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Tetralogy of Fallot is a rare congenital (present at birth) heart defect caused by a combination of four heart defects. These defects, which affect the heart's structure, cause oxygen-poor blood to flow out of the heart and to the rest of the body.Infants with tetralogy of Fallot usually have bluish skin because their blood doesn't have enough oxygen.
The four defects that make up the tetralogy of Fallot include:
Tetralogy of Fallot occurs when the baby's heart is developing in the mother's womb. It is usually diagnosed after birth, during infancy. Sometimes it is not diagnosed until adulthood if the defects and symptoms are not severe.
Symptoms depend on how obstructed the blood flow is into the lungs. Signs and symptoms may include:
Sometimes, babies will suddenly develop deep blue skin, nails, and lips after crying or feeding or when agitated. These episodes are called "tet spells." They are caused by a rapid drop in the amount of oxygen in the blood. If your baby becomes suddenly blue (cyanotic), lay him or her on their side and pull their knees up to his or her chest to increase blood flow to the lungs. Get emergency medical care immediately.
In most cases, the cause of tetralogy of Fallot is unknown.
Factors that might increase the risk include:
A doctor might suspect tetralogy of Fallot if they notice bluish skin or a heart murmur (an abnormal whooshing sound) when listening to the heartbeat. A cardiologist (heart doctor) will conduct a physical examination and use several tests to diagnose.
Tests may include:
In some cases, your child may need medicine to maintain blood flow from the heart to the lungs before surgery. However, surgery is the most effective treatment for tetralogy of Fallot.
Most babies and older children have a type of surgery called intracardiac repair. This open-heart surgery is usually done during the first year after birth and involves several repairs. Adults might undergo this procedure if they didn't have surgical repair as children.
The surgeon places a patch over the VSD to close the hole between the heart's lower chambers (ventricles). They also repair or replace the narrowed pulmonary valve and widen the pulmonary arteries. Because the right ventricle won't need to work as hard, the right ventricle wall will go back to a normal thickness. The oxygen level in the blood will increase, and symptoms will lessen.
Arrhythmias are common after repair and may be treated with medications, a procedure to treat the arrhythmias (ablation), or a special pacemaker device that treats life-threatening heart rhythms (implantable cardioverter-defibrillator).
While most babies and adults do well after intracardiac repair, there can be long-term complications. Infants, children, or adults with these complications might require additional surgeries. Some complications include:
Most adults with repaired tetralogy of Fallot may require another procedure or intervention during their lifetimes. It's very important to have regular follow-ups with a cardiologist trained in caring for people with congenital heart disease.
Sometimes, antibiotics are recommended during dental procedures to prevent infections that might cause endocarditis—an inflammation of the heart's lining. Antibiotics are especially important for those who have had prior endocarditis, have artificial valves, or have had repair with prosthetic material. Ask the doctor what's right for you or your child.
Presbyterian’s Pediatric and Congenital Cardiology team has many different options to help you manage your or your child’s heart condition. The team performs various diagnostic tests and procedures to help form an accurate diagnosis and create individualized treatment plans. Depending on the type of heart condition your child has and its underlying cause, the team can recommend a wide variety of treatment options. Our pediatric cardiologists, pediatric interventional cardiologists, and pediatric cardiovascular surgeons work closely together for cases in which cardiac repair or surgery is the best treatment option.