Natural course of congenital heart disease

The natural history of congenital heart defects varies. In children aged 2-3 weeks, hypoplastic left heart syndrome or pulmonary atresia (with an intact atrial septum) are rare, which is associated with high early mortality in these defects. The overall mortality rate in congenital heart defects 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 the current capabilities of cardiac surgery, almost all newborns with heart defects can undergo surgery. However, not all children with congenital heart defects need surgical treatment immediately after the detection of a "heart problem". In 23% of children with suspected congenital defects, changes in the heart are transient or absent altogether, and the cause of the disorder is extracardiac pathology. Some children are not operated on due to minor anatomical abnormalities or, conversely, due to the impossibility of correcting the defect against the background of severe extracardiac pathology. To determine the treatment tactics, all children with congenital heart defects are divided into three groups:

• patients who need surgery for a congenital heart defect and it can be performed (52%);
• patients for whom surgery is not indicated due to minor hemodynamic disturbances (about 31% of children);
• patients with uncorrectable congenital heart defects or inoperable due to somatic condition (about 17% of children).

A doctor who first suspects a congenital heart defect faces the following tasks.

• Establishing symptoms that indicate a congenital defect.
• Conducting differential diagnostics with other diseases accompanied by a similar clinical picture.
• Determining the urgency of a specialist consultation (cardiologist, cardiac surgeon).
• Conducting pathogenetic therapy according to indications, most often - treatment of heart failure.

There are more than 90 types of congenital heart defects and many combinations of them.

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

• Anatomical and morphological severity, i.e. type of pathology. Several prognostic groups are distinguished:
  • congenital heart defects with a relatively favorable outcome - patent ductus arteriosus, interventricular and interatrial septal defects, pulmonary artery stenosis (natural mortality in the first year of life with these defects is 8-11%);
  • tetralogy of Fallot (natural mortality in the first year of life is 24-36%);
  • complex congenital heart defects - left ventricular hypoplasia, pulmonary atresia, common arterial trunk (natural mortality for these defects ranges from 36-52 to 73-97%).
• The patient's age at the time of manifestation of the defect (the appearance of clinical signs of hemodynamic impairment).
• The presence of other (extracardiac) developmental anomalies (increases mortality in a third of children with congenital heart defects up to 90%).
• Birth weight and prematurity.
• Age at the time of correction of the defect, i.e. the severity and degree of hemodynamic changes, in particular the degree of pulmonary hypertension.
• Type and variant of cardiac surgery.