Congenital heart disease - Causes, symptoms and treatment. MF.
Causes of congenital heart disease
Symptoms
Diagnosis
Treatment
Lifestyle with heart disease
Forecast
As you know, the heart is an important organ in the human body, the well-being of the whole organism depends on its proper functioning. The heart consists of the muscle( myocardium) and connective tissue( heart valves, the walls of large vessels).The activity of the heart is caused by the rhythmic and consequent reduction of its constituent structures( atria and ventricles), as a result of which the blood, flowing through the lungs, is saturated with oxygen( the so-called small circle of blood circulation) and carries oxygen to all organs and tissues( large circulation).
When the fetal heart develops during pregnancy, its work is different from the heart of a newborn baby and an adult. In particular, the small circle of blood circulation begins to function fully only during childbirth, when the opening of the lungs of the newborn occurs, and at the moment of the first cry they fill with blood. Therefore, in the heart of the fetus, there are special openings and ducts that allow blood to circulate in the developing organism bypassing the lungs( arterial duct connecting the aorta and the pulmonary artery, oval window between the atria, venous duct between the umbilical vein and the inferior vena cava).
Sometimes, under the influence of unfavorable factors during fetal development, there are gross violations of the formation of anatomical structures of the heart, which affects its activity and is not always compatible with the child's life. Then either by ultrasound of the fetus during pregnancy, or in the first days after childbirth, the newborn is diagnosed with congenital heart disease .What is it, what is the prognosis for life and what methods of preserving the life and health of such babies are offered by modern medicine, we will try to find out in this article.
So, these pathological conditions represent a large group of heart diseases, united by the following symptoms:
- occur during the period of intrauterine development;
- characterized by gross violations of the architectonics of the heart and large vessels( aorta, inferior and hollow veins, pulmonary arteries and veins);
- anatomical changes cause significant disturbances in hemodynamics( circulation of blood through the body);
- due to underdevelopment of anatomical structures or a change in their normal location in the heart.
The prevalence rate varies from 6 to 9 per 1,000 live births. According to different authors, there are 50 to 100 possible variants of congenital heart disease.
Schematic representation of some heart defects
Causes of congenital heart diseases
Due to the fact that during the intrauterine development there is an active formation of all organs of the human body, especially the heart, Fetus and pregnant woman are vulnerable to various negative factors. So, an increased radiation background, ionizing radiation, infectious diseases of the mother, especially viral - rubella, measles, chickenpox, herpes, may have an effect on the wrong course of organogenesis;taking certain medicinal and toxic substances( drugs, alcohol) during pregnancy, especially in the first trimester( 8 -12 weeks - the period of the most intensive formation of all organs of the fetus).In 4-10% of cases, congenital defects are genetically determined, that is, they are inherited.
Symptoms of congenital heart disease
Clinical symptoms of defects depend on their type. Define the vices of "blue" and "white" types, as well as vices that create an obstacle to blood flow.
The main manifestation of the of the "blue" vices ( transposition of the main arteries, the tetralogy of Fallot, atresia - the infection of the tricuspid valve) is cyanosis - blue staining of the skin of the fingers, hands, feet, nasolabial triangle, ears, nose, or, extremelysevere cases, whole body. Also, the manifestation of arterial hypoxemia( low oxygen) is shortness of breath, loss of consciousness with convulsions or without, tachycardia( rapid heartbeat), lag in growth and development, frequent colds, neurological symptoms due to lack of normal blood supply to the brain. As a rule, vices of this type manifest themselves in the first hours and days of life of a newborn child. Transposition of the main arteries( the hollow veins enter the left and not the right atrium, and the aorta moves away from the right and not the left ventricle) is a serious blemish incompatible with life, and the child, as a rule, dies immediately after birth. Also, a three-chambered heart( two atriums and one ventricle, or one atrium and two ventricles) belong to incompatible vices. The lethality of defects of this type is very high, if children with such vices survive in the first days of life, then without treatment die by the end of the first - the second year.
To defects of the white type include the defect of the interventricular septum, the open Botallov( arterial) duct, the defect of the interatrial septum. Clinically, these vices can begin to manifest themselves not in the period of early childhood, but by 16 - 20 years. Symptoms of defects of the white type are the following: pallor of the skin, developmental lag, frequent ARI, signs of developing right ventricular failure - dyspnea and tachycardia with exercise or rest.
