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About tetralogy of Fallot | Causes | Why a concern? | Symptoms | Diagnosis | Treatment | Postoperative care | Care at home | Long-term outlook | Find a doctor | Locations Tetralogy of Fallot (TOF or “TET”) is a complex condition that is a combination of several congenital (present at birth) heart defects. TOF occurs when the fetal heart develops abnormally during the first eight weeks of pregnancy. “Tetralogy” refers to the fact that four heart problems occur together to cause this rare condition. TET decreases oxygen in the bloodstream, often causing babies to appear blue or become fussy or lethargic. Get a second opinion Tetralogy of Fallot is very treatable, most often requiring surgery before a child’s first birthday. It is a complex heart condition, however, and its symptoms resemble those related to other conditions. Expert diagnosis is necessary to determine how your child’s heart is affected so that treatment can be customized based on your child’s unique needs. Herma Heart Institute’s pediatric cardiologists and heart surgeons have extensive experience in treating tetralogy of Fallot of all types and offer exceptional surgical outcomes for patients. Our program consistently outperforms when it comes to congenital heart surgery outcomes for even the most complex types of heart disease, as evaluated by the Society of Thoracic Surgeons. Learn more about our heart surgery outcomes. Note: Data is provided from the Society of Thoracic Surgeons (STS). New data is not available beyond June 2019, as STS is in the process of refining their reporting methodologies. Hospitals are also limited in providing updated data due to the demands of the COVID-19 pandemic.
Tetralogy of Fallot (TOF or "TET") is a combination of four congenital heart defects, including:
"Tetralogy" refers to four heart problems. The fourth problem is that the right ventricle becomes enlarged as it tries to pump blood past the obstruction into the pulmonary artery. Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped through the aorta out to the body. In tetralogy of fallot, blood flow within the heart varies, and is largely dependent on the size of the ventricular septal defect, and how severe the obstruction in the right ventricle is.
Tetralogy of fallot occurs in about two out of every 10,000 live births. It makes up about 8 percent of all cases of congenital heart disease. Tetralogy of fallot occurs equally in boys and in girls. What causes tetralogy of fallot?Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Maternal abuse of alcohol during pregnancy, leading to fetal alcohol syndrome (FAS), is linked to tetralogy of fallot. Mothers who take medications to control seizures and mothers with phenylketonuria (PKU) are also more likely to have a baby with tetralogy of fallot. Most of the time, this heart defect occurs sporadically (by chance), with no clear reason evident for its development. Why is tetralogy of fallot a concern?The amount of oxygen-poor (blue) blood that passes through the VSD to the left side of the heart varies. If the right ventricle obstruction is severe, or if the pressure in the lungs is high, a large amount of oxygen-poor (blue) blood passes through the VSD, mixes with the oxygen-rich (red) blood in the left ventricle, and is pumped to the body. The more blood that goes through the VSD, the less blood that goes through the pulmonary artery to the lungs, and the less oxygen-rich (red) blood that returns to the right side of the heart. Soon, nearly all the blood in the left ventricle is oxygen-poor (blue). This is an emergency situation, as the body will not have enough oxygen to meet its needs. Some situations, such as crying, increase the pressure in the lungs temporarily, and increasing blueness might be noted as a baby with tetralogy of fallot cries. In other situations, the pathway from the right ventricle to the pulmonary artery becomes tighter, preventing much blood from passing that way, and allowing oxygen-poor (blue) blood to flow through the VSD into the left heart circulation. Both of these situations are nicknamed "TET spells." Sometimes, steps can be taken to lessen the pressure or the obstruction, and allow more blood to flow into the lungs and less through the VSD. These steps, however, are not always effective. Symptoms of tetralogy of fallotThe following are the most common signs and symptoms of tetralogy of fallot. However, each child may experience symptoms differently. Symptoms may include:
Any of these can be symptoms of tetralogy of fallot. The symptoms of tetralogy of fallot may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis. How is tetralogy of fallot diagnosed?Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the obstruction from the right ventricle to the pulmonary artery. Symptoms your child exhibits will also help with the diagnosis. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. However, other tests are needed to help with the diagnosis, and may include the following:
Specific treatment for tetralogy of Fallot will be determined by your child's physician based on:
Tetralogy of Fallot is treated by surgical repair of the defects. A team of cardiac surgeons performs the surgery, usually before an infant is 1 year old. In many cases, the repair is made at around 6 months of age, or even a little earlier. Repairing the heart defects will allow oxygen-poor (blue) blood to travel its normal route through the pulmonary artery to receive oxygen. The operation is performed under general anesthesia, and involves the following:
Postoperative care for your childChildren will spend time in the intensive care unit (ICU) after tetralogy of Fallot repair. During the first several hours after surgery, your child will be very drowsy from the anesthesia that was used during the operation, and from medications given to relax him/her and to help with pain. As time goes by, your child will become more alert. While your child is in the ICU, special equipment will be used to help him/her recover, and may include the following:
Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you. Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child. After being discharged from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while at home, and these will be explained to you. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged. Caring for your child at home following tetralogy of Fallot repairPain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable at home. Your child's physician will discuss pain control before your child is discharged from the hospital. After surgery, older children usually have a fair tolerance for activity. Your child may become tired easily, and sleep more right after surgery, but, within a few weeks, your child should be fully recovered. Long-term outlook after tetralogy of Fallot surgical repairMost children who have had a tetralogy of Fallot surgical repair will live healthy lives. Activity levels, appetite, and growth will eventually return to normal in most children. Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis after discharge from the hospital. Consult your child's physicians regarding the specific outlook for your child.
Find tips for deciding whom to trust with your child’s heart surgery in a blog written by Peter Frommelt, MD, pediatric cardiologist and professor at the Medical College of Wisconsin.
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