Congenital heart disease classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Classification

Acyanotic Defects

Acyanotic heart defects comprise approximately 75% of all congenital heart defects. This contains any heart defect of a group of structural effects. It can be subdivided into two groups depending on whether there is shunting of the blood from the left vasculature to the right (left to right shunt) or no shunting at all.

Left to right shunting heart defects include ventricular septal defect or VSD (30% of all congenital heart defects), persistent ductus arteriosus or PDA, atrial septal defect or ASD, and atrioventricular septal defect or AVSD.

Acyanotic heart defects without shunting include pulmonary stenosis, a narrowing of the pulmonary valve, aortic stenosis and coarctation of the aorta.

Obstruction Defects

Obstruction defects occur when heart valves, arteries, or veins are abnormally narrow or blocked. Common obstruction defects include pulmonary valve stenosis, aortic valve stenosis, and coarctation of the aorta, with other types such as bicuspid aortic valve stenosis and subaortic stenosis being comparatively rare. Any narrowing or blockage can cause heart enlargement or hypertension.

Cyanotic Defects

A cyanotic heart defect is a group-type of congenital heart defects (CHDs). The patient appears blue (cyanotic), due to deoxygenated blood bypassing the lungs and entering the systemic circulation. This can be caused by right-to-left or bidirectional shunting, or malposition of the great arteries. Such defects include persistent truncus arteriosus, total anomalous pulmonary venous connection, tetralogy of Fallot, transposition of the great vessels, and tricuspid atresia.

Septal Defects

The septum is a wall of tissue which separates the left heart from the right heart. It is comparatively common for defects to exist in the interatrial septum or the interventricular septum, allowing blood to flow from the left side of the heart to the right, reducing the heart's efficiency. Ventricular septal defects are collectively the most common type of CHD, although approximately 30% of adults have a type of atrial septal defect called patent foramen ovale. Septal defects may or may not cause cyanosis depending on the severity of the defect.