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Classification of congenital heart disease
Last reviewed: 04.07.2025
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There are several classifications of congenital heart defects: the classification of heart diseases in children (WHO, 1970) with the SNOP codes (systematic nomenclature of pathology) used in the USA and with the ISO codes of the International Society of Cardiology; the classification of congenital heart and vascular defects (WHO, 1976), containing a section on "Congenital anomalies (malformations)" with the headings "Anomalies of the bulbus cordis and anomalies of closure of the cardiac septum", "Other congenital anomalies of the heart", "Other congenital anomalies of the circulatory system".
The creation of a unified classification of congenital heart defects is associated with certain difficulties due to the huge number of types of defects and principles that can be used as its basis. The A.N. Bakulev Scientific Research Center for Cardiovascular Surgery has developed a classification in which congenital heart defects are distributed taking into account anatomical features and hemodynamic disorders. The proposed classification is convenient for use in the practical activities of a doctor. In this classification, all defects are divided into three groups.
- Congenital heart defects of the pale type with arteriovenous shunt, i.e. with blood flow from left to right: interventricular septal defect, atrial septal defect, patent ductus arteriosus (Botallo's duct).
- Congenital heart defects of the blue type with venoarterial shunt, i.e. with shunting of blood from right to left: complete transposition of the great vessels, tetralogy of Fallot.
- Congenital heart defects without shunting, but with an obstacle to the ejection of blood from the ventricles (stenosis of the pulmonary artery and aorta, coarctation of the aorta).
There are also congenital heart defects that do not fall into any of the three groups presented in terms of their hemodynamic characteristics. These are defects without blood shunting and without stenosis: congenital heart valve insufficiency, anomaly of the development of the Ebstein tricuspid valve, corrected transposition of the great vessels. Common malformations of the coronary vessels include abnormal origin of the left coronary artery from the pulmonary artery and some others.
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