The combination of four strictly defined anatomical defects in the structure of the heart and blood vessels gives grounds to diagnose the UPU called "Fallot's Tetrad" - we'll figure out what it is.
This disease was identified and described by French pathologist ELFallo in 1888.Over the past 100-odd years, doctors have been able to achieve good results in the early detection and elimination of this ailment.
Description of the disease, its prevalence
Tetrada Fallot - congenital malformationHeart, which is quite often diagnosed in newborns. In 3% of babies this disease occurs, which is a fifth of all detected congenital heart defects.
30% of all miscarriages and undeveloped pregnancies are associated with the presence of the tetralogy of Fallot in the fetus, and another
7% of babies with this pathology are born dead. The tetralogy of Fallot is diagnosed if there is a combination of just four anatomical defects in the structure of the cardiovascular system in a newborn:
- Stenosis or narrowing of the pulmonary artery that exits the right ventricle. Its immediate purpose is to carry the venous blood to the lungs. If there is a narrowing in the mouth of the pulmonary artery, then blood flows from the ventricle of the heart into the artery with effort. This leads to a significant increase in the burden on the right heart. With age, stenosis increases - that is, this defect has a progressive course.
- Defect in the form of a vent in the interventricular septum( ).Normally, the ventricles of the heart are separated by a dull partition, which allows you to maintain a different pressure in them. In the tetralogy of Fallot, there is a lumen in the interventricular septum and, therefore, the pressure in both ventricles is equalized. The right ventricle, at the same time, pumps blood not only into the pulmonary artery, but also into the aorta.
- Dextrase or displacement of the aorta .Normally, the aorta is on the left side of the heart. With tetralogy of Fallot, 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 load on the right parts of the heart due to narrowing of the artery and displacement of the aorta.
In 20-40% of cases with tetralogy of Fallot there are accompanying cardiac malformations:
- right-sided arch of the aorta;
- opening in interatrial septum;
- open arterial duct;
- additional upper vena cava on the left.
Causes and Risk Factors
Anatomical heart structures of are formed in the fetus in the first three months of the intrauterine development of .
It is at this time that any damaging effects from the outside of the pregnant woman's body can have a fatal effect on the formation of congenital heart disease.
Risk factors at this time are:
- viral diseases occurring in acute form;
- use of pregnant women with certain medications, psychoactive and psychotropic substances( including tobacco and alcohol);
- heavy metal poisoning;
- radioactive irradiation;
- the age of the future mother is more than 40 years.
Types of the disease and the stages of its development
Depending on the anatomical features of the defect, several types of tetralogy of Fallot are distinguished:
- Embryological - maximum arterial narrowing is at the level of the delimiting muscular ring. Stenosis can be eliminated by economical resection of the arterial cone.
- Hypertrophic - maximum arterial narrowing is at the level of the delimiting muscular ring and the entrance to the right ventricle. Stenosis can be eliminated with the help of massive resection of the arterial cone.
- Tubular - the whole arterial cone is narrowed and shortened. Patients with such a defect should not undergo radical surgical correction of pulmonary artery stenosis. Palliative plastic is preferred for them, which helps to prevent the aggravation of the disease.
- Multi-component - arterial stenosis is caused by a number of anatomical changes, the position and characteristics of which determine the successful outcome of surgical correction of stenosis.
Depending on the characteristics of the clinical manifestations of the defect, its following forms are distinguished:
- cyanotic or classical - with pronounced cyanosis of the skin and mucous membranes;
- Acyanotic or "pale" form of - more common in children of the first 3 years of life as a result of partial compensation of the defect.
The severity of the current distinguishes such forms of the disease:
- Heavy .Dyspnea and cyanosis appear almost from birth when crying, feeding.
- Classical .The disease debuts at the age of 6-12 months. Clinical manifestations closely correlate with increased physical activity.
- Paroxysmal .The child suffers from severe dyspnea-cyanotic seizures.
- Light form with late appearance of cyanosis and dyspnea - at 6-10 years.
In its course, the disease passes through three consecutive stages:
- Relative well-being of .Most often this phase lasts from birth to 5-6 months. There are no expressed symptoms of the disease, which is due to partial compensation of the defect due to the features of the heart structure of the newborn.
- The cyanotic phase of .The most difficult period in the life of a baby with a tethered Fallot, which lasts 2-3 years. In this case, all the clinical manifestations of the disease are pronounced: shortness of breath, cyanosis, attacks of suffocation. At this age, the greatest number of lethal outcomes of the disease.
