Tetralogy of Fallot Diagnosis and Treatment
Babies with the congenital heart defect tetralogy of Fallot receive specialized care from our expert team.
Babies with tetralogy of Fallot receive expert diagnosis and care from the team in the Johns Hopkins All Children’s Heart Institute in St. Petersburg, Florida.
Our team includes specialized pediatric cardiologists, heart surgeons, fetal heart specialists, cardiac critical care physicians and others to provide your baby with comprehensive, compassionate care from diagnosis and treatment, to follow up care into adulthood.
What is tetralogy of Fallot?
Tetralogy of Fallot is a combination of four congenital heart defects that are all present at birth and affect the structure of the heart and the way blood circulates in the body.
- Ventricular septal defect (VSD): A hole between the lower pumping chambers of the heart.
- Overriding aorta: The aorta (which carries blood from the heart to the rest of the body) lies over the hole between the lower chambers.
- Pulmonary stenosis: The pulmonary valve is narrowed, creating a blockage in blood flow from the right ventricle to the pulmonary artery.
- Right ventricular hypertrophy: The muscle of the walls of the right ventricle (which pumps blood to the lungs to receive oxygen) becomes thickened.
In tetralogy of Fallot, blood flow to the lungs is limited, so the blood flowing to the rest of the body lacks oxygen.
What causes tetralogy of Fallot?
In most cases, what causes a child to have tetralogy of Fallot is unknown. It may be seen more often in children with Down syndrome or DiGeorge syndrome. Some children may have other heart defects along with tetralogy of Fallot.
How is tetralogy of Fallot diagnosed?
Tetralogy of Fallot may be diagnosed before your baby is born with a fetal echocardiogram. A fetal echocardiogram is a type of ultrasound used to take pictures of a baby’s heart to diagnose various types of congenital heart defects. If your primary obstetrician has a concern about your baby’s heart after an ultrasound, you may be referred to a specialist for a fetal echocardiogram.
At Johns Hopkins All Children’s Hospital, our Fetal Heart Program team provides expert diagnosis using fetal echocardiogram as well as management before birth of congenital heart defects like tetralogy of Fallot.
Babies may also be diagnosed with tetralogy of Fallot after birth if doctors note they have abnormal oxygen levels. Additional tests after birth to confirm that your baby has tetralogy of Fallot may include:
- Electrocardiogram: a test that records the electrical activity of your baby’s heart using small sensors placed on the chest
- Echocardiogram: a type of ultrasound that takes moving images of your baby’s heart
- Chest X-ray
- Cardiac MRI: a test that uses a large magnet, radio waves, and a computer to take images of your child’s heart, to evaluate the structure and function of the heart and blood vessels
- Cardiac catheterization: a procedure in which a very thin tube is guided through the blood vessels into the heart, to examine the heart’s structure and function
Symptoms of tetralogy of Fallot
Because tetralogy of Fallot limits blood flow to the lungs, babies with tetralogy of Fallot often have a blueish tint to their skin from lack of oxygen in the blood.
In those babies who are diagnosed after birth, a lower-than-normal oxygen level is usually a primary symptom of tetralogy of Fallot. However, tetralogy of Fallot is a highly variable condition, meaning that it can impact each child to varying degrees. Some babies with tetralogy of Fallot may have normal oxygen levels and may then be diagnosed a few months after birth if your baby’s doctor detects a heart murmur.
Treatment for tetralogy of Fallot involves surgery, and your child’s individual course of treatment will depend on their health and condition, including factors like their oxygen level.
Some babies with a more severe form of the condition may need medication after birth to allow sufficient blood flow to the lungs. Babies with a milder form of the condition may not need medication. Some children may have a complete surgical repair as infants. This involves closing the hole between the heart’s lower pumping chambers (the ventricular septal defect) and repairing the pulmonary valve area.
Other babies may first have a procedure to place a shunt or a stent in the heart to temporarily improve blood flow to the lungs, allowing time for the baby to grow more before completing the full repair surgery, usually around 4-6 months of age.
Whether your child will have a complete repair or will first need a shunt or stent before the repair depends on their individual condition. Your baby’s care team will fully explain your child’s condition and the recommended course of treatment to you and answer any questions you may have throughout your child’s treatment. Learn more about our board-certified pediatric heart surgeons at Johns Hopkins All Children's.
Caring for your baby between procedures
Babies who receive a shunt or stent are monitored by the expert team in our Cardiac High Risk Monitoring Program while they await their repair surgery. The program provides seamless coordination of care and a consistent point of contact for your family during the time between your child’s procedures.
We provide families with education and technology so that they can track their baby’s health and progress between visits, allowing our team to receive real-time updates about their baby’s vital signs, feedings and other important information. Families also have access to the team through a 24-hour dedicated phone line for any questions or concerns that may arise. Learn more about the Cardiac High Risk Monitoring Program.
Your baby will be cared for by the team in the cardiovascular intensive care unit (CVICU) after surgery. The CVICU team includes pediatric cardiologists, heart surgeons, cardiac critical care physicians, respiratory therapists and other pediatric experts who care for our patients after surgery.
After being discharged, your baby will continue to have regular follow-up appointments with their pediatric cardiologist. A patient’s condition over time depends on how severely the disease impacted their pulmonary valve. Children with more severe cases may need additional surgeries in the future to replace the pulmonary valve.
Pediatric patients can also continue to receive care from our experts as adults in the Adult Congenital Heart Disease Program. Adults with congenital heart defects benefit from seeing a provider who understands how congenital heart defects can impact health long term.
For more information or to make an appointment, give us a call at the phone number below. We serve families in the greater Tampa Bay area and beyond.