnosed with a congenital heart defect of the “blue” type. In 1966, at the age of 10, the patient was examined in the department of congenital heart and vascular defects of the Institute of Cardiovascular Surgery of the USSR Academy of Medical Sciences, as a result of which TF was diagnosed. The patient underwent excision of infundibular pulmonary artery stenosis and plastic surgery of the ventricular septal defect with Dacron under conditions of artificial circulation and hypothermia (31 ° C) (surgeon - S.Ya. Kisis, assistants - B.A. Konstantinov, E.M. Zybin, anesthesiologist - S.V. Tskhovrebov), about which a record was found in the archive of operational logs. After the operation, the patient led an active lifestyle and felt well. She noted a deterioration in her condition from the age of 49, when cardiac arrhythmias first appeared and were documented - paroxysm of atrial flutter. At the same time, during an examination in the hospital, moderate insufficiency of the tricuspid valve (TV) was first detected. In 2009, a permanent form of atrial fibrillation was diagnosed, and progression of tricuspid regurgitation was noted.

Upon admission to the hospital, the patient's condition is serious. At the time of hospitalization, she was constantly taking digoxin, bisoprolol, perindo-pril, furosemide, spironolactone, and warfarin. Heart rate - 65-75 beats/min, blood pressure - 115/75 mm Hg. Art. On auscultation of the heart, the heartbeat was irregular, the first sound was weakened, and a systolic murmur was heard at the base of the xiphoid process. There was an enlargement of the liver by 5-6 cm from the edge of the costal arch. According to Holter ECG monitoring, atrial fibrillation was confirmed with an average heart rate of 65 beats per minute (minimum rate - 34 beats / min, maximum - 131 beats / min), 211 pauses lasting from 2 to 3 seconds were recorded at night, single ventricular extrasys-

tables (429 per day). X-ray of the chest organs revealed a significant expansion of the heart shadow in diameter due to the right atrium and right ventricle (CTI - 63%). According to the echocardiography study, total insufficiency of the TC was noted, caused by dilatation of the fibrous ring (47 mm) and incomplete closure of the leaflets due to prolapse of the anterior leaflet and limited mobility of the septal leaflet pulled to the interventricular septum, right atriomegaly (76 x 78 mm). There is no blood discharge at the level of the heart septa, the systolic pressure gradient between the right ventricle (RV) and the pulmonary artery is 8 mm Hg. Art., linear dimensions of the left ventricle (LV): EDR - 5.4 cm, ESR - 3.0 cm. LVEF - 60%; RV EF - 52%.

The patient underwent tricuspid valve replacement under conditions of cardiopulmonary bypass (120 min), hypothermia (1°C - 28 °C) and pharmacocold cardioplegia (36 min, Сш1оёу1). During the inspection, dilatation of the fibrous ring of the valve up to 60-65 mm, thickening, myxomatous degeneration, scalloping and pronounced prolapse of the anterior and posterior leaflets, limited mobility of the septal leaflet spread along the interventricular septum were found.

partition (see picture). Reconstructive surgery was considered impossible. The valve leaflets are excised, after which a biological valve with leaflets from the Glissonian capsule of cattle liver (“BioLAB”-33) is implanted into the tricuspid position using a continuous suture. After restoration of coronary blood flow, sinus rhythm was restored, which turned into atrial flutter during hemostasis.

The early postoperative period was complicated by an episode of ventricular tachycardia with hemodynamic disturbances (on the 2nd day). After electropulse therapy, sinus rhythm was restored. On the 3rd day after surgery, the patient was extubated and transferred to the surgical department. During the first two weeks after surgery, moderate heart failure was observed. Hemodynamic support was provided by adrenaline (0.05 mcg/kg/min) and dobutamine (0.8 mcg/kg/min). At the same time, blood pressure was 115/70 mm Hg. Art., CVP - 8-9 mm Hg. Art. According to echocardiography, the function of the TC prosthesis was satisfactory: movement of the obturator elements in full, peak diastolic gradient - 5.2 mm Hg. Art., average - 3.1 mm Hg. Art., EF of the left and right ventricles of the heart - 55 and 50%, respectively.

responsibly. The ECG recorded sinus rhythm with a heart rate of 80-90 per minute. After conservative treatment, including cardiotonics, massive antibiotic therapy, amiodarone, potassium supplements, diuretics, and anticoagulant therapy, the patient was discharged from the hospital in satisfactory condition. According to daily ECG monitoring, before discharge from the hospital, the patient had sinus rhythm with an average frequency of 81 beats/min (maximum - 97 beats/min, minimum - 66 beats/min), a rare single atrial extrasystole was noted (143 per day ), no increase in the Q-T interval was recorded. An X-ray examination before discharge from the clinic showed a decrease in the shadow of the heart (CTI - 58%).

The concept, or the very concept of the need for reoperations after radical correction of TF, appeared 5 years after the first publication on intracardiac correction of TF. W Payne et al. in 1961 they published a report on the first repeated operations performed after radical correction of TF. The mortality rate for re-operations given in this report was 29%. Currently, mortality during reoperations for long-term complications is no higher than mortality during radical correction of the defect. The incidence of the need for reoperations after radical correction of TF is quite variable. According to various research groups, from 5 to 20% of patients require re-intervention in the long term. According to J. Monro et al., the rate of freedom from reoperation after 10 years is 91% and 20 years after radical correction of TF - 89%.

