Congenital heart diseases – group of the diseases united by existence of the anatomic defects of heart, its valvate device or vessels which arose in the pre-natal period, leading to change of intracardial and system haemo dynamics. Displays of congenital heart disease depend on its look; pallor or cyanosis of integuments, noise in heart, lag in physical development, symptoms of respiratory and heart failure belong to the most characteristic symptoms. At suspicion of congenital heart disease the ECG, FKG, a X-ray analysis, EhoKG, a kateterization of heart and an aortografiya, a cardiography, heart MRT etc. is carried out. Most often at congenital heart diseases resort to cardiac operation – expeditious correction of the revealed anomaly.
Congenital heart diseases
Congenital heart diseases - very extensive and diverse group of diseases of heart and the large vessels which are followed by change of a blood-groove, an overload and insufficiency of heart. Frequency of occurrence of congenital heart diseases is high and, according to various authors, fluctuates from 0,8 to 1,2% among all newborns. Congenital heart diseases make 10-30% of all congenital anomalies. Enter into group of congenital heart diseases as rather easy violations of development of heart and vessels, and severe forms of pathology of heart, incompatible with life.
Many types of congenital heart diseases meet not only separately, but also in various combinations with each other that considerably makes heavier structure of defect. Approximately in a third of cases of anomaly of heart are combined with extra warm congenital defects of TsNS, the musculoskeletal device, a GIT, urinogenital system and so forth.
Defects of an interventricular partition (DMZhP – 20%), defects of a mezhpredserdny partition (DMPP), an aorta stenosis, an aorta koarktation, the open arterial channel (OAC), a transposition of large main vessels (TKS), a stenosis of a pulmonary artery belong to the most frequent options of congenital heart diseases which are found in cardiology (10-15% everyone).
Reasons of congenital heart diseases
The etiology of congenital heart diseases can be caused by chromosomal violations (5%), a gene mutation (2-3%), influence of factors of the environment (1-2%), polygenic and multifactorial predisposition (90%).
Different chromosomal aberrations lead to quantitative and structural changes of chromosomes. At chromosomal reorganizations multiple polysystem anomalies of development, including congenital heart diseases are noted. In case of a trisomiya of autosomes defects of mezhpredserdny or interventricular partitions, and also their combination are the most frequent heart diseases; at anomalies of sexual chromosomes congenital heart diseases meet less often and are presented, mainly, a koarktatsiy aorta or defect of an interventricular peregoyorodka.
The congenital heart diseases caused by mutations of single genes also in most cases are combined with anomalies of other internals. In these cases warm defects are a part autosomno-prepotent (Marfan, Holt Oram, Kruzon, Nunan's syndromes, etc.), autosomno-recessive syndromes (Kartagener, Carpenter, Roberts's syndrome, Gurler, etc.) or the syndromes linked to the H-chromosome (Goltts's syndromes, to Aaza, Gunyotera, etc.).
Among the damaging factors of the external environment viral diseases of the pregnant woman, the ionizing radiation, some medicines, addictions of mother, production harm lead to development of congenital heart diseases. The critical period of an adverse effect on a fruit are the first 3 months of a bereyomennost when there is a fetalny organogenesis.
Pre-natal defeat of a fruit a rubella virus most often causes a triad of anomalies – glaucoma or a cataract, deafness, congenital heart diseases (Fallo's tetrad, a transposition of the main vessels, an open arterial channel, the general arterial trunk, valvate defects, a stenosis of a pulmonary artery, DMZhP, etc.). Also usually the mikrotsefaliya, violation of development of bones of a skull and skeleton, lag in intellectual and physical development take place.
Except a rubella of the pregnant woman, danger to a fruit in the development plan for congenital heart diseases is constituted by chicken pox, simple herpes, adenoviral infections, serumal hepatitis, a cytomegalic inclusion disease, mycoplasmosis, toxoplasmosis, listeriosis, syphilis, tuberculosis, etc.
The structure of an embriofetalny alcoholic syndrome usually includes defects of an interventricular and mezhpredserdny partition, an open arterial channel. It is proved that teratogenny action on cardiovascular system of a fruit accords the welcome of amphetamines leading to a transposition of the main vessels and DMZhP; the anticonvulsive means causing development of a stenosis of an aorta and pulmonary artery, a koarktation of an aorta, an open arterial channel, Fallo's tetrad, hypoplasia of the left departments of heart; the medicines of lithium leading to an atresia of the three-leaved valve, Ebstein's anomaly, DMPP; the progestagen causing Fallo's tetrad, other difficult congenital heart diseases.
At the women having prediabetes or diabetes, children with congenital heart diseases are born more often than at healthy mothers. In this case at a fruit DMZhP or a transposition of large vessels are usually formed. The probability of the birth of the child with congenital heart disease at the woman with rheumatism makes 25%.
