This disease was identified and described in 1888 by the French pathologist ELFallo. Over the last 100 years, doctors have achieved good results in the early detection and eradication of this disease.
Description of the disease, its prevalence
Tetrada Fallot is a congenital heart disease that is often diagnosed in newborns. In 3% of babies, this disease occurs, which is one-fifth of all congenital heart defects detected.
30% of all miscarriages and undeveloped pregnancies are associated with the presence of Fallot tetralogy in the fetus, and 7% of babies with this pathology are born dead.
The Fallot tetralogy is diagnosed in the case of a newborn having only four anatomical defects in the structure of the cardiovascular system:
Stenosis or narrowing of the pulmonary artery leaving the right ventricle. Its immediate purpose is to carry the venous blood to the lungs. If there is a narrowing of the pulmonary artery, blood flows with effort from the ventricle of the heart into the artery. This leads to a significant increase in the load on the right heart. With increasing age, the stenosis increases - that is, this defect proceeds progressively.
Defect in the form of an opening in the interventricular septum. Normally, the ventricles of the heart are separated by a dull partition that allows them to maintain a different pressure in them. In the Fallot tetralogy, there is a lumen in the interventricular septum and therefore the pressure in both ventricles is balanced. The right ventricle simultaneously pumps blood not only into the pulmonary artery but also into the aorta.
Extrastration or displacement of the aorta. Normally, the aorta is on the left side of the heart. With Fallot tetralogy it is shifted to the right and is located directly above the hole in the interventricular septum.
Increase of the right ventricle of the heart. It develops again, due to increased stress on the right parts of the heart due to narrowing of the artery and displacement of the aorta.
In 20-40% of cases with Fallot tetralogy, concomitant cardiac malformations occur
Causes and risk factors
Anatomical structures of the heart in the fetus formed in the first three months of intrauterine development.
At this time, harmful external effects on the pregnant woman's body can have a fatal effect on the development of congenital heart defects.
Risk factors at this time are:
- Viral diseases that occur in acute form;
- the use of certain medicines, psychoactive and psychotropic substances (including tobacco and alcohol) in pregnant women;
- Poisoning with heavy metals;
- radioactive radiation;
- The age of the future mother is more than 40 years.
Types and stages of the disease
Depending on the anatomical features of the defect, different types of Fallot tetralogy are distinguished:
- Embryological - The maximum narrowing of the artery is at the level of the limiting muscle ring. A stenosis can be eliminated by economic resection of the arterial cone.
- Hypertrophic - The maximum narrowing of the artery is at the level of the muscle ring and the right ventricle. The stenosis can be removed with the help of a massive resection of the arterial cone.
- Tubular - narrowed and shortened the entire arterial cone. Patients with such a defect should not undergo radical surgical correction of pulmonary artery stenosis. Palliative plastic is preferred for her, which helps to prevent the aggravation of the disease.
- Multicomponent artery stenosis is due to a number of anatomical changes whose position and characteristics determine the successful outcome of surgical correction of stenosis.
- Depending on the characteristics of the clinical manifestations of the virus, the following forms are distinguished:
- cyanotic or classic -with pronounced cyanosis of the skin and mucous membranes;
- Acyanotic or "pale" form -more common in children of the first 3 years of life as a result of partial compensation of the defect.
The severity of the river distinguishes such forms of the disease:
- Heavy. Dyspnoea and cyanosis appear almost from birth at crying, feeding.
- Classic. The disease begins at the age of 6-12 months. Clinical manifestations are closely correlated with increased physical activity.
- Paroxysmal. The child suffers from severe dyspnea-cyano attacks.
- Lightweight shape with the late onset of cyanosis and dyspnea - at 6-10 years.
In its course, the disease goes through three successive stages:
- Relative well-being. Mostly this phase lasts from birth to 5-6 months. There are no expressed symptoms of the disease due to partial compensation of the defect due to the characteristics of the neonatal heart structure.
- Cyanotic phase. The hardest time in a baby's life with a tailed fallot lasting 2-3 years. In this case, all clinical manifestations of the disease are pronounced: shortness of breath, cyanosis, suffocation. At this age, the greatest number of fatal consequences of the disease.
- Transition phase or the phase of the formation of compensation mechanisms. The disease persists, but the child adapts to his illness and knows how to prevent or relieve the onset of the disease.
Dangers and complications
The diagnosis "Fallot tetralogy" refers to the category of severe cardiac malformations with high mortality. If the disease is unfavorable, the average life expectancy of the patient is 15 years old.
Such complications of discomfort are not uncommon:
- Thrombosis of the blood vessels of the brain or the lungs due to increased blood viscosity;
- brain abscess.
- acute or congestive heart failure.
- bacterial endocarditis.
- Delay psychomotor development.
Depending on the degree of narrowing of the artery and the size of the opening in the interventricular septum, the age of onset of the first disease symptoms and the degree of their progression vary. The main clinical signs of the Fallot tetralogy are:
- Cyanosis. Most often occurs in children under the age of one year, first on the lips, then on the mucous membranes, face, arms, legs and trunk. Progress as the child's physical activity increases.
- Thickened fingers in the form of "drumsticks" and convex nails in the form of "watch glasses" have already been trained for 1-2 years.
- Shortness of breath in the form of deep arrhythmic breathing. The frequency of inhalation and exhalation does not increase. Dyspnoea increases with the least physical effort.
- Fast fatigue.
- heart hump - bump on the chest in the heart.
- Delaying motor development due to forced limitations of motor activity.
- Noises in the heart.
- Characteristic position of the body Sick child - squatting or lying with legs bent in the stomach. In this position the baby feels better and unconsciously takes it as often as possible.
- Fainting to the point of hypoxemic coma.