The most common reasons for repeated surgical interventions in the long term after radical correction of TF are residual stenosis of the outflow tract of the right ventricle or pulmonary arterial tree, recanalization and residual ventricular septal defects, pulmonary or tricuspid valve insufficiency.

A fairly significant and significant problem in the structure of long-term postoperative complications is TC insufficiency. J. Kobayashi et al., who analyzed long-term

after radical correction of TF, echocardiography data from 133 patients revealed tricuspid insufficiency in 48% of patients, while moderate or severe regurgitation was detected in 19% of the examined patients.

Tricuspid insufficiency in the long-term period after correction of TF is a consequence of valve damage during radical correction of the defect or is caused by the development of RV dysfunction. Damage to the valve is most often associated with improper fixation of the patch on the ventricular septal defect, leading to deformation and limited mobility of the leaflets in the area of the annulus fibrosus adjacent to the edge of the defect. In addition, rupture or perforation of the valve leaflets is possible, which occurs when sutures are placed that fix the patch on the ventricular septal defect, not to the fibrous ring, but to the body of the valve leaflet.

Another reason for the development of TC insufficiency is dilatation and dysfunction of the pancreas that occurs in the long term after correction of tetralogy of Fallot. Overstretching of the myocardium and passive dilatation of the fibrous ring of the bladder lead to the development of tricuspid regurgitation, even with an anatomically normal and intact chamber. Subsequently, dilatation of the pancreas and the TC ring due to uneven tension on the chords can lead to spontaneous rupture of the chords, worsening coaptation of the leaflets and other morphological changes in the valve and subvalvular apparatus.

In our opinion, both of these mechanisms played a role in the pathogenesis of TN in our patient. The iatrogenic genesis of tricuspid regurgitation is supported by an entry in the surgical protocol from 1966: “During the excision of infundibular stenosis, one of the papillary muscles of the right ventricle was cut off from the place of atypical attachment, which was fixed with one U-shaped suture to the place of its attachment " At the same time, taking into account the rather long interval between radical correction of the defect and the first signs of tricuspid regurgitation (~42-43 years), we can assume that the iatrogenic factor is not the only one in the genesis of TN in our patient.

Apparently, an important role was played by the developed dysfunction of the right ventricle, the causes of which, in the absence of pulmonary regurgitation, could be surgical trauma to the right ventricle (incision and scar on the outflow tract) during radical correction of the defect, with the development of zones of hypo- or akinesis.

To correct tricuspid regurgitation in adult patients with congenital heart defects or after correction of congenital heart defects, both reconstructive methods and valve replacement are used. The most important determining factors in choosing a correction method are the nature and severity of morphological changes in the TC, the degree of regurgitation and the age of the patient at the time of correction. The use of reconstructive techniques, although it has a number of advantages (preservation of the native valve, minimal increase in time and risk of surgery, ease of implementation and immediate effectiveness), as a rule, does not eliminate residual regurgitation, which in most cases is minimal. At the same time, valve replacement, despite a number of disadvantages (tissue degeneration of the implanted biological prosthesis, the need for reoperation, etc.), with pronounced morphological changes in the valve leaflets and chordal-papillary apparatus, is often the most reliable or the only possible method correction of tricuspid regurgitation. In our opinion, it was thanks to the complete elimination of tricuspid regurgitation through TC prosthesis that the patient was able to be discharged from the hospital in sinus rhythm after 4.5 years of atrial fibrillation.

In conclusion of this message, I would like to note that patients who have undergone radical correction of TF are apparently subject to lifelong monitoring by a cardiologist, despite the good immediate result of the operation, with an emphasis on the timely detection of possible complications in the long-term postoperative period.

Literature

1. Lillihei C.W., Cohen M., Warden H.E., Read R.C., Aust J.B.,

Dewall R.A. et al. Direct vision intracardiac surgical correction of

the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects; report of first ten cases. Ann. Surg. 1955; 142 (3): 418-42.

7. Podzolkov V.P., Chiaureli M.R., Danilov T.Yu., Yurlov I.A., Sabirov B.N., Kagramanov I.I. et al. Causes and results of surgical correction of tricuspid valve insufficiency after radical correction of tetralogy of Fallot. Thoracic and cardiac vessels. hir. 2009; 6:23-9.

9. Podzolkov V.P., Chiaureli M.R., Danilov T.Yu., Yurlov I.A., Sabirov B.N., Kagramanov I.I. and others. Heart valve replacement in the long term after radical correction of tetralogy of Fallot. Thoracic and cardiac vessels. hir. 2010; 5: 18-26.

1. Lillihei C.W., Cohen M., Warden H.E., Read R.C., Aust J.B., Dewall R.A. et al. Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atre-sia defects; report of first ten cases. Ann. Surg. 1955; 142 (3): 418-42.

2. Payne W.S., Kirklin J.W. Late complication after plastic reconstruction of outflow tract in tetralogy of Fallot. Ann. Surg. 1961; 154: 53-7.

3. Murphy J.G., Gersh B.J., Mair D.D., Fuster V., McGoon M.D., Ilstrup D.M. et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N.Engl. J. Med. 1993; 329(9):593-9.

4. Oechslin E.N., Harrison D.A., Harris L, Downar E., Webb G.D., Siu S.S. et al. Reoperation in adults with repair of tetralogy of Fallot: indications and outcomes. J. Thorac. Cardiovasc. Surg. 1999; 118 (2): 245-51.

5. Monro J.L., Alexiou C., Salmon A.P., Keeton B.R. Reoperations and survival after primary repair of congenital heart defects in children. J. Thorac. Cardiovasc. Surg. 2003; 126 (2): 511-20.