- Transition phase or the phase of the formation of compensatory mechanisms. The clinical picture of the disease persists, but the child adapts to his illness and knows how to prevent or ease the attacks of the disease.
Danger and complications
The diagnosis of "tetralogy of Fallot" refers to the category of severe cardiac malformations with high mortality. In case of an unfavorable course of the disease, the average life expectancy of the patient is 15 years .
Such complications of the disease are not uncommon:
- thrombosis of the vessels of the brain or lungs due to the increased viscosity of the blood;
- brain abscess;
- acute or congestive heart failure;
- bacterial endocarditis;
- delayed psychomotor development.
Symptoms of
Depending on the degree of narrowing of the artery and the size of the opening in the interventricular septum, the age of appearance of the first symptoms of the disease and the degree of their progression vary. The main clinical signs of the tetralogy of Fallot are:
- Cyanosis .Most often occurs in children under the age of one, first on the lips, then on the mucous membranes, face, arms, legs and trunk. Progresses as the child's physical activity increases.
- Thick fingers in the form of "drum sticks" and convex nails in the form of "watch glasses" are formed by 1-2 years.
- Shortness of breath in the form of deep arrhythmic breathing. The frequency of inhalation and exhalation does not increase. Dyspnea increases with the slightest physical exertion.
- Fast fatigue .
- Heart "hump" - convexity on the chest in the heart.
- Delay in motor development of due to forced limitations in motor activity.
- Noises in the heart .
- The characteristic position of the child's body is squatting or lying with legs bent to the stomach. It is in this position that the baby feels better and therefore unconsciously takes it as often as possible.
- Syncope up to the state of hypoxemic coma.
- "Blue" attacks of dyspnea and cyanosis in severe paroxysmal course of the disease, when children of an early age( 1-2 years) suddenly turn blue, start to choke, become restless, and then they may lose consciousness or even fall into a coma. After the attack, the baby is sluggish and sleepy. Often as a result of such an aggravation the baby may even die.
Diagnosis
The disease is diagnosed on the basis of anamnesis, after clinical examination and auscultation of the thorax, taking into account the results of instrumental research methods :
- blood test;
- radiography;
- electrocardiography.
From the diagnostic point of view the most valuable is the ultrasound of the heart with doppler.
Invasive methods for the investigation of the type of angiography and cardiac catheterization of in infants are rare, only in the case of unsatisfactory results with other diagnostic methods.
Differential diagnosis of of tetralogy of Fallot is carried out with similar on clinical displays diseases:
- full transposition of the main vessels;
- anomaly of Ebstein;
- pulmonary artery disease;
- is the only ventricle.
Methods of treatment
Surgical treatment is indicated. Optimal age for surgery up to 3-5 years .But up to this age still it is necessary to grow up, having passed a stage of the expressed cyanosis and a dyspnea or short wind at the child in 1-2 years.
Quite often children with tetralogy Fallo die precisely during severe dyspnea-cyanotic attacks at an early age if they are not provided with competent medical assistance and support:
- prevention and treatment of concomitant diseases( anemia, rickets, infectious diseases);
- prevention of dehydration;
- sedative therapy;
- treatment with adrenoblockers;
- treatment with antihypoxic agents and neuroprotectors;
- oxygen therapy;
- maintenance therapy with vitamins and minerals.
Depending on the anatomical features of the existing defect, the method of surgical intervention is preferred:
- The palliative operation of is practiced with the impossibility of completely eliminating heart disease. It is necessary to optimize the quality of life of the patient. Most often, aorto-pulmonary anastomosis is applied - that is, the pulmonary artery is connected to the subclavian by means of synthetic implants. Sometimes palliative surgery is just the first stage of surgical intervention in a child under 1 year old - it allows the baby to live a few more years before he will undergo a radical correction of the blemish.
- Radical correction of provides for the elimination of the defect of the interventricular septum and the expansion of the pulmonary artery. This is an open heart surgery, after which the child after a while can lead a practically normal life. It is important to note that this operation is recommended for children no younger than 3-5 years.
Find out more about the disease from the video:
Forecast after operation and without it
If the tetralogy of Fallot was not operated on , then every fourth patient with this defect perished in the first year of life, and half of the children do not survive 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 if the first signs of are detected, scrupulous observation of the child and vigilant medical supervision.
The Fallot Notebook - is not a disease in which you can experiment with alternative treatments for and hope for a miracle. After all, the miracle of the life of a small patient with this disease is only in the hands of a cardiac surgeon.
