Living With Tetralogy of Fallot

The outlook for a child born with tetralogy of Fallot is much better today than in the past. Advances in testing and treatment mean that most children who have this congenital heart defect survive to adulthood. However, they need long-term care from specialists to stay as healthy as possible.

Caring for Your Child at Home

Feeding and Nutrition

Babies who have tetralogy of Fallot can tire while nursing or feeding. Small, frequent meals may be easier for your baby to handle.

Your child also may need extra nutrition. A supplement or an extra feeding can give the baby more calories, vitamins, or iron. Your child's doctors will work with you to determine what extra nutrition your baby needs.

Tet Spells

“Tet spells” can occur in babies whose tetralogy of Fallot hasn’t yet been repaired. Lowering your baby's anxiety or stress can help prevent tet spells and save the baby's energy. For example, slowly picking up your baby and speaking in a soothing voice can avoid startling him or her, which may prevent or reduce crying.

Talk to your doctor about how you can manage your child’s tet spells. Your doctor may suggest that you:

• Bring the child's knees up tight against his or her chest (this is called the knee–chest position) or have your child squat down. This will increase blood flow to the lungs.
• Try to calm your child.
• Call 9–1–1 if the symptoms don't improve right away.

Activity Restrictions

Some children who have tetralogy of Fallot may need to limit certain types of physical activity. The limits vary with each child. Talk to your child’s doctor about whether:

• Your child needs to restrict activity or exercise
• Your child can play in organized sports, especially contact sports
• You need a note for your child’s school or coaches about limiting your child's exercise

Ongoing Medical Care

Children who have tetralogy of Fallot should have ongoing medical care. This includes:

• Seeing a pediatric cardiologist for heart checkups as directed
• Seeing a pediatrician or family health care provider for routine exams
• Making sure your child takes medicines as prescribed

Children who have severe heart defects, like tetralogy of Fallot, may be at slightly increased risk for infective endocarditis (IE). IE is a serious infection of the inner lining of your heart chambers and valves.

In a few situations, your child's doctor or dentist may give your child antibiotics before medical or dental procedures (such as surgery or dental cleanings) that could allow bacteria into the bloodstream. Your child’s doctor will tell you whether your child needs to take antibiotics before such procedures.

To reduce the risk of IE, gently brush your young child’s teeth every day as soon as they begin to come in. As your child gets older, make sure he or she brushes every day and sees a dentist regularly. Talk with your child’s doctor and dentist about how to keep your child’s mouth and teeth healthy.

Consider having your child wear a medical alert bracelet or necklace. This alerts anyone caring for your child that the child has tetralogy of Fallot.

You may want to work with your health care providers to put together a packet of medical records and information that covers all aspects of your child's heart defect, including:

• Diagnosis
• Procedures or surgeries
• Prescribed medicines
• Recommendations about medical followup and how to prevent complications
• Health insurance

Keeping your health insurance current is important. For example, if you plan to change jobs, find out whether your new health insurance will cover care for your child's congenital heart defect.

Some health insurance plans may not cover medical conditions that were covered under a previous plan.

Special Needs for Teenagers and Adults

As children who have tetralogy of Fallot grow up and become teens, it's important that they understand the condition, how it was treated, and what kind of care may still be needed.

This understanding will help them take responsibility for their health. It also will help ensure a smooth transition when they start getting care from a cardiologist instead of a pediatric cardiologist. A cardiologist treats adults who have heart problems.

It's also very important for teens to have health insurance as adulthood approaches. Review your current health insurance plan. Find out whether you can extend coverage to your child beyond the age of 18. Some policies may allow you to keep your child on your plan if he or she remains in school or is disabled.

Some teenagers or young adults need additional surgery. For example, the pulmonary valve can narrow again over time, reducing blood flow. The valve may need to be widened or replaced. The cardiologist will discuss with you and your teenager the need for any additional heart surgeries.

Over time, people who have had surgery to repair tetralogy of Fallot also may face a number of other heart problems.

Leaking Heart Valves

The heart has four valves that open and close with each heartbeat. These valves ensure that blood flows in only one direction.

If a valve doesn't seal tightly, blood can leak back into the chamber it came from. This is called backflow or regurgitation (re-GUR-ji-TA-shun), and it can lead to symptoms and complications.

The most frequent problem that occurs after tetralogy of Fallot repair is pulmonary backflow, or leaking from the pulmonary valve. Backflow from the tricuspid valve and aortic valve also can occur. Surgery is needed to repair or replace the leaking valve.

Arrhythmias

Arrhythmias (ah-RITH-me-ahs) are another complication that may develop. Arrhythmias are problems with the rate or rhythm of the heartbeat.

Arrhythmias linked to tetralogy of Fallot include ventricular tachycardia, atrial fibrillation, and atrial flutter. For more information, see “Types of Arrhythmia.” 

Medicines are used to control these arrhythmias. Medical procedures or surgery also may be needed to treat arrhythmias.

Pulmonary Artery Branch Stenoses

Over time, the pulmonary valve can narrow again. This will reduce blood flow to the lungs, making the heart work harder than it should. Several surgical techniques can be used to fix this problem.

Right Ventricular Aneurysms

The patch used to fix the ventricular septal defect (VSD) can cause areas of the ventricle to weaken. The areas, called aneurysms (AN-u-risms), can bulge or “balloon” out.

Aneurysms make it hard for the heart to function as well as it should. This problem must be repaired with surgery.

Residual Ventricular Septal Defects

Sometimes, VSDs still leak even after they’ve been repaired. VSDs are repaired again if they’re large or are causing problems with the function of the right ventricle.

Coronary Heart Disease

As people who have repaired tetralogy of Fallot approach middle age, they can develop coronary heart disease (CHD), just as adults who don’t have heart defects can.

CHD, also called coronary artery disease, is a condition in which a fatty substance called plaque (plak) builds up in the coronary (heart) arteries. CHD can lead to chest pain, shortness of breath, and heart attack.

Preventing CHD is especially important because the procedures done to relieve CHD, like coronary artery bypass grafting, can cause complications in people who have repaired tetralogy of Fallot.

Other Considerations

Many women with repaired tetralogy of Fallot who become pregnant are able to have successful, full-term pregnancies. Others may have difficult pregnancies.

Women with tetralogy of Fallot who want to become pregnant (or who are pregnant) should talk to their doctors about:

• Health risks during pregnancy
• Medicines they can take during pregnancy
• Any new or worsening symptoms

These women also should consult specialists who take care of pregnant women who have heart conditions, such as congenital heart defects.

Adults who were born with tetralogy of Fallot should consider job changes carefully, because health benefits may change. Some health plans have waiting periods or clauses to exclude some types of coverage. Before making any job changes, find out whether the change will affect your health insurance.

Several laws protect the employment rights of people who have health conditions, such as congenital heart defects. The Americans with Disabilities Act and the Work Incentives Improvement Act try to ensure fairness in hiring for all people, including those who have health conditions.