Pronounced: TE-TRAL-o-je of fal-O
Tetralogy of Fallot (TOF) is a congenital (present at birth) heart problem made up of four heart defects that occur together.
TOF is caused by the combination of four heart defects that occur during the growth of the fetus:
A risk factor is something that increases your chance of getting a disease or condition. There are no known risk factors for TOF.
Symptoms may include:
These symptoms usually appear during the first few weeks of life. In mild TOF cases, symptoms may not appear until much later when the child or young adult becomes more active, placing more demand on the heart. Without treatment, symptoms will persist.
In severe cases, a “tetralogy spell” may occur if the oxygen level in the blood drops suddenly. The lips and skin become much more blue. The baby becomes breathless and irritable. If the very low oxygen level persists, the baby may become sleepy or unconscious.
Older children may have shortness of breath and fainting spells when they exercise. When they have a tetralogy spell, they may squat with their knees on their chest to help recover from the breathlessness.
A diagnosis of TOF is often made very soon after birth. The doctor will ask about symptoms and perform a physical exam. A newborn with significant bluish coloring of the skin (cyanosis) is often given extra oxygen. If this does not increase the baby’s oxygen level, a heart defect is suspected. If a baby with TOF does not have blue skin, the first clue is a loud heart murmur.
If a heart defect is suspected, tests may include:
Various medications are often given to relieve symptoms and prevent complications. TOF is primarily treated surgically. Surgical options include:
Small infants with severe TOF may need a temporary operation to provide enough blood flow to their lungs. A passage is made between the aorta, or another major artery, and the pulmonary artery. This increases the amount of oxygen in the blood and allows the child to grow large enough to have a complete repair.
Most children with TOF have open-heart surgery within the first few years of life. The operation involves:
In some cases, a passageway is created between the right ventricle and pulmonary artery.
In many cases, the surgery is successful. However, in some cases further surgery is needed. Long-term follow-up is always needed to detect recurring or new problems.
RESOURCES:
American Heart Association
http://www.americanheart.org
Canadian Adult Congenital Heart Network
http://www.cachnet.org
CANADIAN RESOURCES:
Sick Kids (The Hospital for Sick Children)
http://www.sickkids.ca/
The Heart Defects Society of Windsor and Essex County
http://www.heartdefectssociety.org
References:
American Heart Association website. Available at: http://www.americanheart.org .
Canadian Adult Congenital Heart Network website. Available at: http://www.cachnet.org/ .
The Heart Center Encyclopedia . Cincinnati Children’s Hospital Heart Center; 2000.
Mayo Foundation for Medical Education and Research, Adult Congenital Heart Disease Clinic website. Available at: http://www.mayo.edu/ .
Last reviewed November 2008 by Michael J. Fucci, DO
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
Copyright © 2007 EBSCO Publishing All rights reserved.