6. Kobayashi J., Kawashima Y., Matsuda H., Nakano S., Miura T., Tokuan Y. et al. Prevalence and risk factors of tricuspid regurgitation after correction of tetralogy of Fallot. J. Thorac. Cardiovasc. Surg. 1991; 102 (4): 611-6.

7. PodzoLkov V.P., Chiaureli M.R., Danilov T.Yu., Yurlov I.A., Sabirov B.N., Kagramanov I.I. et al. The causes and results of surgical correction of tricuspid valve insufficiency after radical correction of tetralogy of Fallot. Grudnaya i serdechno-sosudistaya khirurgiya. 2009; 6: 23-9 (in Russian).

8. Ando M., Takahashi Y., Kikuchi T., Tatsuno K. Tetralogy of Fallot with subarterial ventricular septal defect. Ann. Thorac. Surg. 2003; 76 (4): 1059-65.

9. Podzolkov V.P., Chiaureli M.R., Danilov T.Yu., Yurlov I.A., Sabirov B.N., Kagramanov I.I. et al. Heart valve replacement late after radical correction of tetralogy of Fallot. Grudnaya i serdechno-sosud-istaya khirurgiya. 2010; 5: 18-26 (in Russian).

10. Hachiro Y., Takagi N., Koyanagi T., Abe T. Reoperation for tricuspid regurgitation after total correction of tetralogy of Fallot. Ann. Thorac. Cardiovasc. Surg. 2002; 8 (4): 199-203.

Diagnosis and treatment methods, prognosis for recovery.

Tetralogy of Fallot is a severe congenital heart defect with four (tetralogy) characteristic defects:

Strong displacement of the aorta to the right (normally the aorta departs from the left ventricle, with tetralogy of Fallot - completely or partially - from the right ventricle).
Maximum stenosis (narrowing) of the pulmonary artery trunk (normally, blood from the right ventricle passes through it into the lungs and is saturated with oxygen).
Absence of the interventricular septum.
Dilatation (increase in volume) of the right ventricle.

What happens during pathology? Due to defects:

venous and arterial blood mixes in the ventricles and enters the systemic circulation, insufficiently oxygenated;
increases oxygen starvation of tissues and organs, displacement of the aorta and narrowing of the pulmonary trunk (the stronger the stenosis, the less blood is saturated with oxygen in the lungs and the more remains in the ventricle, exacerbating congestion);
serious disturbances in the large (from the left ventricle to the aorta) and small (from the right ventricle to the pulmonary artery) circle quickly lead to the development of chronic heart failure.

As a result, the sick child develops characteristic cyanosis (blueness initially of the limbs, nasolabial triangle, and then all skin), shortness of breath, ischemia of the brain and the whole body.

Cyanosis in a child

Surgical correction of tetralogy of Fallot is performed by cardiac surgeons, and patients are monitored before and after surgery by the attending cardiologist.

Reasons for appearance

medications (hormonal, sedatives, sleeping pills, antibiotics, etc.);
infectious diseases (rubella, measles, scarlet fever);
harmful industrial and household chemical compounds (salts of heavy metals, pesticides and fertilizers);
toxic effects of alcohol, drugs and nicotine.

The risk of a defect increases in families where close relatives had children with intrauterine cardiac anomalies.

Symptoms of pathology

After the operation, the patient’s well-being improves, but physical activity is limited so that it does not provoke the development of shortness of breath and other symptoms of heart failure.

The main signs of the defect are caused by disturbances in the enrichment of blood with oxygen, which is why it is called “blue”.

Characteristic symptoms of tetralogy of Fallot:

Shortness of breath that appears and increases after any activity (crying, sucking)

Severe weakness (caused by the most basic actions)

Loss of consciousness (the last two symptoms are due to progressive cerebral ischemia)

Breathing and pulse suddenly increase (from 80 beats per minute).
Shortness of breath increases.
The child is worried.
Cyanosis noticeably intensifies to a purple hue.
Severe weakness appears.
The attack may result in loss of consciousness, convulsions, respiratory arrest, coma, stroke or sudden death.

Due to oxygen deficiency, children with congenital defects lag behind in development, are less able to master various skills (cannot hold their head up, etc.), and often get sick.

Diagnostics

Over time, patients develop characteristic external signs that can be used to make a primary diagnosis:

the most characteristic indicator is acrocyanosis (cyanosis of the peripheral parts - hands, feet, tips of the ears, fingers, nose, and then the whole body);
thickening of the fingertips in the form of “drumsticks” and deformation of the nails in the form of “hour glasses” (convex, round);
retardation in physical development, loss of body weight;
flattened chest (a chest hump appears less frequently).

When listening to the heart, a rough “buzzing” or “scraping” sound is detected.

Tetralogy of Fallot in children is confirmed using hardware methods:

Ultrasound, which is used to determine changes in the size of the chambers of the heart (dilatation of the right ventricle).
The ECG shows incomplete blockade of the conductive branches of the (right) bundle of His and hypertrophy (enlargement, thickening) of the right ventricular myocardium.
Using radiography, a characteristic pattern of the lungs (due to lack of blood supply, they appear translucent) and the heart (increase in size and shape in the form of a boot or shoe, with a raised top of the heart) is recorded.
Dopplerography allows you to determine the direction of blood flow and the diameter of blood vessels.

