Congenital heart defects

Congenital heart disease is one of the most frequent anomalies of development, occupying the third place after anomalies of the central nervous system and the musculoskeletal system. The birth rate of children with congenital heart disease in all countries of the world ranges from 2.4 to 14.2 per 1,000 newborns. The incidence of congenital heart defects among live births is 0.7-1.2 per 1000 newborns. Flaws with the same frequency of occurrence are often represented differently in the nosological structure among patients entering the cardiological departments (for example, a small defect of the interatrial septum and the tetralogy of Fallot). This is due to the varying degrees of threat to the health or life of the child.

The problems of diagnosis and treatment of congenital heart defects are extremely important in pediatric cardiology. Therapists and cardiologists, as a rule, are not sufficiently familiar with this pathology due to the fact that the overwhelming majority of children by the age of maturity undergo surgical treatment or die without receiving timely adequate care. The causes of congenital heart disease are unclear. The most vulnerable period is 3-7 weeks of gestation, that is, the time when the heart structures are laid and formed. Of great importance are teratogenic factors of the environment, diseases of the mother and father, infections, especially viral, as well as alcoholism of parents, drug use, mother's smoking. Associated with congenital heart defects are many chromosomal diseases.

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 a relatively favorable outcome: an open arterial duct, an interventricular septal defect (AVM), an interatrial septal defect (ASD), pulmonary artery stenosis; With these defects, the natural mortality rate in the first year of life is 8-11%;
• tetralogy of Fallot, 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 the manifestation of the defect (the appearance of clinical signs of hemodynamic disorders).
2. The presence of other (extracardiac) malformations, increasing mortality in a third of children with CHD to 90%.
3. Body weight at birth and prematurity.
4. Age of the child at the time of correction of the blemish.
5. Severity and degree of hemodynamic changes, in particular - the degree of pulmonary hypertension.
6. Type and variant of cardiosurgical intervention.

[1], [2], [3], [4], [5]

The natural course of congenital heart disease

Without surgical treatment, congenital heart defects proceed in different ways. For example, in children at the age of 2-3 weeks, left heart heart failure syndrome or pulmonary atresia (with an intact atrial septal artery) are rarely noted, which is associated with high early mortality in such malformations. The total mortality in congenital heart disease is high. By the end of the first week, 29% of newborns die, by the first month - 42%, by the first year - 87% of children. Taking into account the modern possibilities of cardiosurgical care, it is possible to carry out the operation to a newborn in almost all congenital heart defects. However, not all children with congenital heart defects need surgical treatment immediately after revealing the pathology. The operation is not indicated for minor anatomical disorders (in 23% of children with suspected UPU changes in the heart are transient) or in severe non-cardiac pathology.

Taking into account the tactics of treatment, patients with congenital heart diseases are divided into three groups:

1. patients for whom surgery for congenital heart disease is necessary and possible (about 52%);
2. patients who are not shown surgery due to insignificant hemodynamic disorders (about 31%);
3. patients for whom correction of congenital heart diseases is impossible, as well as inoperable in somatic condition (about 17%).

Before the doctor, who suspected congenital heart disease, there are the following tasks:

• identification of symptoms that indicate the presence of UPU;
• conducting differential diagnosis with other diseases that have similar clinical manifestations;
• the decision of a question on necessity of urgent consultation of the expert (the cardiologist, the cardiosurgeon);
• pathogenetic therapy.

There are more than 90 variants of congenital heart diseases and many of their combinations.

Symptoms of congenital heart disease

When interviewing parents, it is necessary to clarify the timing of the child's static functions: when he began to sit on his own in the crib, walk. It is necessary to find out how the child was gaining weight in the first year of life, since heart failure and hypoxia accompanying heart defects are accompanied by increased fatigue, "lazy" sucking and poor weight gain. Defects with hypervolemia of the small circle of blood circulation can be accompanied by frequent pneumonia and bronchitis. If suspicion of vice with cyanosis should be clarified the timing of the appearance of cyanosis (from birth or during the first half of life), under what circumstances there is cyanosis, its localization. In addition, for vices with cyanosis there is always polycythemia, which can be accompanied by disorders of the central nervous system, such as hyperthermia, hemiparesis, paralysis.

Body type

Changing the physique happens with few vices. So, coarctation of the aorta is accompanied by the formation of an "athletic" build, with the predominance of the development of the shoulder girdle. In the majority of cases, congenital heart defects are characterized by a reduced diet, often up to 1 degree of hypotrophy, and / or hypostature.

It is possible to form such symptoms as "drum sticks" and "watch glass", which is characteristic of congenital heart defects of the blue type.

[6], [7], [8], [9], [10]

Skin covers

With vaginal palsy - pallor of the skin, with vices with cyanosis - diffuse cyanosis of the skin and visible mucous membranes, with a predominance of acrocyanosis. However, the rich "crimson" color of terminal phalanges is also characteristic of high pulmonary hypertension accompanying vices with left-right discharge of blood.

Respiratory system

Changes in the respiratory system often reflect the state of increased pulmonary blood flow and manifest in the early stages of dyspnea, signs of dyspnea.

