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 the majority of children with this congenital heart defect survive to adulthood. However, they do need long-term care by specialists to stay as healthy as possible.

Babies and Children With Tetralogy of Fallot

Caring for Your Child at Home

Babies with tetralogy of Fallot can tire while nursing or feeding. Small, frequent meals may be easier for your baby to handle. He or she 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 decide what extra nutrition your baby may need.

Lowering your baby's anxiety or stress can help prevent "tet spells" and save the baby's energy. For example, picking up your baby slowly and speaking in a soothing voice can avoid startling him or her, which may prevent or lessen crying.

Tet Spells

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

• Bring the baby's or 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 with tetralogy of Fallot may need to limit certain types of exercise. The limits vary with each child. Talk to your 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 the school or coaches about limiting your child's exercise

Routine Medical Care

Regular medical care for your child is important. This includes:

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

Children with severe heart defects, like tetralogy of Fallot, are at increased risk for bacterial endocarditis. This is a serious infection of the heart valves or lining of the heart. These children may need to take antibiotics before medical and dental procedures (such as surgery or dental cleanings) that could allow bacteria to enter the bloodstream. Talk to your child's doctor about whether your child needs to take antibiotics before such procedures.

Consider having your child wear a medical alert bracelet or necklace. This tells anyone caring for your child that the child has a congenital heart defect.

You may want to work with your health care providers to put together a packet with 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 you or your family member had before joining the new plan.

Special Needs for Teenagers and Adults

As children with tetralogy of Fallot grow up and become teens, it's important that they understand their heart defect, how it was treated, and what kind of care may still be needed. This understanding will help the teen take responsibility for his or her health. It also will help ensure a smooth transition from care by a pediatric cardiologist to care by a specialist in adult congenital heart disease.

It's also very important for teens to have health insurance as adulthood approaches. Review your current health insurance plan. Find out how coverage can be extended 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 over time, reducing blood flow. The valve may need to be widened or replaced. Your 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, such as:

• Leaking heart valves
• Arrhythmias
• Pulmonary artery branch stenoses
• Right ventricular aneurysms
• Residual ventricular septal defects
• Coronary artery disease (CAD)

Leaking Heart Valves

Heart valves make sure that blood flows only in one direction. If the valve doesn't seal tightly, blood can leak back into the chamber it came from. This backward flow of blood is called 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 regurgitation, or leaking from the pulmonary valve. Regurgitation of the tricuspid valve and aortic valve also can occur. Surgery is necessary to repair or replace the leaking valve.

Arrhythmias

Arrhythmias—problems with the speed or rhythm of the heartbeat—also can occur. Arrhythmias linked to tetralogy of Fallot include ventricular tachycardia, atrial fibrillation, and atrial flutter. Ventricular tachycardia is when the ventricles (lower chambers of the heart) beat too fast. Atrial fibrillation is a fast and irregular contraction of the atria (the upper chambers of the heart). Atrial flutter is a fast but regular contraction of the atria.

Medicines are used to control these arrhythmias. In rare cases, a person may need a procedure or surgery to fix the problem.

Pulmonary Artery Branch Stenoses

Over time, the pulmonary blood vessels that were enlarged to repair the tetralogy of Fallot defect 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 patches used when repairing tetralogy of Fallot can create weakened areas in the ventricle that can bulge or “balloon” out. These aneurysms (AN-u-risms) 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, a ventricular septal defect still leaks even after it has been repaired. It's repaired again if it's large or is causing problems with the function of the right ventricle.

Coronary Artery Disease

As people with repaired tetralogy of Fallot approach middle age, they can develop CAD, just as adults without a heart defect can. CAD occurs when coronary arteries become blocked due to the buildup of a material called plaque (plak) on the inside of the blood vessels. This can lead to chest pain, shortness of breath, and sometimes heart attack.

Preventing CAD is especially important because any procedures, like coronary artery bypass grafting, that are done to relieve symptoms of CAD can cause complications in people with 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 doctor about:

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

These women also may want to consult specialists who take care of pregnant women with health conditions.

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 kinds 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 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 with health conditions such as heart defects.