Treatment methods

It is impossible to completely cure the pathology:

in 30% of cases, disorders are combined with other congenital anomalies, which complicates prognosis and treatment;
in 65% of cases, hemodynamic (blood flow) disturbances are so pronounced that surgical treatment improves the patient’s condition for a while, prolonging the period and improving the quality of life, but the pathology gradually progresses, leading to the development of chronic heart failure.

The following treatment methods are used:

Drug therapy (emergency care for cyanotic attacks).
Palliative intervention (preparation for radical surgery, temporary elimination of critical hemodynamic disorders).
Radical correction (restoration of the interventricular septum, relocation of the aortic mouth, expansion of pulmonary artery stenosis, etc.).

Life expectancy and further prognosis depend entirely on how timely the surgery was performed.

Drug therapy

Drug therapy is used as emergency treatment for a cyanotic attack:

oxygen inhalations are used to restore oxygen saturation of tissues and blood;
sodium bicarbonate solution is administered to relieve acidosis (accumulation of metabolic products);
to facilitate the breathing process, use bronchial and antispasmodics (aminophylline);
to prevent shock due to gas exchange disorders, adhesion (clumping of red blood cells) and the formation of blood clots, a plasma substitute solution (reopolyglucin) is administered intravenously.

After the onset of seizures, the child’s prognosis worsens; surgical correction of the defect is needed in the near future.

Temporary elimination of hemodynamic disturbances

Temporary correction or palliative methods are used immediately after birth or at an early age, these include the creation of various anastomoses (connections, paths) between the vessels.

Full operation

Method of operation: radical correction of congenital defect of Fallot.

Goal: restore hemodynamics and gas exchange in tissues and organs, eliminate symptoms of heart failure and cerebral ischemia, improve the condition, quality of life and prognosis of the patient.

How it is performed: The vascular system is connected to a heart-lung machine; the heart does not work at the time of the operation; it is cooled with special solutions:

if necessary, preliminary palliative anastomoses are eliminated;
move the aortic mouth into the left ventricle;
apply a patch to the interventricular septum;
expand the pulmonary artery stenosis, cutting the fibrous ring;
The trunk of the pulmonary artery is expanded by applying a longitudinal flap.

Emergency intervention is necessary if:

Cyanotic attacks appear or become more frequent, ending in fainting, convulsions and loss of consciousness.
Signs of heart failure have increased (shortness of breath at rest).
The general health and condition of the child noticeably deteriorated.
There is a serious lag in physical and mental development.

Possible complications after surgery

The most common postoperative complications (in 20%) of cases can be:

thrombosis of artificial anastomoses;
attack of acute heart failure;
pulmonary hypertension (increased pressure in the pulmonary artery);
atrioventricular block (impaired conduction between the ventricles and atria);
aneurysm (protrusion) of the right ventricle;
various arrhythmias.

Prognosis for recovery

Tetralogy of Fallot is a common type of congenital heart defect.

As is known, blue and white congenital heart defects in children occur, Tetralogy of Fallot being one of the first. This is a congenital anomaly that requires medical supervision and planned treatment. With timely help, it is possible to bring your health condition back to normal.

Features of the disease

Heart pathology caused by a combination of four disorders:

The exit of venous blood through the natural channels of the right half is difficult due to one of the variants of stenosis:
- pulmonary trunk,
- valve,
- on the branches of the pulmonary artery,
- various combinations of the listed violations.
Deviation from the correct location of the aorta, which leads to a situation where blood from both ventricles enters it.
Pathological disorder in the structure of the septum between the ventricles.
Due to the obstructed exit of blood from the ventricle of the right side, it is susceptible to destructive changes.

The disease is a type of heart defect. Represents a congenital anomaly. Each of the four breakdowns in the structure of the heart has a wide variety of options, as the pathology manifested itself in a particular case.

The first three points are primary congenital disorders. The topic of the fourth paragraph appears as a consequence of the effect on the health of the heart of primary disorders.

A child, while in the womb, if he has this type of heart defect, does not experience any difficulties in connection with it. Discomfort appears after birth. Often these children have a bluish skin color.

Poor health does not allow him to be mobile. At an early age, the highest percentage of child mortality is due to this pathology. According to statistics, 25% of sick children, if surgical intervention is not undertaken, die before they are one year old. The remaining part may experience developmental delays both physically and mentally. By the second year of life, the duct in the artery may close.

If by this time the body has not been able to adapt to the situation and has not created collateral blood circulation (bypass paths), then life becomes at risk. Surgery is recommended.

There are cases that the body includes mechanisms that compensate for the disease and therefore survives to adulthood. This:

the muscles of the right ventricle adapt to the situation through hypertrophy,
a bypass path for blood flow is formed in the vessels - collateral circulation,
the lack of oxygen is compensated by erythrocytosis, this carries the risk of blood clots.

A video from a famous TV presenter will tell you in more detail about the features of such a defect as Tetralogy of Fallot:

Forms and classification

The defect manifests itself through three phases of the disease.

Celebrated at an early age: from birth to six months. There may be a condition where significant deviations from the norm are not noticed.
The appearance of attacks characterized as dyspnea-cyanotic. In this regard, there are complications with the brain, and critical situations with a fatal outcome are possible.
The body adapts to pathology, the threshold of the adult period.

The disease is classified according to four points. Expresses the congenital nature of the anomaly of the outflow tract from the right ventricle.

The anomaly is expressed in the underformation of the pulmonary cone of the truncus arteriosus. It turns out shortened and narrowed.