- "Blue" seizures of dyspnoea and cyanosis in the case of severe paroxysmal disease, when small children (1-2 years old) suddenly turn blue, begin to suffocate, become restive, and then they may lose consciousness or even go into a coma. After the attack the baby is lazy and sleepy. Often as a result of such aggravation, the baby may even die.
diagnosis
The disease is diagnosed on the basis of a medical history, after clinical examination and auscultation of the breast taking into account the results instrumental research methods:
- Blood test
- radiography
- Electrocardiography
- From a diagnostic point of view the most valuable ultrasound of the heart with Doppler
- Invasive methods of angiography and cardiac catheterization in neonates are rare, but with unsatisfactory results using other diagnostic methods
- Differential Diagnosis Fallot tetralogy is performed with similar diseases on clinical displays
Methods of treatment
The surgical treatment is shown. Optimal age for surgery up to 3-5 years. But before this age it is necessary to grow up, having passed a stage of the expressed cyanosis and a dyspnoea or short wind after the child in 1-2 years.
Quite often, children with tetralogy fallo die precisely during severe dyspnoea-cyanootic attacks at an early age when they are not provided with competent medical help and support:
- Prevention and treatment of comorbidities (anemia, rickets, infectious diseases).
- Prevention of dehydration.
- Calming therapy.
- Treatment with adrenoblockers;
- Treatment with antihypoxants and neuroprotectors.
- Oxygen therapy.
- Maintenance therapy with vitamins and minerals.
- Depending on the anatomical features of the existing defect, the surgical procedure is preferred.
Palliative surgery practiced with the impossibility to completely eliminate heart disease. It is necessary to optimize the quality of life of the patient. Most often, an aorto-pulmonary anastomosis is used - that is, the pulmonary artery is connected to the subclavian via synthetic implants. Sometimes palliative surgery is just the first stage of surgery in a child under 1 year old - it allows the baby to live for a few more years before undergoing a radical correction of the blemish.
Radical correction eliminates the defect of the interventricular septum and enlargement of the pulmonary artery. This is an open heart surgery, after which the child can lead a practically normal life after a while. It is important to note that this operation is recommended for children younger than 3-5 years.
If the Fallot tetralogy was not operated on, then in the first year of life, every fourth patient dies with this vice, and half of the children do not live up to 5 years. With timely surgery, the mortality of small patients is reduced to 5%.
The only measure to prevent a sad outcome of the disease is timely access to a doctor when the first signs are found illness, careful observation of the child and attentive medical supervision.
Tetrada Fallot - This is not a disease where you can experiment with alternative therapies and hope for a miracle. After all, the miracle of the life of a small patient with this disease lies only in the hands of a cardiac surgeon.
The tetrad of Fallot
The tetrad of Fallot - concurrent congenital adenocarcinoma, characterized by stricture of the excretory organ tract of the right ventricle, ventricular septal defect, aortic dextroposition and right ventricular hypertrophy. Fallot clinically manifested early cyanosis, developmental delay, respiratory distress and odyshechno-cyanotic attacks, dizziness and fainting. Instrumental diagnosis of tetralogy Logie involves the conduction of phonocardiography (PCG), electrocardiography (ECG), cardiac ultrasound, chest X-ray, ventriculography cardiac catheterization cavities. Operative treatment of the Fallot tetralogy may be palliative (overlapping intersystem anastomoses) and radical (complete surgical correction of the defect).
The tetrad of Fallot
Fallot tetralogy - a complex congenital heart disease, "blue" type, which is based on morphological four features: obstruction of the right ventricular outflow department, extensive ventricular septal defect (VSD), right ventricular hypertrophy and aortic displacement. In cardiology Fallot tetralogy occurs in 7-10% of cases of all congenital heart disease, and is cyanotic type for half of all malformations. Detailed anatomical feature of vice as an independent nosological form, was first given by the French physician, pathologist ELA Fallot given in 1888, after which he later called.
According to the structure of the defect Fallot's tetralogy on the next fallot triad (stenotic holes pulmonary artery, atrial septal defect and right ventricular hypertrophy) and pentad tetralogy (Fallot's tetralogy and DMPP). pravoraspolozhennoy aortic arch abnormalities of the coronary arteries, stenosis of the pulmonary artery branches, persistent ductus arteriosus, complete form of open atrioventricular channel, additional left superior vena cava, partial abnormal pulmonary vein drainage: Fallot tetralogy combined with other abnormalities of the heart and blood vessels.
Causes of the Fallot tetralogy
The Fallot tetralogy is formed due to a disruption of the process of cardiogenesis for 2-8 weeks. embryonic development. The cause of the defect pregnant women can lead to infantile infectious diseases (measles, scarlet fever, rubella); Taking medicines (hypnotics, sedatives, hormones, etc.), drugs or alcohol; Exposure to harmful factors of production. The founding of the UPU tracks the influence of inheritance.
Fallot tetralogy is commonly found in children with the syndrome of Cornelia de Lange (Amsterdam dwarfism), including mental retardation and more malformations ("clown face", choanal atresia, ear deformation, gothic palate, strabismus, myopia, astigmatism, optic atrophy, hypertrichosis , Deformation of the sternum and spine, syndactyly stop, to the number of fingers, malformations of internal organs and the like. D is decreased.).
The triggering mechanism of the Fallot tetrad is the wrong rotation (counterclockwise) of the arterial cone that causes the aortic valve to move to the right of the relative lung cone. In this case, the aorta is above the interventricular septum ("aortic rider"). The wrong position of the aorta determines the displacement of the pulmonary trunk, which is slightly elongated and narrowed. The rotation of the arterial cone prevents the connection of the septum with the interventricular septum, which causes the formation of VSD and the subsequent expansion of the right ventricle.
Classification of the Fallot tetralogy
Given the nature of the obstruction of the right ventricular outflow tract, the anatomical variants of the Fallot tetralogy are represented by four types: embryological, hypertrophic, tubular, and multicomponent.
I do the tetralogy of Fallot - embryologically. Obstruction is caused by the displacement of the cone septum forward and to the left or (and) its low position. The zone of maximum stenosis corresponds to the height of the limiting muscle ring. The pulmonary artery pulmonary ring is virtually unchanged or moderately hypoplastic.
