Heart and Vascular Center

Complex (Tetralogy-of-Fallot)

Another type of heart defect is congenital cyanotic heart defects. In these defects, blood pumped to the body contains less oxygen than normal. This causes a condition called cyanosis, a blue discoloration of the skin. Infants with cyanosis are often called "blue babies." An example of cyanotic defects is Tetralogy of Fallot.

Tetralogy of Fallot has four components. The two major ones are a large hole, or ventricular septal defect, that lets blood pass from the right to the left ventricle without going through the lungs; and a narrowing (stenosis) at or just beneath the pulmonary valve. This narrowing partially blocks the blood flow from the heart's right side to the lungs. The other two components are: the right ventricle is more muscular than normal, and the aorta lies directly over the ventricular septal defect.

This results in cyanosis (blueness), which may appear soon after birth, in infancy or later in childhood. These "blue babies" may have sudden episodes of severe cyanosis with rapid breathing. They may even become unconscious. During exercise, older children may become short of breath and faint. These symptoms occur because not enough blood flows to the lungs to supply the child's body with oxygen.

Some infants with severe Tetralogy of Fallot may need an operation to give temporary relief by increasing blood flow to the lungs with a shunt. This is done by making a connection between the aorta and the pulmonary artery. Then some blood from the aorta flows into the lungs to get more oxygen. This reduces the cyanosis and allows the child to grow and develop until the problem can be fixed when they are older.

Most children with Tetralogy of Fallot have open-heart surgery before school age. The operation involves closing the ventricular septal defect and removing the obstructing muscle. After surgery the long-term outlook varies, depending largely on how severe the defects were before surgery. Lifelong medical follow-up is needed.

People with Tetralogy of Fallot, before and after treatment, are at risk for getting an infection within the aorta or the heart valves (endocarditis).