Hypertrophic

The violation is expressed by a deviation in the location of the septum to the left and anteriorly. It may be low. The proximal segment of the septum has hypertrophic changes. Narrowing in the area of the outlet of the right half.

Embryological

The septum is located low or there is a displacement to the left side and anteriorly. The maximum narrowing occurs at the dividing ring.

Multicomponent

Anatomically, the pathology is expressed in the fact that the septum is significantly elongated. It may be that the moderator strand has a high origin.

Peculiarities of blood movement associated with pressure differences in different areas divide tetralogy of Fallot according to clinical and anatomical characteristics into three forms:

acyanotic,
abnormal closure of the mouth of the pulmonary artery,
cyanotic form, varying degrees of narrowing of the artery mouth is observed.

Diagram-photo of Tetralogy of Fallot

Causes

The pathology develops in the first or second months of fetal development and is a consequence of defects in the structure of the heart.

taking medications by the expectant mother,
influence of harmful chemical agents,
the presence of unfavorable environmental factors,
if the pregnant woman has suffered from infectious diseases, especially in the early stages of gestation;
taking drugs, alcohol;
genetic predisposition.

Symptoms

One of the most common signs of this disease is a bluish discoloration of the skin. The intensity of the color depends on the extent to which the blood entering the arterial bed has the opportunity to be enriched with oxygen. Mixing of the blood of the two ventricles due to pathology of the wall or improper location of the pulmonary vessel makes the arterial blood depleted and causes bluish skin. With any even light physical activity, cyanosis intensifies.
Shortness of breath appears at an early age or at other stages of the disease. The slightest physical movements and actions increase shortness of breath. The patient feels a loss of strength.
In the position of squatting, some relief comes.
Dizziness and tachycardia are added to shortness of breath.
The pathology leads to a severe manifestation: dyspnea-cyanotic attacks. They usually first appear between two and five years of age. A crisis occurs with increased shortness of breath, bluish skin, convulsions and loss of consciousness are possible.
Children have weak immunity to infectious diseases.
Children often experience insufficient development both physically and mentally.
Adult patients with this pathology can develop pulmonary tuberculosis.

You can see what Tetralogy of Fallot looks like in the following video:

Diagnostics

The disease is primarily determined through anamnesis: analysis of visible deviations from the norm, linked to the individual characteristics of the patient:
- cyanosis,
- changing the shape of the last phalanges of the fingers, thickening them; nails take the shape of “watch glasses”;
- sometimes there is deformation of the chest,
- characteristic noises are determined by listening.
Using cardiac ultrasound, you can see the anatomical features characteristic of tetralogy of Fallot.
The ECG provides information based on the rhythmic pattern about the state of the myocardium and the presence of deviations from the axis.
Phonocardiography will provide complete information about rhythm disturbances and heart murmurs.
An MRI of the organ will show structural abnormalities in great detail.
Aortography will clarify information about whether there are any violations of the pulmonary artery, whether the body has made bypass paths for blood flow.
X-ray will show the shape of the heart, the pulmonary pattern will show how depleted it is.
Probing can determine whether there is communication between the ventricles and the level of pressure in them.

Read on to learn more about how Tetralogy of Fallot is treated in newborns.

Treatment

A defect is a set of defects in the anatomical structure. The main method of treatment is correction to varying degrees through surgery.

Scheme of the defect Tetralogy of Fallot

Therapeutic

If surgery is not planned in this period of time, in order to alleviate the condition during dyspnea-cyanotic attacks, therapeutic assistance is provided - with humidified air using the inhalation method.

Medication

If attacks occur, they resort to taking medications to improve their general condition. Intravenous administration is prescribed:

Surgical

There are two types of prompt assistance. The choice of intervention method depends on age and complexity of abnormal abnormalities.

For newborns and children of the first years of life, if their condition requires surgical assistance, then palliative operations are used. These operations enable the patient to wait out the necessary time for more radical intervention. A greater flow of blood into the small circle is achieved. This type of operation includes a large group of methods that provide various corrective interventions. They perform various types of bypass surgery and removal of obstacles using special equipment.
The actions prepare the patient for the second stage of surgery. They are performed on an open heart and solve the problem of correcting structural defects as much as possible using plastic surgery.

There are also some nuances regarding anesthesia for Tetralogy of Fallot. Due to the fact that this type of defect creates the preconditions for hypertension, anesthesiological tactics include the preparatory period before surgery. The specialist prescribes medications that can prevent uncontrolled increases in blood pressure during surgery. After this, observation should be continued in this direction.

Now let's find out what to do so as not to hear about the diagnosis of Tetralogy of Fallot.

Disease prevention

Taking good care of your health will eliminate a number of factors that initiate the abnormal development of Tetralogy of Fallot in the fetus.

avoid places with ionizing radiation,
do not come into contact with harmful chemicals,
do not drink alcohol,
When communicating with infected patients, take precautions
follow hygiene rules to protect yourself from possible infection with diseases;
no smoking,
plan your pregnancy, communicate the day before with geneticists and other specialists.

Complications

Pathology, if not carried out planned treatment, can lead to undesirable consequences:

Now let's find out about the prognosis after surgery for Tetralogy of Fallot.

Forecast

At an early age, surgical intervention has a fatal outcome of no more than five percent. This method allows the patient to live a full life.

Unpleasant symptoms go away: shortness of breath, cyanosis, the blood normalizes its composition. And without surgery, patients with this diagnosis survive to the age of forty, only one twentieth of the sick people.

We will talk further about how tetralogy of Fallot manifests itself during pregnancy.