II Type of Fallot tetralogy Hypertrophic At the center of the obstruction is the displacement of the cone septum anteriorly and to the left or (and) its deep position, as well as marked hypertrophic changes in the proximal segment. The zone of maximum stenosis corresponds to the level of the opening of the exit portion of the right ventricle and the limiting muscle ring.
III Type Tetrady Fallot - tubular. The obstruction is caused by unequal separation of the common artery trunk, causing the lung cone to be severely hypoplastic, narrowed and shortened. With this type of tetralogy, Fallot may have fibrotic ring hypoplasia or valve stenosis of the pulmonary trunk.
IV Type of Fallot tetralogy - multi-component. The cause of the obstruction is a significant lengthening of the conical septum or a strong detachment of the marginal-marginal trabeculae of the moderator strand.
In connection with the peculiarities of hemodynamics, three clinical-anatomical forms of the Fallot tetralogy are distinguished:
1) with atresia of the pulmonary artery mouth
2) cyanotic form with oral narrowing of various degrees
3) acyanotic form
Features of hemodynamics with Fallot tetralogy
The degree of hemodynamic disturbances in the Fallot tetralogy is determined by the severity of the obstruction of the right ventricular exit portion and the presence of a defect in the interventricular septum.
The presence of significant stenosis of the pulmonary artery and septal defect of large size determines the vast supply of blood from both ventricles into the aorta and less - into the pulmonary artery, which is accompanied by arterial hypoxemia. In view of the large defect of the septum, the pressure in both ventricles becomes equal. In the extreme form of the Fallot tetralogy, which is associated with the atresia of the pulmonary artery, the blood from the aorta penetrates through the open arterial duct or through the collaterals into the small circle of the bloodstream.
With moderate obstruction, the total peripheral resistance is higher than the resistance of the stenosed exit tract, resulting in a left-right blood flow leading to the emergence of the acyanotic (pale) form of the Fallot tetralogy. However, as the stenosis progresses, a cross-shaped, veno-arterial (right-left) blood output initially results, meaning the transformation of the defect from the "white" shape to the "blue" shape.
Symptoms of the Fallot tetralogy
Depending on the time of onset of cyanosis are five clinical forms and, accordingly, the same number of periods of manifestation of the Fallot tetralogy: early cyanotic form (appearance of cyanosis in the first months or first year of life), classic (the occurrence of cyanosis at the second or third year of life), severe (drives with odyshechno-cyanotic consequences), tardive cyanotic (appearance of cyanosis on 6-10 years) and acyanotic (pale) form.
In severe forms of tetralogy the fallo-cyanosis of the lips and skin appears 3-4 months and is stably expressed by 1 year. Cyanosis increases with feeding, crying, exertion, emotional stress, physical activity. Any physical activity (walking, running, moving game) is accompanied by an increase in dyspnea, the appearance of weakness, the development of tachycardia, dizziness. The characteristic position of patients with the Fallot tetralogy after a strain is squat.
Extremely severe manifestation of the disease of the Fallot tetralogy are odyshechno-cyanotic episodes, which usually appear between the ages of 2-5 years. The attack develops suddenly accompanied by the child's anxiety, increased cyanosis and shortness of breath, palpitations, weakness, loss of consciousness. Perhaps the development of sleep apnea, hypoxic coma convulsions with subsequent hemiparesis phenomena. Odyshechno-cyanotic development results due to a sharp spasm of the right ventricular infundibulyarnogo department, resulting in a total volume of venous blood passing through a defect in the interventricular septum into the aorta and increased central nervous system (CNS) hypoxia.
Children with Phallo therapy may be left behind in the physical (hypotrophy of II-III st.) And motor development; They are often ill with repeated ARVI (acute respiratory viral infections), chronic tonsillitis, sinusitis, recurrent pneumonia. In adult patients with the Fallot tetralogy it is possible to fix pulmonary tuberculosis.
Diagnosis of the Fallot tetralogy
An objective examination of patients with Fallot tetralogy respects paleness or blueness of the skin, thickening of the finger phalanges ("clubs" and "hourglass"), compulsion posture, weakness; less common - deformation of the breast (heart pump). Percutaneously, there is an insignificant extension of the boundaries of the heart in both directions. Typical features of auscultatory fallot tetralogy are gross systolic in II-III intercostal space to the left of the sternum, the weakening II tone of the pulmonary artery and others. Fully auscultatory pattern defect is fixed via phonocardiography.
Radiographs of thoracic organs show moderate cardiomegaly, a typical heart shape in the form of a shoe, depletion of the lung pattern. The ECG pattern is characterized by a significant deviation from EOS to the right, hypertrophic changes in the right ventricular myocardium, incomplete blockade of the right leg.
With the help of ultrasound of the heart all anatomical components of the Fallot tetrad are directly determined: the degree of pulmonary stenosis, the size of the aortic shift, the size of the BCV (Biphasic cuirass ventilation) and the severity of right ventricular hypertrophy.
The probing of the heart cavities makes it possible to detect a high pressure in the right ventricle, a decrease in the saturation of the arterial blood with oxygen, the passage of the catheter from the right ventricle into the aorta. When carrying out an aortography and pulmonary arteriography there is a presence of collateral blood flow, OAP, pathology of a pulmonary artery. If necessary, left ventricular, selective coronary angiography, multislice CT (MSCT) and magnetic resonance imaging (MRI) of the heart are performed.
The differential diagnosis of the Fallot tetralogy is carried out with the transposition of the main vessels, a double exit of the aorta and the pulmonary artery from the right ventricle, a single-chamber heart, a two-chamber heart.
Treatment of the Fallot tetralogy
All patients with Fallot Tetrad undergo surgery. Drug therapy is indicated odyshechno-cyanotic consequences for development: humidified oxygen inhalation, intravenous reopoliglyukina, sodium bicarbonate, glucose, aminophylline. In case of ineffective drug therapy, immediate use of the aorto-pulmonary anastomosis is necessary.
The method of operative correction of Fallot's tetralogy depends on weight of a course of a defect, its anatomical-hemodynamic option, age of the patient. Newborns and infants with a severe form of the Fallot tetralogy require palliative surgery in the first stage, which reduces the risk of complications in the subsequent radical correction of the defect.
