Tetralogy of Fallot (ToF) is a heart condition present at birth (congenital heart defect). ToF develops early in pregnancy, when the heart is dividing and forming its pumping chambers. The heart defects of ToF prevent some of the blood from your body from going directly to your lungs for oxygen. This causes the blood entering your heart to mix with the blood leaving your heart. ToF makes it difficult for your body to get enough oxygen-rich blood.
There are 4 defects that cause ToF:
A hole between the right and left side of the heart (VSD).
A missing or underdeveloped valve that allows blood to flow from the right side of the heart to the lungs (pulmonary stenosis).
Excess thickness of the muscles
on the right side of the heart.
The main blood vessel that carries blood away from the heart (aorta) not being in the normal position.
You may continue to have some signs and symptoms of your heart defect as an adult, even if you had surgery to correct the defect as an infant or child. You may be at risk for problems that often affect adults living with congenital heart defects.
CAUSES
The exact cause of ToF is not known. In some cases, it may have been caused by genes passed down through your family.
RISK FACTORS
Babies born with Down syndrome or DiGeorge syndrome may be at higher risk for ToF.
SYMPTOMS
The signs and symptoms you have as an adult will depend on the type of defect you were born with. How successful the surgery to correct the defect was will also affect the symptoms you have. The most common problem for adults with ToF is a leaky valve between the heart and lungs. Problems in other areas of the heart or lungs can also develop. Signs and symptoms of ToF in adulthood may include:
Low energy or fatigue.
Inability to exercise.
An abnormal heart rate (too fast or too slow) or an abnormal heart rhythm.
A feeling that the heart is fluttering.
Dizziness or fainting.
Cough or shortness of breath.
DIAGNOSIS
Have regular checkups with your heart specialist (cardiologist) to make sure you are not developing complications from your heart defect. Possible complications may include:
A leaky valve.
An enlarged aorta.
Heart failure.
Heart rhythm problems (arrythmias).
Heart infections (endocarditis).
Lung problems.
Your cardiologist will ask whether you have any new symptoms and do a physical exam. You may also have tests, such as:
Electrocardiogram (EKG) to check for arrhythmias.
EKG during exercise (stress test).
Echocardiogram to check for heart changes.
Blood tests to check your liver function.
Using a portable type of EKG that you wear over a period of time (Holter or event monitoring).
Having a long flexible tube (catheter) placed into your heart to measure your heart activity and function (cardiac catheterization).
Heart MRI to check for heart changes.
TREATMENT
Your treatment will depend on how well your heart functions now and what changes are likely to develop in the future. Possible treatments may include:
Medicine to control your heart rate or your heart's ability to pump.
Medicine to control your blood pressure.
Blood thinning medications to prevent blood clots from forming in your heart.
Water pills (diuretics).
Medicine to prevent infection (endocarditis) before certain dental procedures.
A pacemaker to regulate your heart rate.
Surgery to repair or replace a leaky heart valve.
A heart transplant if heart failure begins to develop. This is rare.
HOME CARE INSTRUCTIONS
Keep a record of your medical information to share with your health care providers. Make sure you know:
What type of defect you were born with.
What surgieries you had.
What medicines you are taking.
Exercise as directed by your health care provider. You may need to limit strenuous exercise, such as weight lifting or competitive sports.
Eat a heart-healthy diet and maintain a healthy weight. Work with a dietician, if needed.
Do not smoke.
Do not abuse drugs or alcohol.
Ask your health care provider if you need to take antibiotics before certain dental procedures.
If you are a woman who becomes pregnant or wants to become pregnant, you may need special care from a cardiologist and obstetrician who are familiar with managing heart defects during pregnancy. You may be advised to have a blood test to check whether you carry any genes that could cause ToF in your baby.
SEEK MEDICAL CARE IF:
You develop any new symptoms or signs of heart disease, such as:
Feeling dizzy.
Feeling very tired.
Feeling your heart flutter.
Feeling your heart beating too fast or too slow.
Having trouble exercising.
Coughing often.
You are a woman and you become pregnant or you want to become pregnant.
SEEK IMMEDIATE MEDICAL CARE IF:
You have chest pain or trouble breathing.
You faint.