Illness during pregnancy

If, during pregnancy, a woman suffering from tetralogy of Fallot has not previously made adjustments through surgical intervention, then her condition worsens as pregnancy progresses. Experts allow palliative surgery during pregnancy, but this measure results in only thirty percent of live births, most of them premature newborns. It is more correct for such patients to correct the condition in a timely manner and not leave the situation during pregnancy.

During the perinatal life of the fetus, ultrasound can detect structural defects that make up the tetralogy of Fallot. During this period, violations do not pose much discomfort for the child.

Specialists can predict what kind of help he will need at birth and when it is best to make a correction. After birth, the child acquires a bluish skin color.

This may happen later. In mild cases, the skin has a bluish tint or no cyanosis is present at all.

Child vaccinations

The possibility of vaccinations for Tetralogy of Fallot also raises questions. The decision on whether to vaccinate a child is made by specialists based on information about the patient’s condition and personal characteristics.

Is there any disability for Tetralogy of Fallot?

Based on the examination materials of the patient, a medical and social examination is carried out. As a result, the severity of the patient’s condition is determined and a decision is made to receive disability.

The following video will tell you more about Tetralogy of Fallot, as well as other heart defects in children:

Tetralogy of Fallot

Tetralogy of Fallot is one of those defects in which cyanosis can appear gradually. Sometimes it is barely noticeable, and only indicators of hemoglobin and red blood cells can indicate a constant undersaturation of arterial blood with oxygen (there is even the term “pale tetralogy”), but this does not change the anatomical essence of the defect itself.

By definition (“tetrad” means “four”) with this defect there are four violations of the normal structure of the heart.

The first of the four components of a tetrad is a large ventricular septal defect. Unlike the defects mentioned above, with tetralogy it is not just a hole in the septum, but the absence of a section of the septum between the ventricles. It simply does not exist, and thus the communication between the ventricles is unimpeded.

The second component is the position of the aortic mouth. It is shifted forward and to the right relative to the norm, and appears to be sitting “on top” of the defect. The word “on horseback” fits very accurately here. Imagine a man riding a horse - one leg to the right, the other to the left of the croup, and the torso in the center and above it. So the aorta turns out to be sitting in the saddle above the formed hole and above both ventricles, and does not extend only from the left, as in a normal heart. This is the so-called "dextroposition"(i.e., displacement to the right) of the aorta, or its partial origin from the right ventricle, is the second of four components of the tetralogy of Fallot.

The third component is a muscular, intraventricular, narrowing of the outflow tract of the right ventricle, which opens at the mouth of the pulmonary artery. The trunk and branches of this artery are also often much narrower than normal.

And finally, fourth, a significant thickening of all the muscles of the right ventricle, its entire wall, several times greater than its normal thickness.

What happens in the heart, to which nature has given such a difficult task? How to provide oxygen to the body of a newborn child? After all, you have to deal with this!

Let's see what happens to the blood flow in such a situation. Venous blood from the vena cava, i.e. from the whole body, passes into the right atrium. It enters the right ventricle through the tricuspid valve. And here there are two ways: one - through a wide-open defect into the aorta and into the systemic circulation, and the other - into the pulmonary artery narrowed at the beginning, where the resistance to blood flow is much greater.

It is clear that in a small circle, i.e. a smaller part of the venous blood will pass through the lungs, and most of it will go back to the aorta and mix with the arterial blood. This admixture of venous, unoxidized blood creates general undersaturation and causes cyanosis. Its degree will depend on what part of the blood in the large circle is undersaturated, i.e. venous, and to what extent those “protection” mechanisms were activated - an increase in the number of red blood cells, which we talked about above. Thickening of the muscular wall of the right ventricle is only its response to a significantly increased load compared to the norm.

Immediately after birth, the child looks normal, but after a few days you can notice his anxiety, shortness of breath at the slightest exertion, the main of which is now sucking.

The cyanosis may be completely unnoticeable or may only be detected when crying. The child is gaining weight normally. However, sometimes he suddenly begins to choke, rolls his eyes, and it is not entirely clear whether he is conscious at such a moment or not. The condition lasts from a few seconds to several minutes, and goes away as suddenly as it began. This - dyspnea-cyanotic attack, dangerous even if it is short-lived, because its outcome is unpredictable. Of course, even at the slightest suspicion of such a condition, you should immediately see a doctor.

With tetralogy of Fallot, attacks, as part of the clinical picture, can occur even in the absence of pronounced cyanosis. In general, cyanosis with this defect is revealed, as a rule, in the second half of life, and sometimes later. There may also be no attacks - they are associated with the degree of narrowing of the outflow tract of the right ventricle, which, of course, is different for all patients.

Children with tetralogy of Fallot can live for several years, but their condition inevitably worsens: the cyanosis becomes very pronounced, children look exhausted, and are sharply behind their peers in development. The most comfortable position for them is squatting, with your knees tucked under you. They find it difficult to move, play, lead and enjoy a normal life. They are seriously ill. The diagnosis will be made at the first competent cardiological examination, after which the question of surgical care will immediately arise. The degree of urgency depends on the specific situation, but the operation cannot be delayed: the consequences of cyanosis and seizures can become irreversible if they lead to neurological disorders and, especially, damage to the central nervous system. In a situation where cyanosis is little or not expressed at all (the so-called “pale tetrad”), the danger is less, but it still exists.

What are the surgical treatment methods for tetralogy of Fallot?