To a palliative (shunt) types of operations in Fallot tetralogy includes overlay subclavian-lung anastomosis Bleloka-Taussig, vnutriperikardialnoe anastomosis of the ascending aorta and right pulmonary artery superimposed central aorto-pulmonary anastomosis using the synthetic or biological prosthesis, anastomosis between the descending Aorta and the left pulmonary artery and others. To reduce hypoxemia applied infundibuloplastiki open surgery and balloon valvuloplasty.
Radical correction of the Fallot tetralogy VSD (Ventricular septal defect) provides for elimination of plastics and obstruction of the right ventricular outflow department. Usually it is done at the age of six months to 3 years. Specific complications performed surgery, when Fallot tetralogy, may be anastomotic thrombosis, congestive heart failure, pulmonary hypertension, right ventricular aneurysm, AV (Atrioventricular) block, arrhythmias, infective endocarditis.
Prediction tetralogy of Fallot
The natural course of the defect largely depends on the degree of pulmonary stenosis. A quarter of children with a severe form of tetralogy die in the first year of life, half of them - at the time of the newborn. Without surgery, life expectancy is 12 years, less than 5% of patients survive until the 40th anniversary. The cause of death of patients with Fallot tetralogy is often a thrombosis of the vessels of the brain (ischemic stroke) or brain abscess.
The long-term results of the radical correction of the Fallot tetralogy are good: patients are able to work and socially active, they tolerate physical stress satisfactorily. However, since a radical surgery is performed at a later age, the long-term results are even worse. All patients with the Fallot tetralogy need the supervision of a cardiologist and a cardiac surgeon, antibiotic prophylaxis of endocarditis prior to performing any dental or surgical procedures that are potentially dangerous to the development of bacteremia.
Tetrada Fallot: symptoms, diagnosis, correction, prognosis
About 100 years ago, the diagnosis of the "Fallot tetralogy" sounded like a verdict. Of course, the complexity of this defect allowed for the possibility of surgical treatment, but the operation was performed for a long time to relieve only the patient's suffering as it could not eliminate the cause of the disease. Medical science went first, the best minds, developed new methods, did not stop hoping that the disease could be dealt with. And no mistake - thanks to the efforts of people who have dedicated their lives to fighting heart failure, it has been possible to heal, prolong life and improve quality, even with such discomforts as Fallot tetralogy. Now new technologies in cardiac surgery allow,
The very name of the disease implies that it is due to its appearance not just one, but four deficiencies that determine the condition of a person: Tetralogy Fallot is a congenital heart disease in which 4 abnormalities are combined:
- Defect of the septum between the ventricles of the heart, usually there is no membrane part of the septum. The length of this defect is quite large.
- Increased volume of the right ventricle.
- Narrowing of the lumen of the pulmonary trunk.
- The shift of the aorta to the right (dextrose position), to the state when it partially or even completely leaves the right ventricle.
Circulatory disorders in the Fallot tetralogy
The Fallot tetralogy refers to the so-called "blue" or cyanotic vices. A defect in the septum between the ventricles of the heart leads to a change in the blood flow, causing the blood to flow into the large circulation, which does not bring enough oxygen into the tissues and in turn starve them.
Due to the increasing hypoxia, the patient's skin gets a cyanotic (cyanotic) shadow, so this vice is called "blue". The situation with the Fallot tetralogy is reinforced by the presence of a constriction in the area of the pulmonary trunk. As a result, insufficient volume of venous blood can escape through the narrowed opening of the pulmonary artery into the lungs, leaving a considerable number of their remnants in the right ventricle and (so patients are blue) in the venous part of the systemic circulation. This mechanism of venous stasis, in addition to reducing the oxygen supply to the blood in the lungs, promotes a fairly rapid progression of CHF (chronic heart failure), which manifests itself:
- Cyanosis with Fallot tetralogy.
- Deterioration of cyanosis.
- Disturbance of metabolism in tissues.
- Accumulation of liquid in cavities.
- Presence of edema.
- In order to prevent such a development of events, the patient is shown cardiac surgery (radical or palliative surgery).
Symptoms of the disease
Given that the disease manifests quite early, this article focuses on the age of the child, beginning with birth. The main manifestations of the Fallot tetralogy caused by elevation of Congestive Heart Failure (CHF), even if these children are not excluded and the development of acute heart failure (arrhythmia, respiratory distress, anxiety, rejection of the breast). The appearance of the child depends largely on the severity of the constriction of the pulmonary trunk and also on the extent of the defect in the septum. The more these disorders occur, the faster the clinical picture develops. The child's appearance largely depends on the severity of the constriction of the pulmonary trunk and also on the extent of the defect in the septum. The more these disturbances occur.
On average, the first manifestations begin with the child's four weeks of life. The main symptoms are:
- Cyanosis staining of the skin in the child first appears when crying, sucking, then cyanosis may also exist at rest. First, only the nasolabial triangle, the fingertips, the ears (acrocyanosis) appear as cyanosis, then, with progressive hypoxia, the development of total cyanosis is possible.
- The child remains in physical development (later it starts to hold his head, sit down, crawl).
- Thickening of the end phalanges of the fingers in the form of "drumsticks".
- Nails become flattened and round.
- The ribcage is flattened, in rare cases, the formation of "heart hump".
- Decreased muscle mass.
- Incorrect growth of the teeth (large interdental spaces), caries develops rapidly.
- Deformation of the spine (scoliosis).
- Flat feet develop.
- A characteristic feature is the appearance of cyanotic attacks involving the child
- breathing becomes more frequent (up to 80 breaths per minute) and deep.
- the skin becomes cyanotic-purple.
- strongly dilated pupils.
- there is shortness of breath.
- weakness is characteristic to the loss of consciousness as a result of the development of hypoxic coma.
- It can give muscle spasms.
typical zones of cyanosis
Older children tend to squat during combat as this position eases their condition slightly. Such an attack lasts on average 20 seconds to 5 minutes. After that, however, children complain of a pronounced weakness. In severe cases, such an attack can lead to a stroke or even death.
