Tetralogy of Fallot
Tetralogy of Fallot (TOF) is a group of four congenital heart defects – meaning present from birth – that often occur together in infants and children. These defects change the way blood flows through the heart and lungs and require surgical intervention to repair the heart abnormalities. The experienced pediatric heart team at University Hospitals Rainbow Babies & Children’s offers expert diagnosis and treatment for this rare but serious condition.
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What Is Tetralogy of Fallot?
The four heart defects that make up tetralogy of Fallot are:
- Hypertrophy of the right ventricle: This thickening of the lower right chamber of the heart makes it harder to pump blood through the pulmonary valve.
- Overriding aorta: The aorta, which delivers oxygen-rich blood to the body, is out of place, rising above both the right and left ventricle instead of just the left ventricle, as it does in a normal heart.
- Pulmonary stenosis: This narrowing of the pulmonary valve impedes blood flow from the heart to the lungs.
- Ventricular septal defect (VSD): This defect is characterized by a hole between the left and right ventricles, causing the body restrictions in receiving enough oxygen-rich blood.
In a normal heart, blood travels through the heart to the lungs, where it receives oxygen, then goes back to the heart, which pumps the now oxygen-rich blood to the rest of the body. Tetralogy of Fallot makes it harder to get blood to the lungs, so the body receives blood without enough oxygen.
What Causes Tetralogy of Fallot?
Experts aren’t clear on what causes TOF. Family history can play a role, and it may also be present in children with certain genetic syndromes such as Down syndrome or DiGeorge syndrome. However, in many cases the defects simply occur by chance. The condition occurs in about one in every 2,500 babies born in the U.S. each year.
Tetralogy of Fallot Symptoms
Symptoms of tetralogy of Fallot vary from child to child. The most common is cyanosis, which is when the skin or lips have a bluish color due to a drop in oxygen levels. Other symptoms include:
- Difficult or rapid breathing
- Fainting
- Fatigue
- Fussiness
- Heart murmur
- Trouble feeding or gaining weight
Complications if left untreated can include:
- Blood clots
- Endocarditis (Infection in the lining of the heart and heart valves)
- Arrhythmias (abnormal heart beat)
- Heart failure
Diagnosing Tetralogy of Fallot
TOF is sometimes diagnosed before a baby is born with a fetal echocardiogram. It can also be diagnosed shortly after birth if your child’s provider detects a heart murmur or bluish tint to the skin. In that case, your child may be referred to a pediatric cardiologist for further testing. These tests can include:
Pulse oximetry
Monitors oxygen levels in the blood
Electrocardiogram (ECG)
Records electrical activity in the heart
Echocardiogram (echo)
Uses sound waves to show the internal structure of the heart
Cardiac MRI
Takes a 3-D image of the heart’s abnormalities
Cardiac catheterization
Uses a catheter (a thin, flexible tube) inserted through a vein to view the heart and potentially treat blocked passages
Chest X-ray
Takes an image of the heart and lungs
Because symptoms of TOF are similar to those of other congenital heart defects, it’s important that your child is diagnosed by a pediatric cardiologist in order to receive an accurate diagnosis and receive the necessary treatment.
Recovering from Surgery
Your child will recover from surgery in the state-of-the-art pediatric cardiac intensive care unit (PCICU) at UH Rainbow, where they will be closely monitored around the clock by a team of pediatric cardiac intensive care experts and will have access to other pediatric specialists as needed. As they recover, your child will transition from the PCICU to the pediatric cardiac stepdown unit, where they will receive continuity of care by specially trained staff. Before discharge, our cardiac nursing staff will work with families to set up follow-up appointments, educate them on how to care for their child once they return home, and ensure they have the necessary equipment and supplies.
Treating Tetralogy of Fallot
Tetralogy of Fallot requires surgery to fix. Most children will undergo surgery around 6 months of age, although the exact timing will depend on other factors such as the severity of your child’s symptoms and their overall health.
Temporary shunt: If an infant is not strong enough to undergo a full surgical repair, surgeons may place a temporary shunt between the aorta and pulmonary artery to stabilize blood flow until a permanent repair can take place.
Surgical repair: With a TOF surgical repair, a team of pediatric surgeons will place a patch over the ventricular septal defect, remove thickened tissue from the right ventricle, and widen the pulmonary valve. This repair will allow blood to flow the normal route to the lungs to pick up oxygen.
Procedures to treat tetralogy of Fallot are performed in our state-of-the-art hybrid catheterization and surgical suites. Our facility offers cutting-edge technology, including the most advanced imaging available worldwide and 3-D capabilities. This helps our surgeons better understand and visualize each patient’s unique anatomy while making complex repairs, leading to improved patient safety and outcomes.
Follow-up Care
Following repair, your child will need regular care and monitoring from a pediatric cardiologist. However, most children with TOF will go on to live normal, active lives. Some pediatric patients may require further treatment once they reach adulthood, so continued care with an adult congenital heart expert is recommended.
Because infants who undergo cardiac surgery are at a higher risk of neurodevelopmental delays, UH Rainbow offers complete developmental assessments through our Cardiac Neurodevelopmental Iniatitive (CaNDI). These evaluations can identify issues early and get children access to intervention and therapy that can help their long-term development.