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Natural history of congenital heart defects

Alexey Portnov, medical expert
Last reviewed: 20.11.2021

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The natural course of congenital heart disease is different. In children 2-3 weeks of left heart heart failure or pulmonary atresia (with intact interatrial septum) are rare, which is associated with high early mortality in these vices. The total mortality in congenital heart disease is high. By the end of the first week, 29% of newborns die, by the end of the first month - 42%, by the year - 87% of children. With modern possibilities of cardiac surgery, almost all newborns with heart defects can perform the operation. However, not all children with congenital heart defects need operative treatment immediately after identifying a "heart problem". In 23% of children with suspected congenital malformation, changes in the heart have a transitory character or are generally absent, and the cause of the disorder is extracardiac pathology. Some children are not operated because of minor anatomical disorders or, conversely, because of the inability to correct the blemish against a background of severe non-cardiac pathology. To determine the tactics of treatment, all children with congenital heart disease are divided into three groups:

patients who are required to perform an operation for congenital heart disease and carry it out (52%);
patients who are not shown surgery due to minor hemodynamic disorders (about 31% of children);
patients with incontinent congenital heart disease or inoperable in a physical condition (about 17% of children).

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

Establishment of symptoms indicative of congenital malformation.
Conducting differential diagnosis with other diseases, accompanied by a similar clinical picture.
Determination of urgency of specialist consultation (cardiologist, cardiac surgeon).
Conducting according to indications of pathogenetic therapy, most often - treatment of heart failure.

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

Survival in congenital heart diseases is determined by the following factors.

Anatomo-morphological severity, i.e. Type of pathology. There are several prognostic groups:
- congenital heart defects with a relatively favorable outcome - open arterial duct, defects of interventricular and interatrial septum, pulmonary artery stenosis (natural mortality in the first year of life with these defects is 8-11%);
- tetralogy of Fallot (natural mortality is 24-36% in the first year of life);
- complex congenital heart defects - left ventricular hypoplasia, pulmonary atresia, a common arterial trunk (the natural mortality for these vices is from 36-52 to 73-97%).
Age of the patient at the time of the manifestation of the defect (the appearance of clinical signs of hemodynamic disorders).
The presence of other (extracardiac) developmental anomalies (increase the mortality rate in a third of children with congenital heart disease to 90%).
Body weight at birth and prematurity.
Age at the time of correction of the defect, i.e. Severity and degree of hemodynamic changes, in particular the degree of pulmonary hypertension.
Type and variant of cardiosurgical intervention.