The cardiovascular system

On examination, the "hump" is located, located bisternally or on the left. When palpation - the presence of systolic or diastolic tremor, a pathological heartbeat. Percutally - change the boundaries of relative dullness of the heart. When auscultation - in which phase of the cardiac cycle is heard the noise, its duration (what part of the systole, diastole takes), the variability of noise when changing the position of the body, the conductivity of noise.

Changes in blood pressure (BP) with CHD are infrequent. So, coarctation of the aorta is characterized by an increase in blood pressure on the hands and a significant lowering on the legs. However, such changes in blood pressure can also occur with vascular pathology, in particular, with nonspecific aortoarteriitis. In the latter case, a significant asymmetry of BP on the right and left arm, on the right and left leg, is possible. Decrease in blood pressure can be with vices with pronounced hypovolemia, for example, with aortic stenosis.

Digestive system

With CHD, an increase in the liver, spleen due to venous congestion with heart failure (usually no more than 1.5-2 cm) is possible. Venous plethora of the vessels of the mesentery, esophagus may be accompanied by vomiting, more often with physical exertion, and abdominal pain (due to the dilatation of the liver capsule).

[11], [12], [13], [14], [15], [16], [17], [18], [19], [20]

Classification of congenital heart disease

There are several classifications of congenital heart defects.

International classification of diseases of the 10th revision. Congenital heart diseases belong to section Q20-Q28. Classification of heart diseases in children (WHO, 1970) with SNOP (a systematic nomenclature of pathology) used in the US, and with the ISC codes of the International Society of Cardiology.

Classification of congenital heart and vascular defects (WHO, 1976), containing the section "Congenital malformations (malformations)" with the headings "Heart bulb anomalies and cardiac wall anomaly", "Other congenital heart anomalies", "Other congenital abnormalities of the circulatory system".

The creation of a single classification presents certain difficulties in connection with a large number of congenital heart disease types, as well as with the difference in principles that can be used as a basis for classification. In the Scientific Center of Cardiovascular Surgery. A.N. Bakuleva developed a classification in which congenital heart diseases are divided into groups, taking into account 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 discharge of blood from left to right (DMZHP, DMP, open arterial duct);
2. A blue-type IPN with a veno-arterial vent, i.e. With discharge of blood from right to left (complete transposition of the main vessels, tetralogy of Fallot);
3. CHD without a discharge, but with an obstruction to ventricular ejection (pulmonary artery stenosis, aortic coarctation).

There are still congenital heart defects that do not enter their hemodynamic characteristics into any of the three groups listed. These are heart defects without a discharge of blood and without stenosis. Among them, in particular, include: congenital insufficiency of the mitral and tricuspid valves, the anomaly of development of the Ebstein tricuspid valve, corrected transposition of the main vessels. To the common malformations of coronary vessels is also the abnormal departure of the left coronary artery from the pulmonary artery.

[21], [22], [23], [24], [25]

Diagnosis of congenital heart disease

In the diagnosis of congenital heart defects, all methods of examination are important: the collection of anamnesis, objective data, functional and roentgenological methods.

[26], [27], [28], [29], [30], [31], [32],

Electrocardiography

ECG is important already at the initial stages of diagnosis of congenital heart defects. All parameters of the standard electrocardiogram are important.

The change in the characteristic of the pacemaker is not typical for congenital heart defects. The frequency of the heart rate almost always increases with congenital heart defects due to hypoxia and hypoxemia. The regularity of the heart rate rarely changes. In particular, cardiac rhythm disturbances are possible with ASD (characterized by extrasystole), with an abnormality of the development of the tricuspid valve of Ebstein (paroxysmal tachycardia attacks).

Deviation of the electric axis of the heart has a certain diagnostic value. To reload the right ventricle, a pathological deviation of the electric axis of the heart to the right is characteristic (DMZHP, DMPP, tetralogy of Fallot, etc.). The pathological deviation of the electrical axis of the heart to the left is typical for an open arterial flow, an incomplete form of atrioventricular communication. Such changes on the ECG may be the first signs that cause suspicion of congenital heart defects.

There may be a change in intraventricular conduction. Some variants of intraventricular blockades occur at certain heart defects. So, for DSA it is typical to have incomplete blockade of the right leg of the bundle, and for the anomaly of the tricuspid valve of Ebstein - the complete blockade of the right leg of the bundle of His.

[33], [34], [35], [36], [37], [38],

X-ray examination

X-ray examination should be carried out in three projections: direct and two oblique. Assess the pulmonary blood flow, the state of the chambers of the heart. This method in the topical diagnosis of congenital heart disease is important in conjunction with other methods of examination.

Echocardiography

Echocardiography (EchoCG) in most cases is the decisive method in the topical diagnosis of such a pathology as congenital heart disease. However, the element of subjectivity should be deleted as far as possible.

[39], [40], [41], [42], [43], [44], [45]

Phonocardiography

Phonocardiography has now lost its diagnostic importance and can only make some clarifications in the auscultation data.

Angiography

Angiography and catheterization of the heart cavities are performed to determine the pressure, saturation of the blood with oxygen, the direction of intracardiac discharges, the type of anatomical and functional disorders.

[46], [47], [48], [49], [50]