Tetralogy of Fallot (ToF) is group of birth defects in the heart. The heart has 4 sections, or chambers. There are two upper chambers each called atria, and the two lower chambers are called ventricles. The heart muscle squeezes to push blood through these 4 chambers to the lungs and the rest of the body. Blood flows from the right atrium into the right ventricle, and the right ventricle pumps it to the lungs. As it passes through the lungs, the blood picks up oxygen and leaves behind carbon dioxide. Then the blood flows back to the heart and into the left atrium, and from there into the left ventricle. The left ventricle pumps the blood out to the rest of the body, with a small amount going to the heart muscle itself. The heart pushes blood out through the arteries and blood returns to the heart through the veins.
The heart has 4 valves that open and close with each heartbeat to keep the blood flowing in the right direction through the heart. They are made of thin tissues that open and close easily. The valves are closed while the chambers fill with blood and then they open to push blood out.
ToF means that the heart has these 4 problems:
These 4 problems mean that not enough blood can get to the lungs to get oxygen. The oxygen-poor blood is pumped to the rest of the body.
The cause of this birth defect is not known. It is more common in babies with Down syndrome.
Children with this birth defect often have a blue color of their skin, lips, and fingernails. The blueness may appear at birth or soon after. Babies may have spells during which they turn blue, breathe very fast, and cry. They may pass out.
Older children often get short of breath during activities and may tire easily. They may squat during an activity to help them catch their breath. These symptoms happen because the children are not getting enough oxygen. Not enough blood is flowing to the lungs to pick up the oxygen their body needs.
Your healthcare provider will ask about your child's symptoms and medical history and examine your child. Children with this defect usually have a heart murmur, which your healthcare provider can hear with a stethoscope. A heart murmur is a whooshing sound as blood moves through the heart.
Tests may include:
The problem is treated with surgery. If your baby is too sick or there are other problems, a surgeon can place a small tube, called a shunt, that lets more blood enter the lungs and pick up oxygen. Then more surgery can be done at a later time to correct the defect.
After surgery, most children have few limits on their activities. Some may need to take medicine to control their heart rate and to help their heart pump better. Later in life, they may have other heart problems.
Your child may need to have regular follow-up visits with a specialist in congenital heart disease. Follow your child’s healthcare provider's instructions. Ask your provider:
Make sure you know when your child should come back for a checkup. Keep all appointments for provider visits or tests.