Algorithm of the action when an attack occurs
It is necessary to help to "squat" in the child, or take a "knee-elbow" position. Such reduces a position of the venous blood flow from the lower body to the heart and thus reduces the stress on the heart muscle.
The oxygen supply through the oxygen mask at a rate of 6-7 l / min.
Intravenous administration of beta-blockers (e.g. "propranolol" calculated at 0.01 mg / kg body weight) eliminates tachycardia.
Introduction of opioid analgesics ("morphine") reduces the sensitivity of the respiratory center to hypoxia, decreased respiratory rate.
If the attack does not stop within 30 minutes, an emergency operation may be required.
Important! Do not use medications that increase the heart contractions during the attack (cardiotonics, cardiac glycosides)! The effect of these drugs leads to an increase in contractility of the right ventricle, which causes additional shunt due to the defect in the septum. And this means that venous blood is produced in a large circulation circle, which contains virtually no oxygen, which leads to an increase in hypoxia. So there is a "vicious circle".
On the basis of which studies is the diagnosis of the Fallot tetralogy?
At the heart of the auscultation revealed: the attenuation II tone in the second intercostal space on the left is determined by the rough, noise "scraping".
After electrocardiography, it is possible to detect ECG signs of an increase in the right heart and a shift of the heart axis to the right.
Most meaningful is the ultrasound of the heart, where it is possible to identify a defect in the interventricular septum and a displacement of the aorta. Thanks to Doppler imaging, blood flow in the heart can be closely examined: the leakage of blood from the right ventricle to the left and the difficulty of blood flow to the pulmonary trunk.
treatment
If you have a Fallot tetralogy, it is important to remember a simple rule: The operation will be shown to all (without exception!) Patients with this heart defect.
The main method of treating this heart defect is surgical. The optimal age for the surgery is 3-5 months. Surgical procedures should be planned best.
There are situations in which an emergency operation may be required at an earlier age:
- Frequent seizures.
- Appearance of cyanotic skin, shortness of breath, fast heart rate at rest.
- Pronounced delay of physical development.
As a rule, the so-called palliative surgery is urgently performed. At this time, there is no artificial shunt (connection) between the aorta and the pulmonary trunk. This procedure allows you to temporarily strengthen the patient before a complex, multi-component and prolonged operation aimed at eliminating all defects in the Fallot tetralogy.
How is the operation performed?
Given the combination of four abnormalities in this heart disease, surgical intervention in this pathology is particularly difficult in cardiac surgery.
Under general anesthesia, the thorax is cut through the front line.
After access to the heart, the device of the artificial circulation is connected.
Held section of the heart muscle from the right ventricle so as not to offend the coronary arteries.
From a cavity of the right ventricle access to a pulmonary trunk is carried out, narrowing of the narrowed aperture, plastic of valves is carried out.
The next step is to include a ventricular septal defect with synthetic hypoallergenic (Dacron) or biological (from cardiac tissue pockets - pericardium) material. This part of the operation is quite complicated because the anatomical defect of the septum is close to the driver of the heart rhythm.
After the successful completion of the previous stages, the wall of the right ventricle is sewn, the blood circulation is restored.
This operation is performed exclusively in highly specialized cardiosurgical centers, where relevant experience in the treatment of such patients is collected.
Possible complications and prognosis
The most common complications after surgery are:
- Preservation of the narrowing of the pulmonary trunk (in case of insufficient dissection of the valve).
- When fibers are injured that cause arousal in the heart muscle, it is possible to develop various arrhythmias.
On average, the postoperative mortality is up to 8-10%. But without surgical treatment, the life expectancy of children does not exceed 12-13 years. In 30% of the cases, the death of a child in childhood occurs due to heart failure, strokes, and increasing hypoxia.
However, the surgical treatment, performed for children up to 5 years, the vast majority of children (90%) of the re-examination at the age of 14, no signs of delay in the development of their colleagues is noted.
In addition, 80% of the operated children lead a normal lifestyle, virtually no different from their peers, in addition to restrictions on excessive physical effort. Proved that the earlier the radical surgery was performed to eliminate this deficiency, the sooner the child recovers and comes up with the development of his peers.
Is the disabled group registered for the disease?
All patients prior to radical cardiac surgery, as well as 2 years after the operation, will be notified of a disability record after which a re-examination is performed.
When determining the disability group, the following indicators are very important:
- Is there a circulatory disorder after surgery?
- Whether the stenosis of the pulmonary artery remains.
- The effectiveness of surgical treatment and whether there are complications after surgery.
Can I diagnose the tetralogy of Fallot in utero?
The diagnosis of this heart defect depends directly on the qualifications of a specialist who carries out ultrasound diagnostics during pregnancy and also at the level of the ultrasound machine.
When ultrasound expert class specialists demonstrated high-grade Fallot tetralogy performing 95% of cases up to 22 weeks in the third trimester of pregnancy actively defect is diagnosed in almost 100% of cases.
Another important factor is genetic research, the so-called "genetic deuces and triplets," which are studied for all pregnant women over a 15-18 week period. Proved that Fallot's tetralogy is associated with other anomalies in 30% of cases, most commonly chromosomal diseases.
What if the pathology is found in the fetus during pregnancy?
When this heart disease is diagnosed in combination with a serious chromosome abnormality, accompanied by severe mental retardation, a woman is offered abortion due to medical conditions.
If only heart disease is diagnosed, a consultation will take place: Obstetrician gynecologists, cardiologists, cardio-surgeons, neonatologists, as well as a pregnant woman. At this consultation, the woman is explained in detail: the more dangerous the pathology is to the child, the consequences it may have, and also the possibilities and methods of surgical treatment.
Despite the complex nature of the Fallot tetralogy, this heart disease belongs to the functional capacity, that is, he undergoes a surgical correction. This disease does not represent a sentence to a child. The modern medical level, in 90% of cases, significantly improves the patient's quality of life through a complex, multi-stage operation.