Except immediate causes, mark out risk factors of formation of anomalies of heart at a fruit. Refer age of the pregnant woman to them 15-17 years are younger and 40 years, toxicoses of the I trimester, threat of spontaneous termination of pregnancy, endocrine violations at mother, still birth cases in the anamnesis, existence in a family of other children and close relatives with congenital heart diseases are more senior.
Classification of congenital heart diseases
There are several options of classifications of congenital heart diseases which basis the principle of change of haemo dynamics is. Taking into account influence of defect on a pulmonary blood-groove allocate:
- congenital heart diseases with not changed (or slightly changed) a blood-groove in a small circle of blood circulation: atresia of the aortal valve, aorta stenosis, insufficiency of the pulmonary valve, mitralny defects (insufficiency and stenosis of the valve), koarktation of an aorta of adult type, trekhpredserdny heart, etc.
- congenital heart diseases with the increased blood-groove in lungs: not leading to development of early cyanosis (an open arterial channel, DMPP, DMZhP, aortolegochny fistula, a koarktation of an aorta of children's type, Lyutambashe's syndrome) leading to development of cyanosis (an atresia of the three-leaved valve with big DMZhP, an open arterial channel with pulmonary hypertensia)
- congenital heart diseases with the grown poor blood-groove in lungs: not leading to development of cyanosis (the isolated stenosis of a pulmonary artery), leading to development of cyanosis (difficult heart diseases – Fallo's disease, a hypoplasia of the right ventricle, Ebstein's anomaly)
- the combined congenital heart diseases at which anatomic relationship between large vessels and various departments of heart is broken: a transposition of the main arteries, the general arterial trunk, Taussig-Bing's anomaly, otkhozhdeny aortas and a pulmonary trunk from one ventricle and so forth.
In practical cardiology division of congenital heart diseases into 3 groups is used: defects of "blue" (tsianotichesky) type with the venoarterialny shunt (Fallo's triad, Fallo's tetrad, a transposition of the main vessels, an atresia of the three-leaved valve); defects of "pale" type with arteriovenozny dumping (septalny defects, an open arterial channel); defects with an obstacle in a way of emission of blood from ventricles (stenoses of an aorta and pulmonary artery, an aorta koarktation).
Violations of haemo dynamics at congenital heart diseases
As a result above the called reasons at the developing fruit the correct formation of structures of heart can be broken that is expressed in incomplete or untimely closing of membranes between ventricles and auricles, the wrong formation of valves, insufficient turn of primary warm tube and an underdevelopment of ventricles, an abnormal arrangement of vessels etc. After the birth a part of children has open arterial channel and an oval window which in the pre-natal period function in a physiological order.
In view of features of antenatalny haemo dynamics, blood circulation of the developing fruit at congenital heart diseases, as a rule, does not suffer. Congenital heart diseases are shown at children right after the birth or through some time that depends on terms of closing of the message between big and small circles of blood circulation, expressiveness of pulmonary hypertensia, pressure in system of a pulmonary artery, the direction and volume of dumping of blood, individual adaptation and compensatory opportunities of an organism of the child. Quite often the respiratory infection or any other disease leads to development of gross violations of haemo dynamics at congenital heart diseases.
At congenital heart diseases of pale type with arteriovenozny dumping owing to a gipervolemiya hypertensia of a small circle of blood circulation develops; at defects of blue type with the venoarterialny shunt at patients the gipoksemiya takes place.
About 50% of children with big dumping of blood into a small circle of blood circulation perish without the cardiac help on the first year of life from the phenomena of heart failure. At the children who stepped this critical boundary, dumping of blood into a small circle decreases, the health is stabilized, however sclerous processes in vessels of lungs gradually progress, causing pulmonary hypertensia.
At tsianotichesky congenital heart diseases venous dumping of blood or its mixture leads to an overload big and gipovolemiya of a small circle of blood circulation, causing decrease in saturation of blood oxygen (gipoksemiya) and emergence of cyanosis of skin and mucous. For improvement of ventilation and perfusion of bodies the collateral network of blood circulation therefore, despite the expressed violations of haemo dynamics, the condition of the patient can remain a long time satisfactory develops. In process of exhaustion of compensatory mechanisms, owing to long hyperfunction of a myocardium, heavy irreversible dystrophic changes in a cardiac muscle develop. At tsianotichesky congenital heart diseases surgery is shown already at early children's age.
Symptoms of congenital heart diseases
Clinical manifestations and the course of congenital heart diseases is defined by a type of anomaly, the nature of violations of haemo dynamics and terms of development of a decompensation of blood circulation.
At newborns with tsianotichesky congenital heart diseases cyanosis (cyanosis) of integuments and mucous membranes is noted. Cyanosis amplifies at the slightest tension: sucking, crying of the child. White heart diseases are shown by a skin pobledneniye, a cold snap of extremities.
