Laurie Rosenblum, MPH
Tetralogy of Fallot (TOF) is a combination of 4 heart defects. It is present at birth. These defects make it difficult for your child to get oxygen to the entire body.
The defects are:
VSD and overriding aorta can decrease the amount of oxygen in the blood. This makes it difficult for the body to get the oxygen it needs. Pulmonary stenosis and right ventricular hypertrophy can make it difficult for blood to pass to the lungs. This will also decrease the amount of oxygen in the blood.
A child's heart develops very early in pregnancy. TOF is caused by a problem during this development. It is not known exactly why these problems happen. Some have been associated with genetics, mother's nutrition, or infections. Most have no known cause.
Factors that may increase your child's chance of TOF include:
Some people who have TOF also have a chromosome disorder. This may include
Down syndrome, CHARGE syndrome, and VACTERL association.
For most, symptoms will appear during the first few weeks of life. For children with mild TOF, symptoms may not appear until much later. They may not show until your child or young adult becomes more active. Physical activity places more demand on the heart. A heart with TOF cannot keep up with the extra demand, causing symptoms. Without treatment, symptoms will continue to occur.
Symptoms may include:
In severe cases, a tetralogy spell may occur. This happens when the oxygen level in the blood drops suddenly. Symptoms include:
A diagnosis of TOF is often made soon after birth. The doctor will ask about your child's symptoms and the mother's medical history. A physical exam will be done.
The doctor may begin tests based on your child's symptoms. A newborn with blue skin will often be given extra oxygen. If the oxygen does not help, a heart defect may be suspected. If the skin color is normal, the first clue is a loud heart murmur.
Images may be taken of your child's heart. This can be done with:
Heart problems may also be detected using
Medications may be given to relieve symptoms. They may also help to prevent complications like a tetralogy spell.
The defects of the heart are treated with surgery. Surgical options include:
Small infants with severe TOF may not be strong enough for a complete repair surgery. A temporary operation will help increase the amount of oxygen in the blood. This will give the baby time to grow, get stronger, and prepare for a complete repair.
During this operation, a temporary, new path is made for the blood. The blood will be sent past the problems in the heart and go directly to the lungs. This will increase the amount of blood getting to the lungs. The oxygen in the blood will increase as a result.
Most children with TOF have open-heart surgery within the first few years of life. The operation includes:
For some, a path is created between the heart and the blood vessels to the lungs.
In many cases, the surgery is successful. For some, further surgery may be needed. Long-term follow-up is always needed to detect recurring or new problems.
There are no current guidelines to prevent TOF.
American Heart Association
National Heart, Lung, and Blood Institute
About Kids Health—The Hospital for Sick Children
Canadian Adult Congenital Heart Network
Explore tetralogy of Fallot. National Heart, Lung, and Blood Institute website. Available at:
http://www.nhlbi.nih.gov/health/health-topics/topics/tof. July 1, 2011. Accessed February 12, 2016.
Seki M, Kuwata S, Kurishima C, et al. Report from the Japanese Society of Pediatric Cardiology and Cardiac Surgery Research Committee on Cardiovascular Function in Adult Patients with Congenital Heart Disease: Mechanism of aortic root dilation and cardiovascular function in patients with tetralogy of Fallot. Pediatr Int. 2016 Jan [Epub ahead of print].
Tetralogy of Fallot. American Heart Association website. Available at:
http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Tetralogy-of-Fallot_UCM_307038_Article.jsp#.Vr5Ayk2FMdU. Updated October 26, 2015. Accessed February 12, 2016.
Tetralogy of Fallot in infants and children. EBSCO DynaMed Plus website. Available at:
http://www.dynamed.com/topics/dmp~AN~T115349/Tetralogy-of-Fallot-in-infants-and-children. Updated January 25, 2016. Accessed February 12, 2016.
Tetralogy of Fallot (TOF) in children. Cincinnati Children's Hospital website. Available at:
http://www.cincinnatichildrens.org/health/t/tof. Updated July 2015. Accessed February 12, 2016.
10/1/2013 DynaMed's Systematic Literature Surveillance
http://www.dynamed.com/topics/dmp~AN~T115349/Tetralogy-of-Fallot-in-infants-and-children: Molgaard-Nielsen D, Pasternak B, Hviid A. Use of oral fluconazole during pregnancy and the risk of birth defects. N Engl J Med. 2013;369(9):830-839.
Last reviewed January 2015 by Rimas Lukas, MD
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