Currently, cardiac surgeons use virtually no palliative surgery that only temporarily improves the condition of the patient. The priority is a radical surgery in early childhood (up to a year). This approach allows us to normalize the overall physical development, avoid the formation of permanent deformations in the body, which significantly improves the quality of life.
Features of Fallot Tetralogy: What is it, causes and treatment?
From this article you will learn: What is the name of Fallot's tetralogy, which defects are combined with this congenital malformation? What causes the occurrence of the tetrad, the characteristic symptoms of pathology. Diagnosis and treatment methods, prognosis for recovery.
The Fallot tetralogy is a severe congenital heart disease with four (tetrad) characteristic defects:
- Strong aortic shift to the right (the normal aorta leaves the left ventricle with the tetralogy of Fallot - all or part - from the right ventricle).
- The maximum stenosis (constriction) of the pulmonary artery strain (usually blood from the right ventricle enters the lungs and is saturated with oxygen).
- Absence of interventricular septum.
- Dilatation (increase in volume) of the right ventricle.
What happens in pathology? Due to defects:
- venous and arterial blood is mixed into the ventricles and enters the large circulation, which is insufficiently saturated with oxygen;
- improves anoxia of tissues and organs Aorta and pulmonary artery shift narrowing (stenosis of the stronger, the smaller blood in the lungs enriched with oxygen and stay longer in the ventricle, aggravating the overload);
- Severe disorders in the large (from the left ventricle to the aorta) and small (from the right ventricle to the pulmonary artery) rapidly lead to the development of chronic heart failure.
- As a result, the sick child has a characteristic cyanosis (cyanosis at the first limbs, nasolabial triangle and then all skin), shortness of breath, ischemia of the brain and the whole organism.
The Fallot tetralogy is a congenital malformation, all defects occur in the period of intrauterine organ formation and appear immediately after the birth of the child. The average life expectancy is no more than 10-12 years, after the operation the prognosis improves depending on how pronounced the malformations of organ formation are. Only about 5% of children with pathology mature and live up to 40 years, so it is usually considered a pediatric pathology.
When we talk about the differences between the manifestations of Fallot tetralogy in childhood and adulthood, then they do not exist; at any age, the development of chronic heart failure can lead to a loss of working capacity and severe disability.
The pathology is considered one of the most serious congenital heart defects, it is the dangerous rapid development of complications of heart failure and ischemia of the organs and tissues. In the first two years more than 50% of children die of stroke (acute oxygen deficiency of the cerebral vessels), brain abscess (purulent inflammation), acute heart failure. Inexperienced vice leads to a serious delay in the development of the child.
After all, it is impossible to cure heart disease, surgical methods can only improve the patient's prognosis and extend the lifespan. In this case, there is a direct dependency on the time of surgery - the earlier it was performed (preferably in the first year of life), the greater the chance of a positive outcome.
The surgical correction of the Fallot tetralogy is performed by cardiovascular surgeons, observation of patients before and after surgery - cardiologist.
Causes of the appearance
Since the heart is laid and shaped in the first trimester, especially dangerous for the occurrence of the Fallot tetralogy, one should consider the effect of toxins at 2-8 weeks gestation. Most common are:
- Medications (hormonal, sedative, hypnotics, antibiotics, etc.).
- Infectious diseases (rubella, measles, scarlet fever).
- harmful chemical compounds of industry and household (heavy metal salts, pesticides and fertilizers).
- toxic effects of alcohol, drugs and nicotine.
- The risk of a blemish increases in families where close relatives have children with intrauterine anomalies of heart development.
Symptoms of pathology
Fallot's tetralogy is a very serious, life-threatening disease of the heart that quickly becomes complicated by the manifestations of heart failure and cerebral circulatory disturbances, which significantly worsens the prognosis and makes life difficult for the patient. From the earliest childhood on, even elemental physical activity ends with dyspnea attacks, severe cyanosis (cyanosis), weakness, dizziness, fainting.
In the future, seizures may cause breathing to stop, seizures, hypoxic coma (due to lack of oxygen in the blood), in the future - partial or total disability. The characteristic posture of the patients is to squat, under tension, to facilitate the post-loading condition.
After the operation, the well-being of the patient improves, but the physical activity is so limited that no shortness of breath or other symptoms of heart failure occur.
The main features of a defect are due to a disturbed accumulation of the blood with oxygen, which is why it is called "blue".
Characteristic Symptoms of Fallot Tetralogy:
- Shortness of breath that occurs after any actions (crying, sucking) and builds up
- Strong weakness (caused by the most elementary acts)
- Loss of consciousness (the last two symptoms are due to progressive ischemia of the brain)
Complication of pathology - cyanotic seizures, the onset of which indicates severe hypoxia (hypoxia) and severely worsens the patient's prognosis. As a rule, they appear around 2-5 years and are accompanied by such symptoms:
- Breathing and heart rate increase more frequently (from 80 beats per minute).
- Dyspnea rises.
- The child is worried.
- Cyanosis significantly increased to a purple hue.
- There is a strong weakness.
- The attack can lead to unconsciousness, seizures, respiratory arrest, coma, stroke or sudden death.
Due to lack of oxygen, children with congenital malformations are lagging behind in development, learning other skills (not keeping their heads, etc.), often getting sick.
diagnosis
Over time, patients develop characteristic external signs that can be used to make a primary diagnosis:
- the most characteristic indicator is the acrocyanosis (cyanosis of the peripheral parts - brushes, feet, ear tips, 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 "watch glasses" (convex, round);
- Delay in physical development, weight loss;
- a flattened ribcage (more rarely a chest bump).
- When listening to the heart, a harsh "humming" or "scratching" is detected.
The Fallot tetralogy in children is confirmed by hardware methods:
- Ultrasound, which determines the change in the size of the ventricles (dilatation of the right ventricle).
- Incomplete blockade of the fascia (right) of the fasciculus and hypertrophy (enlargement, thickening) of the right ventricular myocardium is recorded on the ECG.
- With the help of X-rayography, a characteristic lung pattern (due to circulatory disorders it looks translucent) and heart (increase in size and shape in the form of a boot or shoe with a raised heart peak) is recorded.
