The problems of diagnosis and treatment of congenital heart disease are extremely important in pediatric cardiology. As a rule, therapists and cardiologists are not sufficiently familiar with this pathology, as the vast majority of children are treated surgically or die without being adequately cared for at maturity. The causes of congenital heart disease are unclear. The most vulnerable period is 3-7 weeks gestation, that is, the time in which the heart structures are laid and formed. Of great importance are teratogenic factors of the environment, diseases of the mother and the father, infections, especially viral, as well as alcoholism of the parents, drug use, smoking of the mother. Many chromosomal diseases are associated with congenital heart defects.
ICD code 10
Q20. Congenital anomalies (malformations) of the heart chambers and joints.
Factors of survival in congenital heart disease
Anatomo-morphological severity, i.e. type of pathology. The following prognostic groups are distinguished:
- congenital heart defects with relatively favorable outcome: open arterial vein, interventricular septal defect (IVD), septal defect (SD), pulmonary artery stenosis; With these shortcomings, the natural mortality rate in the first year of life is 8-11%;
- Fallot tetralogy, natural mortality in the first year of life - 24-36%;
- complex congenital heart defects: left ventricular hypoplasia, pulmonary atresia, common arterial trunk; natural mortality in the first year of life - from 36-52% to 73-97%.
1. Age of the patient at the time of manifestation of the defect (the onset of clinical signs of hemodynamic disorders).
2. The presence of other (extracardiac) malformations increases mortality in one third of children with CHD to 90%.
3. Body weight at birth and premature birth.
4. Age of the child at the time of correction of the defect.
5. Severity and degree of hemodynamic changes, in particular - the degree of pulmonary hypertension.
6. Type and variant of cardiac surgery intervention.
The natural history of congenital heart disease
Without surgical treatment, congenital heart defects occur in different ways. For example, in children 2-3 weeks hypoplastic left heart syndrome, atresia or pulmonary artery (with intact atrial septum) are rare in age, due to the high early-onset mortality in this vice. Overall mortality from congenital heart disease is high. At the end of the first week, 29% of newborns die in the first month - 42%, in the first year - 87% of children. Taking into account the modern possibilities of cardiosurgical care, it is possible to carry out the operation on a newborn in almost all congenital heart defects. However, not all children with congenital heart disease need surgery immediately after the pathology is revealed.
Taking into account the tactics of treatment, patients with congenital heart disease are divided into three groups:
1. Patients in whom surgery for congenital heart disease is necessary and possible (about 52%);
2. Patients with no surgery due to minor hemodynamic disorders (approximately 31%);
3. Patients in whom correction of congenital heart disease is impossible and inoperable in the somatic state (about 17%).
Before the doctor who conjectures congenital heart defect, there are the following tasks:
Identification of symptoms ;
⦁ Conducting a differential diagnosis with other diseases having similar clinical manifestations;
⦁ the decision of a question on need of urgent advice of the expert (the cardiologist, the cardiologist);
⦁ pathogenetic therapy.
There are more than 90 variants of congenital heart disease and many of their combinations.
Symptoms of a congenital heart disease
When parents are interviewed, it is necessary to clarify the timing of the child's static functions: when he started to sit in the crib himself, he left. It is necessary to find out how the child gains weight in the first year of life, such as congestive heart failure and hypoxia, concomitant heart disease, accompanied by fatigue, "lazy" suction weakness and adding weight. Malignant hypervolaemia defects may be associated with frequent pneumonia and bronchitis. If suspected vices with cyanosis should be clarified the timing of cyanosis (from birth or in the first half of life), under what circumstances it is cyanosis, its localization. In addition, if cyanotic defects are always polycythemia, which can be associated with disorders of the central nervous system.
constitution
Change the physique with few vices. The coarctation of the aorta is thus accompanied by the formation of an "athletic" build, whereby the development of the shoulder girdle predominates. In most cases, congenital heart defects are characterized by a reduced diet, often up to 1 degree hypotrophy and / or hypostasis.
It is possible to form such symptoms as "drum sticks" and "watch glass", which is characteristic of congenital heart defects of the blue type.
skin covers
With vaginal palsy - paleness of the skin, with lice with cyanosis - diffuse cyanosis of the skin and visible mucous membranes, with a predominance of acrocyanosis. However, the rich "crimson" color of the terminal phalanges is also characteristic of the high pulmonary hypertension that accompanies the vices with the left-right discharge of the blood.
respiratory system
Changes in the respiratory system often reflect the state of increased pulmonary blood flow and manifest in the early stages of dyspnoea, signs of dyspnea.