There are two ways. The first is to close the ventricular septal defect and remove the obstruction to blood flow in the right ventricle and pulmonary artery. This - radical correction of the defect. It is clear that it is performed on an open heart under artificial circulation. Today it can be done at any age, however, not always and not everywhere. There is always a risk with open heart surgery. But the variants of the anatomy of the tetralogy of Fallot, although they have one common name, differ from each other, sometimes significantly, and the risk is sometimes too great to perform such a large reconstructive operation “in one go.” Fortunately, there is another way - to first perform a palliative, auxiliary operation.

Anastomosis between the systemic and pulmonary circles

During this operation, an anastomosis is created - an artificial shunt, i.e. communication between the circulation, which actually represents a new arterial duct (instead of the one that closed naturally). When one of the vessels of the systemic circulation is connected to the pulmonary artery, the “blue”, “semi-venous” blood, undersaturated with oxygen, will pass through the lungs, and the amount of oxygen in it will increase significantly. This operation is closed, does not require artificial circulation, and is very well developed, even for the smallest children.

Today it is performed by sewing a short synthetic tube between the beginning of the subclavian artery and the pulmonary artery. The diameter of the tube is 3–5 mm, and the length is 2–3 cm.

This operation, which has saved the lives of thousands of children, is used not only for tetralogy of Fallot, but also for other congenital defects with cyanosis, the cause of which is a narrowing of the outflow tract of the right ventricle and insufficient blood flow into the pulmonary bed, i.e. into the pulmonary circulation. In the future, regarding other defects, we will not dwell on the principle of this operation in such detail, but will say “anastomosis between the systemic and pulmonary circles,” implying that you already know what we are talking about.

The results of the operation are amazing: the child turns pink right on the operating table, as if he took a deep breath for the first time in his life. Signs of cyanosis disappear immediately, as do dyspnea-cyanotic attacks, and the child’s immediate life seems cloudless. But it only seems so. The main flaw remains. Moreover, we added another one to him, although we thereby helped him survive.

Patients who have had an anastomosis can live 5-10 years or more. But even if there are no complications, over time the function of the anastomosis deteriorates and becomes insufficient: after all, the child is growing, the defect is not corrected, and the size of the anastomosis is constant. And although the child feels well, the thought that he has not been completely cured will not leave you alone. We advise you to prepare yourself for subsequent correction of the defect within 6–12 months after the first operation.

Radical correction consists of closing the defect with a patch (after which the aorta will arise only from the left ventricle, as it should be), removing the narrowed area in the outflow tract of the right ventricle and dilating the pulmonary artery with a patch when necessary. If an anastomosis was previously performed, it is simply bandaged.

Which treatment method will be chosen depends on the specific situation - on the anatomy of the defect and on the condition of the child. Therefore, here we can limit ourselves to advice only.

The main thing is to try to calm down. You see, it is necessary and possible to treat: there are reliable, time-tested methods of treatment. When should they be used? If a child is unwell, he is blue, he is developmentally delayed, he has seizures, which we wrote about above - there is simply no time for thinking. He needs to undergo palliative surgery, i.e. perform an anastomosis. And urgently to avoid possible complications. In addition, this operation will prepare the child and his heart for repeated, radical correction.

With a “pale” course of tetralogy of Fallot without attacks and without pronounced cyanosis and in the presence of conditions, it is possible to immediately make a radical correction without resorting to anastomosis. But it is advisable to perform such an operation in clinics where there is not only sufficient technical equipment, but also significant experience. There are more and more such clinics in our country.

The first serious attempts at surgical treatment of tetralogy of Fallot were made more than half a century ago, and it would not be an exaggeration to say that this is where all surgery for cyanotic congenital heart defects began. Over such a long period of time, treatment methods for tetralogy of Fallot have been developed in detail, and the results, even long-term (i.e., over a period of time) are excellent. And the accumulated experience shows that today this operation - in a one- or two-stage version - is quite safe and rewarding.

Patients who undergo treatment in early childhood become practically healthy, full-fledged members of society. They can study, work, and women can give birth and raise children, and many forget about the illness they suffered in childhood. As for the moral injuries associated with the entire process of surgical treatment, the child forgets about them, and it is very important that parents do not remind or instill in him that he was once very sick. This does not mean that there is no need to see doctors; after all, there was an operation, and it was complicated. Monitoring is necessary, since in the long term (after several years) heart rhythm disturbances or signs of pulmonary valve insufficiency may appear. These possible consequences of the defect (they can hardly even be called complications) are correctable, and the time is not far when the most common of them will be eliminated using closed X-ray surgical methods. The main condition for successful treatment of these phenomena is their timely recognition.

Let's summarize. Tetralogy of Fallot is a fairly common, severe, but completely curable heart defect. The sooner it is corrected surgically, the better results can be expected in the future. A child, and later a teenager and an adult, who was operated on in childhood for tetralogy of Fallot, should be periodically observed by specialists and lead a healthy lifestyle.

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It is now recognized that the most important problem affecting long-term outcomes is pulmonary regurgitation. It is observed quite often and is well tolerated by most patients for decades. However, in some it leads to progression of RV dilatation, HF, tricuspid regurgitation and supraventricular arrhythmia. In such cases, pulmonary valve replacement is necessary, which results in significant clinical improvement in most patients.