- Dopplerography can determine the direction of blood flow and the diameter of the vessels.
- In the general analysis of blood (instead of the norm) - twice the increased number of red blood cells (erythrocytes). This is because the body tries to compensate for the lack of oxygen by increasing the cells that can meet this need.
Methods of treatment
Healing pathology is completely impossible:
- in 30%, violations are combined with other congenital anomalies, making prognosis and treatment more difficult;
- in 65% of the hemodynamic disorders (blood flow) are so pronounced that the surgical treatment improves the condition of the patient for some time, prolongs the time and improves the quality of life, but the pathology gradually progresses, leading to the development of chronic heart failure.
Treatment methods include:
- Drug therapy (emergency aid for cyanotic attacks).
- Palliative intervention (preparation for radical surgery, temporary elimination of critical hemodynamic disorders).
- Radical correction (restoration of the interventricular septum, movement of the aortic aorta, enlargement of the pulmonary artery stenosis, etc.).
Life expectancy and further prognosis depend exclusively on how promptly the operation was performed.
Medical therapy
Drug therapy is used as an emergency for a cyanotic attack:
- Oxygen inhalations are used to restore the saturation of tissues and blood with oxygen;
- a solution of sodium bicarbonate is injected to relieve acidosis (accumulation of metabolic products);
- to facilitate the breathing process, use broncho- and spasmolytics (euphyllin);
- To avoid shock due to gas exchange disorders, adhesion (clumping of erythrocytes) and the formation of thrombi, intravenously injected solution of plasma replacement (Rheopolyglucin).
- After the onset of seizures, the prognosis of the child worsens, in the near future a surgical correction of the defect is required.
Temporary removal of hemodynamic disorders
Temporal correction or palliative methods are used immediately after birth or at an early age, including the creation of various anastomoses (joints, pathways) between the vessels.
Fallot tetralogy in children
In the group of congenital heart defects of tetralogy Fallot occupies a stable tenth place. The prevalence among the "blue" vices is half. Medical reports and reference literature often use the abbreviation of the UPU, which is synonymous with the term "congenital heart disease".
In the International Classification of Diseases ICD-10, it is included in the congenital anomaly group under code Q21.3. An unusual combination of violation of the formation of the heart and the great vessels, which was described in 1888 by A. Fallot as a separate syndrome. His name remained in the history of medicine.
What anomalies is the syndrome, features of anatomy
The tetralog of Fallot contains a combination of four anomalies:
- Defect in the interventricular septum.
- right-sided position of the aorta (as if one were "sitting astride" on both ventricles).
- Stenosis or complete invasion of the pulmonary artery, lengthened and narrowed due to the rotation of the aortic arch.
- Right ventricular hypertrophy of the myocardium.
- Among the combinations of pulmonary artery stenosis defects and septal defects, there are 2 other forms also described by Fallot.
The triad consists of:
- Openings in the interatrial septum;
- Stenosis of the pulmonary artery;
- Right ventricular hypertrophy.
- Pentada - the first variant adds the broken integrity of the interatrial septum.
In most cases, the aorta receives a large amount of right heart blood without sufficient oxygen concentration. Hypoxia is formed by the type of circulation. Cyanosis is detected in a newborn baby or in the first few years of a baby's life.
This narrows the funnel of the right ventricle, forming a cavity above it, similar to an additional third ventricle. The increased load on the right ventricle contributes to its hypertrophy to the left.
The only compensatory mechanism in this situation can be considered to be the emergence of a significant collateral (accessory) network of veins and arteries that deliver blood to the lungs. An open botanical duct temporarily maintains and improves hemodynamics.
For the Fallot tetralogy, a combination with other development anomalies is typical:
- prevention of botulinum gangrene;
- an additional superior vena cava;
- additional coronary arteries;
- Dandy-Walker syndrome (hydrocephalus and underdevelopment of the cerebellum);
- in ¼ of patients the embryonic right arch of the aorta is preserved (Corvizar disease);
- congenital dwarfism and oligophrenia in children (Cornelia de Lange syndrome);
- Defects of internal organs.
causes
The causes of anomalies are the effects on the fetus in the early stages of pregnancy (from the second to the eighth week):
- Infectious diseases of the future mother (rubella, measles, influenza, scarlet fever).
- Take alcohol or drugs.
- Treatment with hormone preparations, sedatives and hypnotics.
- toxic effect of nicotine.
- Intoxication with toxic industrial products in harmful industries.
- Hereditary predisposition is possible.
- It is important that a woman does not notice a pregnancy in a very short time and triggers the pathology of the fetus itself.
Variations of the Fallot tetralogy
It is common to distinguish four types of Fallot tetralogy according to the peculiarities of anatomical changes.
Embryonic constriction is caused by the shift of the septum to the left and low localization. The maximum stenosis is consistent with the height of the anatomic demarcation muscle. At the same time, the structures of the pulmonary valve are virtually unchanged; moderate hypoplasias are possible.
Hypertrophic - a 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 caused by incorrect dividing during the embryonic period by the total arterial trunk, due to which the pulmonary cone (future pulmonary artery) are underdeveloped and a short constricted. At the same time you can change the valve device.
Multi-component - all listed factors participate in education.
Features of hemodynamics
The severity of the defect is due to the degree of narrowing of the diameter of the pulmonary artery. To diagnose and determine the tactics of treatment, it is important to distinguish three types of anomalies:
- Fully unperforated (atresia) arterial lumen: the most severe injury in high interventricular hole mixed blood of both ventricles are directed primarily to the aorta, in the case of complete atresia blood pronounced anoxia flows through the ductus arteriosus or lung collaterals.
- acyanotic form: mild stenosis at a lower pressure barrier than in the aorta can overcome on the way of blood flow from the right ventricle, while shunt favorably called by the artery into the vein, wrong option called 'white', such as cyanosis of the skin not trained.
- Cyanotic form with varying degrees of stenosis: caused by progression of obstruction, blood leakage from right to left; This causes a transition from the "white" shape to the "blue" shape.
symptoms
a significant cyanosis - around the lips, in the upper half of the body, is strengthened, nourished, strained by the crying of the child.