Cardiovascular system
In the investigation is the "hunchback", which is bad or left. If palpation - the presence of systolic or diastolic tremor, a pathological heartbeat. Percutaneously - change the boundaries of the relative dullness of the heart. At auscultation - at what stage of the cardiac cycle is heard the noise, its duration (which part of the systole, diastole), the variability of the noise in changing the position of the body, the conductivity of noise.
Changes in blood pressure (BP) with CHD are rare. Thus, aortic coarctation is characterized by an increase in blood pressure on the hands and a significant lowering of the legs. However, such changes in blood pressure may also occur in vascular pathology, especially in nonspecific aortoarteritis. In the latter case, a significant asymmetry of the BP is possible on the right and left arm, on the right and left leg. Drop in blood pressure may occur in louses with severe hypovolemia, such as aortic stenosis.
The digestive system
In CHD, an increase in liver, spleen and venous stasis with heart failure (usually not more than 1.5-2 cm) is possible. Venous fullness of the vessels of the mesentery, esophagus may be accompanied by vomiting, more often with physical exertion and abdominal pain (due to the enlargement of the liver capsule).
Classification of congenital heart disease
There are several classifications of congenital heart disease.
International Classification of Diseases of the 10th Revision. Congenital heart disease belongs to section Q20-Q28. Classification of heart disease in children (WHO, 1970) using SNOP (a systematic nomenclature of pathology) in the US, and with the ISC codes of the International Society of Cardiology.
Classification of Congenital Heart Defects and Vessel (WHO, 1976), "Congenital Anomalies (Birth Defects)" section titled "Heart Pear Abnormalities and Closing Anomalies of the Heart Septum", "Other Congenital Heart Defects", with "Other Congenital Anomalies of the Circulatory System."
The creation of a single classification presents certain difficulties associated with a large number of congenital heart failure types, as well as the difference in principles that can be used as a basis for classification. At the scientific center for cardiovascular surgery. AN Bakuleva has developed a classification that classifies congenital heart disease in consideration of anatomical features and hemodynamic disorders. The proposed classification is convenient for use in practical activities. In this classification, all UPUs are divided into three groups:
1. The PPS is pale with an arteriovenous shunt, i.e. with left-to-right blood flow (open arterial canal);
2. A blue-type veno-arterial vent, i.e. with right-to-left blood flow (full rearrangement of the main vessels, Fallot tetralogy);
3. CHD without discharge, but with ventricular ejection obstruction (pulmonary artery stenosis, aortic coarctation).
There are still congenital heart defects that do not bring their hemodynamic properties into any of the three groups listed. These are heart defects without blood leakage and without stenosis. These include in particular: congenital insufficiency of the mitral and tricuspid valves, the anomaly of the development of the Ebstein tricuspid valve, corrected transposition of the main vessels. Common abnormalities of the coronary arteries include the abnormal discharge of the left coronary artery from the pulmonary artery.
Diagnosis of congenital heart defects
In the diagnosis of congenital heart defects, all examination methods are important: medical history, objective data, functional and radiographic methods.
electrocardiography
ECG is important in the early stages of the diagnosis of congenital heart disease. All parameters of the standard electrocardiogram are important.
The change in cardiac pacemaker characteristics is not typical of congenital heart defects. Frequency of heart rate almost always increases with congenital heart disease due to hypoxia and hypoxemia. The regularity of the heart rate seldom changes. In particular, cardiac arrhythmias in ASD (characterized by extrasystole) with an abnormality of development of the tricuspid valve of Ebstein (paroxysmal tachycardia attacks) are possible.
The deviation of the electrical axis of the heart has a certain diagnostic value. In order to reload the right ventricle, a pathological deviation of the electrical axis of the heart to the right is characteristic (Fallot tetralogy, etc.). The pathological deviation of the electrical axis of the heart to the left is typical of an open arterial flow, an incomplete form of atrioventricular communication. Such changes in the ECG may be the first signs of suspected congenital heart disease.
There may be a change in intraventricular conduction. Some variants of intraventricular blockages occur in certain heart defects. For digital subtraction angiography (DSA), it is typical to have an incomplete blockade of the right leg of the bundle and the anomaly of the tricuspid valve of Ebstein - the complete blockade of the right leg of the His bundle.
X-ray
X-ray examination should be carried out in three projections: direct and two oblique. Assess the pulmonary blood flow, the state of the heart chambers. This method in the topical diagnosis of congenital heart disease is important in conjunction with other examination methods.
echocardiography
Echocardiography (EchoCG) is in most cases the crucial method in the topical diagnosis of such pathology as congenital heart disease. However, the element of subjectivity should be deleted as much as possible.
phonocardiography
Phonocardiography has now lost its diagnostic significance and can only make a few clarifications in the auscultation data.
angiography
Angiography and catheterization of the heart cavities are performed to determine the pressure, the saturation of the blood with oxygen, the direction of the intracardiac discharges, the type of anatomical and functional disorders.