However, it appears that RV function does not improve in all patients, and therefore a key ongoing research question is to determine the optimal timing for pulmonary valve implantation to preserve cardiac function. Evidence is accumulating regarding the benefits of early intervention, but the benefit of pulmonary valve replacement must be weighed against the risk of reoperation. The management of these patients may be impacted in the near future by a new method of percutaneous pulmonary valve implantation (Fig. 1). Patients with tetralogy of Fallot often have stenosis of the peripheral branches of the pulmonary artery; in this case, stenting may be performed during pulmonary valve replacement.

Rice. 1. A - tissue valve inserted into the stent, used for percutaneous implantation of the pulmonary valve.

B - balloon-covered transport catheter designed for percutaneous pulmonary valve implantation.

B - 3D MRI reconstruction of the outflow tract of the pancreas and the branches of the pulmonary arteries is used to select the ideal location for installing the valve inserted into the stent.

D - lateral angiogram performed immediately after valve installation, confirming successful removal of obstruction and valve functionality.

Sudden death occurs infrequently, occurring in approximately 0.5-0.6% of patients over 30 years of age, accounting for approximately one third of late deaths. Brief ventricular arrhythmia is common but is not a risk indicator, so there is no need to routinely prescribe antiarrhythmic therapy to a patient in the absence of symptoms. Typically, the arrhythmogenic site is in the pancreatic outflow tract at the site of infundibulectomy or at the site of VSD closure. A risk factor is considered to be a combination of pulmonary regurgitation, RV dilatation and late ventricular arrhythmia with a QRS complex duration on the ECG of more than 180 ms (Fig. 2).

The best treatment for ventricular arrhythmia is often repeat surgery if the injury is resectable. Atrial tachyarrhythmia is a significant factor in the formation of late morbidity and even mortality. Supraventricular arrhythmia (AF or AF) is relatively common and occurs in 1/3 of patients undergoing surgery. This may be one of the first signs of hemodynamic decompensation, so a full cardiac evaluation is warranted in such cases. Improvement in hemodynamics during reoperation in most patients leads to the disappearance of arrhythmia.

Rice. 2. A - when conducting a 12-lead ECG in an adult patient after surgical correction of tetralogy of Fallot, a pattern of right bundle branch block and a prolongation of the QRS complex >180 ms were detected.

B - comparison of the duration of the QRS complex in patients with restrictive and non-restrictive mechanisms after surgical correction of tetralogy of Fallot. A QRS complex duration of >180 ms is a risk factor for sudden ventricular tachycardia.

Source (with permission): Gatzoulis M.A., Till J.A., Somerville J. et al. Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death // Circulation. - 1995. - Vol. 92. - P. 231-237.

If palpitations or syncope develop, it is necessary to immediately pay attention to this and conduct a full hemodynamic and electrophysiological examination. If, according to non-invasive monitoring data, no long-term arrhythmia is noted, then in most centers they stop at electrophysiological observation. When treating patients with prolonged ventricular tachycardia (rare), it is necessary to decide on the need to correct existing hemodynamic disturbances, the feasibility of radiofrequency destruction and ICD.

In women who have undergone successful surgical treatment and have a normal functional hemodynamic state, pregnancy is tolerated well even in the presence of pulmonary regurgitation. After successful correction of tetralogy of Fallot in patients without arrhythmia, there is no need to limit physical activity.

The appearance of late aortic dilatation and aortic regurgitation is increasingly noted. There are also signs of median degeneration of the aortic wall, as in Marfan syndrome. If the diameter of the aortic root increases beyond 55 mm, “replacement” of the aortic root may be indicated.

Supporting patients with tetralogy of Fallot and pulmonary atresia is one of the most difficult problems associated with congenital heart diseases. Blood supply to the lungs is highly variable and determines clinical presentation, natural history, management, and outcome. For the survival of newborns with pulmonary atresia and duct-dependent blood supply to the lungs, prostaglandin infusion is necessary, followed by urgent surgical intervention. Other patients may experience increased blood supply to the lungs as a result of the function of multiple large aortopulmonary collateral arteries, which will manifest as HF. Patients of the third group with a “balanced” blood supply to the lungs can feel well for many years without any treatment. They develop obstructive vascular disease in an unprotected segment of the lungs supplied by a vessel arising from the aorta.

Management strategies vary widely among institutions and have evolved very rapidly over the past few years. Therefore, it is difficult to make any generalizations. In cases where the only source of pulmonary blood flow adequately supplies blood to the pulmonary arteries going to all bronchopulmonary segments, a complete correction can be performed in one step. The timing will depend on the need to form an anastomosis between the RV and PA to ensure antegrade blood flow into the PA (often occurs). If the central pulmonary arteries are tiny or absent, many consider these patients to be beyond repair, and in such cases the goal of treatment is to optimize the pulmonary circulation through surgical techniques or interventional catheterization, depending on the patient's condition. Other patients have a small central pulmonary artery supplying one or another part of the pulmonary segments; the rest are supplied by an almost indefinite number of large aortopulmonary collateral arteries.

The prospects for correction depend on the possibility of creating a single source of pulmonary blood flow (“unifocalization”), which can already be connected to the pancreas simultaneously with the closure of the VSD. Achieving this result may require multi-stage palliative surgery, and in some cases this may not be possible. The long-term results of such operations are just beginning to emerge. Despite cautious optimism, patients may not undergo surgery and may be left with right ventricular hypertension, which is likely to limit length and quality of life. The anastomosis between the RV and PA will "wear out" over time and require further surgical correction or percutaneous pulmonary valve implantation.

John E. Deanfield, Robert Yates, Folkert J. Meijboom and Barbara J.M. Mulder

Congenital heart defects in children and adults