Flaws with an obstruction to blood flow: aortic stenosis, coarctation( segmental narrowing) of the aorta, stenosis of the pulmonary artery are clinically manifested by shortness of breath, tachycardia, pain in the chest, decreased endurance, swelling, developmental lag and circulatory disturbances in the lower half of the trunk. With coarctation of the aorta, children survive to a maximum of 2 years of age.
Diagnosis of congenital heart disease
As a rule, it is possible to diagnose malformations of the fetus, including the heart, during the ultrasound examination of a pregnant woman. Then, depending on the type of defect, a woman can be recommended either to terminate pregnancy( for vices incompatible with life, for multiple deformities, etc.), or to maintain pregnancy with a more detailed examination of the pregnant woman and to decide on the prompt treatment of the child at onceor some time after delivery. But sometimes, for some reasons( not visiting a pregnant woman's consultation and ultrasound examination of the cabinet, insufficient equipping of obstetrical stations with ultrasound equipment, etc.) developmental defects can be diagnosed only after childbirth.
Although the symptoms of congenital malformations are quite vivid, in the course of a clinical examination of a newborn child, the diagnosis can only be presumed, since many of the symptoms are not strictly specific, but may be due to other severe conditions of newborns( respiratory distress syndrome, intracranial hemorrhages,etc.).Also, not all defects can give a characteristic auscultatory picture( in the process of listening to the chest), and vice versa, noises, clicks or other auscultatory manifestations can occur with slight deviations from the norm in the structure of the heart( small anomalies).Therefore, if the heart defect was not diagnosed by ultrasound of the fetus, all children with symptoms of circulatory disorders( diffuse or acrocyanosis, dyspnoea at feeding or at rest, loss of consciousness, convulsions) should have an ultrasound of the heart during the stay in the hospital.
Echocardiography( ultrasound of the heart) is one of the most informative methods of visualization of heart defects. EKG can also be prescribed( it will show rhythm disturbances, atrial and / or ventricular hypertrophy, if any), chest x-ray( will show stagnation of blood in the lungs, if there is, an increase in the shadow of the heart due to the expansion of its chambers).In especially difficult cases or before surgical treatment, ventriculography( introduction of X-ray contrast substance into the ventricular cavity of the heart), angiography( introduction of contrast through the vessels in the heart cavity), probing of the heart chambers with measurement of the pressure in them can be prescribed.
Now let's take a closer look at ultrasound diagnosis of frequently occurring heart defects.
A. Congenital heart flogging with volumetric overload of vessels in the lungs( small circle of blood circulation).
1. Defect of the interatrial septum - one-dimensional echocardiography reveals signs of volume overload and dilatation( expansion) of the right ventricle, in two-dimensional echocardiography, an echo cancellation between the atria is seen, and in the Doppler study, a turbulent( with "swirls") flow of blood through the interatrial septum anddegree of pulmonary blood flow disorders.
2. Defect of the interventricular septum - when performing ultrasound of the heart, the opening in the ventricular septum, dilatation of the left and right ventricles, turbulent blood flow from the left to the right ventricle are visualized, the degree of severity of pulmonary hypertension is estimated, the pressure difference in the ventricles is measured.
The defect of the septum between the ventricles during echocardiography with doppler manifests itself. Left - the normal interventricular septum, to the right - its defect( VSD).
3. Open Botallov duct - manifested in echocardiography by continuous blood flow through communication in the aorta and pulmonary arteries, changes in blood flow at the mouth of the pulmonary trunk.
4. Coarctation of the aorta - a segmental segment of narrowing of the aortic lumen is seen, accelerated blood flow below the segment.
5. Transposition of the main arteries - improper withdrawal of blood vessels from the heart is visualized.
B. Heart disease with a decrease in blood volume in the small circle of the circulation.
1. Fallot tetraphy - in echocardiography, in addition to stenosis( constriction) of the pulmonary artery and the defect of the septum between the ventricles, hypertrophy of the right ventricle and aortic dissection from the right ventricle are determined, and the extent of intracardiac hemodynamics is assessed.