Shortness of breath - associated with paroxysmal signs of physical activity, the child gets the most comfortable posture "squat", explaining the temporary extra reflex spasm of the pulmonary artery and the blood oxygen saturation of the completion of a factor of 2.
Fingers in the form of "drumsticks".
physical underdevelopment and weakness of children; Running, outdoor games cause increased fatigue, dizziness.
Cramps - are associated with hypoxia of brain structures, blood thickening, a tendency to thrombosis of the cerebral vessels.
early manifestations in the form of cyanosis immediately after birth or during the first 12 months of life.
the classical course is the manifestation of cyanosis at the age of two to three years;
severe form - paroxysmal clinic with dyspnea and cyanosis.
late - cyanosis occurs only after 6 or 10 years.
acyanotic form.
Attack of breathlessness may occur alone: The child becomes restless, increasing cyanosis and breathlessness, increased heart rate, a possible loss of consciousness, followed by convulsions and focal manifestations in the form of partial paralysis of the limbs.
diagnosis
The diagnosis is made by observation of the child and the presence of objective signs. Information from relatives on development and activity, attacks of loss of consciousness and cyanosis will be considered.
When looking at children, reference is made to the cyanotic nature of the lips, the altered shape of the end phalanges of the fingers. Rarely formed "heart hump".
Percutaneously, the boundaries of the heart are not altered or enlarged in either direction. If auscultation is heard in the fourth intercostal space to the left of the sternum, systolic coarse noise due to passage of blood through the hole in the interventricular septum. It is better to listen to the patient prone.
Because of the absence of the arch of the pulmonary artery, the retraction occurs at the site where the vessels are normally located. The impoverished lung appears more transparent. There is no enlargement of the heart to big sizes.
In the general analysis of blood, an adaptation response to hypoxia is found in terms of an increase in the number of erythrocytes and the growth of hemoglobin.
Ultrasound Diagnosis Using a conventional ultrasound machine or Doppler studies using just the changes in the ventricles, abnormal vessel development will determine the direction and amount of blood flow.
On an ECG (electrocardiogram) there are signs of right-hand hypertrophy of heart, it is possible to block the right leg of a bundle of Hits, the electric axis is considerably distracted to the right.
The probing of cardiac cavities with pressure measurement in chambers and vessels takes place in specialized clinics in deciding on the question of surgical treatment.
Less commonly, coronary angiography may require magnetic resonance imaging.
In the differential diagnosis it is necessary to exclude a number of diseases:
the transposition of the pulmonary artery causes a significant increase in the heart as the child grows.
Infection at the level of the tricuspid valve promotes hypertrophy of the left ventricle, not the right.
Eisenmenger's tetrad - a vise that is not accompanied by infection, but by the extent of the pulmonary artery, its pulsation and the characteristic pattern of the lung fields is determined on an X-ray.
Stenosis of the lumen of the pulmonary artery is not accompanied by a picture of the "shoe".
Atypical forms help to differentiate Dopplerographic findings.
treatment
The drug therapy of the patient with the Fallot tetralogy is performed only for the purpose of preparation for the operation or in the postoperative period. The only goal is to support the myocardium, to prevent possible thrombosis after attacks and a disturbed coronary and cerebral circulation.
Inhalation of an oxygen-air mixture by nasal catheter or in an oxygen cylinder. Newborns are kept in special resuscitation boxes to reduce hypoxia.
intravenously injected solution of reopoliglyukin, eufillin (in the absence of tachycardia).
In conjunction with tissue acidosis, a sodium bicarbonate solution is needed.
Operations can be:
- an immediate measure for the temporary help.
- Shunt type for the relief of blood flow along a new canal.
- Radical choice with correction of the interventricular septal defect and the location of the aorta.
- In the form of emergency care, the creation of an artificial joint (anastomosis) between the aorta and the pulmonary artery is used with the aid of a prosthesis.
It is used as the first stage of surgical intervention for newborns and infants. It is believed that such actions allow the child to be prepared and avoid complications with further treatment, reducing the risk to 5-7%.
It is necessary to decide the question of final planned correction of deficiency at the age of up to three years. Temporary anastomoses can be superimposed between clavicle and pulmonary arteries.
Radical surgery involves plastic membrane exit of the right ventricle, the withdrawal opening in the ventricular septum, valvotomy (dissection overgrown pulmonary valve). It is performed on the open heart, requires the use of the device of artificial circulation.
Can operative complications develop?
After surgery there is a risk of complications. This includes:
- Development of acute heart failure;
- increased thrombus formation at the site of the anastomosis;
- Pulmonary hypertension;
- Arrhythmias or atrio-ventricular blockades;
- infective endocarditis;
- Aneurysm of the wall of the right ventricle.
- The success of surgical treatment depends on the timeliness and adequate preparation of the patient, the experience of cardiac surgeons.
What is the prognosis of patients with a defect?
Children with Fallot tetralogy whose parents disagree with the operation become weak, can not move enough, play with their peers. Common to them are common infectious diseases (influenza, acute respiratory viral infection, tonsillitis, sinusitis and other sinusitis, repeated inflammation in the lungs). The average age of survival is 12 years.
The adult often joins tuberculosis. Against the background of a blemish, the prognosis of a disease is unfavorable, all diseases are severe, with cardiac decompensation, thrombosis. The most common causes of death are ischemic stroke, brain abscess. Up to 40 years, not more than 5% of people live with this anomaly. As a rule, these are people with a high degree of disability who need external care.
In severe cases of malformation, 25% of children die without surgery in the first year of life, half in the first month.
All patients are monitored by the cardiologist, consulted by cardiochirurgens. Every year he is shown antibiotic prophylaxis, it is recommended to maintain the health in the oral cavity.
Under the conditions of modern medicine, patients with the Fallot tetralogy are treated by clinics of a cardiosurgical profile. After the operation, the patient disappears cyanosis, suffocation attacks. Parents should listen to the opinion of specialists. The lost term can not be assigned by an adult child.