Children with congenital heart diseases usually uneasy, refuse a breast, quickly are tired in the course of feeding. They have a perspiration, tachycardia, arrhythmias, short wind, swelling and a pulsation of vessels of a neck. At chronic violation of blood circulation children lag behind in addition of weight, growth and physical development. At congenital heart diseases usually at once the field of the birth warm noise are listened. Further symptoms of heart failure are found (hypostases, a kardiomegaliya, a cardiogenic hypotrophy, a gepatomegaliya, etc.).
The bacterial endocarditis, politsitemiya, thromboses of peripheral vessels and a thrombembolia of vessels of a brain, stagnant pneumonia, sinkopalny states, odyshechno-tsianotichesky attacks, a stenokarditichesky syndrome or a myocardial infarction can become complications of congenital heart diseases.
Diagnosis of congenital heart diseases
Detection of congenital heart diseases is carried out by comprehensive examination. At survey of the child note coloring of integuments: existence or absence of cyanosis, its character (peripheral, generalized). At a heart auskultation change (easing, strengthening or splitting) of warm tones, existence of noise quite often comes to light and so forth Fizikalny inspection at suspicion of congenital heart disease is supplemented with tool diagnostics - an electrocardiography (ECG), a phonocardiography (FKG), a X-ray analysis of bodies of a thorax, an echocardiography ().
The ECG allows to reveal a hypertrophy of various departments of heart, a pathological deviation of EOS, existence of arrhythmias and violations of conductivity that in total with data of other methods of clinical inspection allows to judge weight of congenital heart disease. By means of daily holterovsky ECG monitoring the hidden violations of a rhythm and conductivity are found. By means of FKG character, duration and localization of warm tones and noise is more carefully and in details estimated. Data of a X-ray analysis of bodies of a thorax supplement the previous methods at the expense of assessment of a condition of a small circle of blood circulation, an arrangement, a form and the sizes of heart, changes from other bodies (lungs, a pleura, a backbone). When carrying out EhoKG anatomic defects of partitions and valves of heart, an arrangement of the main vessels are visualized, sokratitelny ability of a myocardium is estimated.
At difficult congenital heart diseases, and also the accompanying pulmonary hypertensia, for the purpose of exact anatomic and haemo dynamic diagnostics, there is a need for performance of sounding of cavities of heart and angiocardiography.
Treatment of congenital heart diseases
The most complex problem in children's cardiology is surgical treatment of congenital heart diseases at children of the first year of life. The majority of operations at early children's age is carried out concerning tsianotichesky congenital heart diseases. At absence at the newborn of symptoms of heart failure, moderate expressiveness of cyanosis operation can be postponed. Observation of children with congenital heart diseases is carried out by the cardiologist and the heart surgeon.
Specific treatment in each case depends on a version and severity of congenital heart disease. Operations at congenital defects of partitions of heart (DMZhP, DMPP) can include plasticity or ushivany partitions, rentgenendovaskulyarny occlusion of defect. In the presence of the expressed gipoksemiya to children with congenital heart diseases the first stage carries out the palliative intervention assuming different imposing of intersystem anastomoz. Similar tactics improves blood oxygenation, reduces risk of complications, allows to carry out radical correction in more favorable conditions. At aortal defects the resection or balloon dilatation of a koarktation of an aorta, plasticity of an aortal stenosis, etc. is carried out. At OAP its bandaging is made. Treatment of a stenosis of a pulmonary artery consists in carrying out an open or endovascular valvuloplastika etc.
Anatomic difficult congenital heart diseases at which radical operation is not represented possible demand performance of haemo dynamic correction, i.e. division of arterial and venous streams of blood without elimination of anatomic defect. In these cases Fonten's operations, Senninga, Mastarda, etc. can be performed. The serious defects which are not giving in to expeditious treatment demand carrying out heart transplantation.
Conservative treatment of congenital heart diseases can include symptomatic therapy of odyshechno-tsianotichesky attacks, sharp left ventricular insufficiency (cardiac asthma, hypostasis of lungs), chronic heart failure, ischemia of a myocardium, arrhythmias.
Forecast and prevention of congenital heart diseases
Congenital heart diseases win first place in structure of mortality of newborns. Without rendering the qualified cardiac help within the first year of life 50-75% of children perish. In compensation period (2-3 years) mortality decreases to 5%. Earlier identification and correction of congenital heart disease allows to improve the forecast significantly.
Prevention of congenital heart diseases demands careful planning of pregnancy, an exception of impact of adverse factors on a fruit, carrying out medico-genetic consultation and explanatory work among women of risk groups on the birth of children with cardiac pathology, solutions of a question of prenatal diagnostics of defect (ultrasonography, a biopsy of a horion, amniocentesis) and indications to termination of pregnancy. Conducting pregnancy at women with congenital heart diseases requires special attention from the obstetrician-gynecologist and the cardiologist.