2. Stenosis of the pulmonary artery - reveals narrowing of the lumen of the pulmonary artery, and acceleration of blood flow in the mouth of the pulmonary artery and hypertrophy of the right ventricle.
3. Epstein's anomaly is a pathology of the development of the tricuspid heart valve, when the valve flaps are attached not to the fibrous ring between the atrium and the ventricle to the right, but to the walls of the right ventricle, which causes a decrease in its volume. With ECHO-CG, the pathology of the valve is determined, the degree of enlargement of the heart cavities and the violation of intracardiac blood flow are estimated.
4. Atrial tricuspid valve - with ECHO-CG there is no echo reflection from the tricuspid valve, hypertrophy of the right atrium and left ventricle is recorded.
B. Heart defects with a decrease in the volume of circulating blood in a large circle of blood circulation( in the vessels of all vital organs).
1. Coarctation of the aorta( see above).
2. Isolated aortic stenosis - the degree of constriction is assessed, visualized accelerated blood flow through the aortic valve and deformed valve flaps.
G. Heart defects without hemodynamic disorders.
- Dextrocardia( mirror location of the heart on the right) is a rare enough anomaly, as a rule, ultrasound of the heart of hemodynamic disorders is not recorded.
Treatment of congenital heart disease
Complete recovery of the vast majority of heart defects is possible only with the help of their surgical correction. Cardiac surgery can be performed in the first hours or days of a newborn's life, or in the first year of a child's life. When the arterial duct is open, wait-and-see tactics are acceptable( in the absence of symptoms of life-threatening conditions), since this duct can independently close in the first two years of life.
Operations can be performed both on the open heart( with dissection of the chest wall) and cardiovascular method( when access to the heart is carried out by inserting a probe into the vessels of the probe reaching the heart cavities).The latter method, for example, is used to correct defects between the atria or ventricles, to which an occluder closing the orifices is fed by means of a probe.
But with some heart defects that are not compatible with life, for example, a three-chambered heart( one atrium and two ventricles, or two atria and one ventricle), surgical correction is unfortunately not possible.
In addition to cardiosurgery, the patient is prescribed medicamentous therapy to improve the contractile function of the heart and to "unload" the small circle of circulation. ACE inhibitors( enalapril, perindopril, lisinopril, ramipril, etc.), diuretics( furosemide, indapamide, etc.), B-adrenoblockers( carvedilol, bisoprolol, etc.) are used.
Lifestyle with congenital heart disease
The patient should follow the following recommendations regarding his lifestyle:
is a balanced diet;
- observance of a diet with restriction of table salt and volume of consumed liquid( for reduction of volume overload of heart and vessels);
- sufficient stay in the open air;
- exclusion of any sport activities and restriction of strong physical exertion;
- sufficient duration of sleep;
- regular monitoring of a cardiologist and a cardiac surgeon with the necessary medical and diagnostic measures;
- Pregnancy for women with vices of the "blue" type is strictly contraindicated, but if surgical treatment was performed, the possibility of maintaining pregnancy is determined in each case individually with joint management by a pregnant cardiosurgeon, cardiologist and obstetrician-gynecologist in a specialized hospital. The delivery is usually done by cesarean section.
Prognosis for congenital heart disease
Prognosis for the life of most heart defects is unfavorable. As mentioned above, cardiosurgical treatment for such babies is required in the first days and months after delivery, otherwise they die within the first two years because of progression of heart failure or complications( bacterial endocarditis, fatal rhythm disorders, increased risk of thrombosis and thromboembolic complications, frequent diseases of the bronchopulmonary system( prolonged bronchitis, severe pneumonia). Exceptions are white flaws, in which, in the absence of surgical treatmentfor some reason, children are able to survive until the age of 16 to 18.
After surgical correction, the prognosis for life is favorable, but still the child's condition remains serious enough, therefore, such children should be closely monitored by parents and doctors.
In conclusion,that the given diagnosis at the child at the present stage of development of medicine is not a verdict, as the possibilities of domestic and foreign cardiology of childhood allow to bear, give birth and raise a baby,to lead a full-fledged way of life, despite such a serious illness.
Doctor therapist Sazykina O.Yu.