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Tetrology Of Fallot Term Paper

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 decide whether your baby needs extra nutrition.

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.

Ask your doctor 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

If your child was born with tetralogy of Fallot, he or she may have some physical activity limits. The limits vary with each child. Ask your child's doctor 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 making sure your child:

  • Sees a pediatric cardiologist for heart checkups as directed
  • Sees a pediatrician or family health care provider for routine exams
  • 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 the heart chambers and valves.

Your child's doctor or dentist may give your child antibiotics before some medical or dental procedures (such as surgery or dental cleanings) that can 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 ID bracelet or necklace. This alerts anyone caring for your child that the child has a heart defect.

Special Needs for Teenagers and Adults

As children who have heart defects grow up and become teens, they should learn how their hearts differ from normal hearts. They also should know what kind of defects they have, how they were treated, and what type of care is still needed. They should be able to recognize signs and symptoms and know how to respond.

Work with your child's health care providers to compile 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

Review your current health insurance plan so you understand your coverage. Keeping your health insurance current is important. If you plan to change jobs, find out whether your new health insurance will cover care for your child's congenital heart defect.

Transition of Care

The move from pediatric care to adult care is an important step in treatment. Talk with your teen's health care team about creating a plan to help your teen transition to adult care. Start planning as soon as your teen is able and willing to fully take part in this process.

Following a transition plan has many benefits. It will help your teen:

  • Get used to talking with health care providers
  • Learn about the adult health care system
  • Understand the importance of having health insurance and learn what his or her insurance covers
  • Take responsibility for his or her medical care

A transition plan also can help your teen think about other important issues, such as future education and employment, birth control and pregnancy planning, and making healthy choices about nutrition, physical activity, and other lifestyle habits.

Additional Surgery and Other Complications

Some teenagers or young adults need additional surgery. For example, the pulmonary valve can narrow again over time, reducing blood flow. A surgeon may need to widen or replace the valve. 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 other heart problems.

Leaking heart valves. The heart has four valves that open and close with each heartbeat. These valves ensure that blood flows only in 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-jih-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 used to repair or replace the leaking valve. In some cases, catheter-based procedures may be used to replace leaking valves.

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

Arrhythmias associated with tetralogy of Fallot include ventricular tachycardia, atrial fibrillation, and atrial flutter. For more information, go to the Health Topics Arrhythmia article.

Doctors use medicines to control these arrhythmias. They also may recommend medical procedures or surgery to treat arrhythmias.

Pulmonary artery branch stenosis. 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 a ventricular septal defect (VSD) can cause areas of the ventricle to weaken. These areas, called aneurysms (AN-u-risms), can bulge or "balloon" out. Aneurysms make it hard for the heart to work as well as it should. Aneurysms are repaired with surgery.

Residual ventricular septal defects. Some 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). (Adults who don't have congenital heart defects also can develop CHD.)

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

For people who have repaired tetralogy of Fallot, preventing CHD is important. The procedures used to treat CHD, such as coronary artery bypass grafting, can cause problems for these people.

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 with their doctors about:

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

If possible, these women 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.

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What causes it?

In most children, the cause of tetralogy of Fallot isn't known. It's a common type of heart defect. It may be seen more commonly in children with Down syndrome or DiGeorge syndrome. Some children can have other heart defects along with tetralogy of Fallot.

How does it affect the heart?

Normally the left side of the heart only pumps blood to the body, and the heart's right side only pumps blood to the lungs. In a child with tetralogy of Fallot, blood can travel across the hole (VSD) from the right pumping chamber (right ventricle) to the left pumping chamber (left ventricle) and out into the body artery (aorta). Obstruction in the pulmonary valve leading from the right ventricle to the lung artery prevents the normal amount of blood from being pumped to the lungs. Sometimes the pulmonary valve is completely obstructed (pulmonary atresia).

How does tetralogy of Fallot affect my child?

Infants and young children with unrepaired tetralogy of Fallot are often blue (cyanotic). The reason is that some oxygen-poor blood is pumped to the body through the hole in the wall between the right and left ventricle instead of being pumped to the lungs.

What can be done about tetralogy of Fallot?

Tetralogy of Fallot is treated surgically. A temporary operation may be done at first if the baby is small or if there are other problems. Complete repair comes later. Sometimes the first operation is a complete repair.

Temporary Operation

In some infants, a shunt operation may be done first to provide adequate blood flow to the lungs. This is not open-heart surgery and doesn't fix the inside of the heart. The shunt is usually a small tube of synthetic material sewn between a body artery (or the aorta) and the pulmonary artery. The shunt is closed when a complete repair is done later.



 

Complete Repair

Complete repair tends to be done early in life. The surgeon closes the ventricular septal defect with a patch and opens the right ventricular outflow tract by removing some thickened muscle below the pulmonary valve, repairing or removing the obstructed pulmonary valve and, if needed, enlarging the branch pulmonary arteries that go to each lung.

Sometimes a tube is placed between the right ventricle and the pulmonary artery. This is sometimes called a Rastelli repair. It's similar to the type of repair used for some other heart defects.

Will my child's activities be limited?

Your child may need to limit physical activity, particularly for competitive sports, if there is leftover obstruction or leak in the pulmonary valve, which is common after repair. Children with decreased heart function or rhythm disturbances may need to limit their activity more.

If the tetralogy has been repaired with surgery, and there's no obstruction or leak in the pulmonary valve, your child may be able to participate in normal activities without much increased risk.

Your child's pediatric cardiologist will help decide if your child needs limits on physical activity.

What will my child need in the future?

If your child has had tetralogy of Fallot repaired, he or she will need regular follow-up with a pediatric cardiologist. As an adult, your child will need lifelong regular follow-up with a cardiologist who's had special training in congenital heart defects.

Some long-term problems can include leftover or worsening obstruction between the right pumping chamber and the lung arteries. Children with repaired tetralogy of Fallot have a higher risk of heart rhythm disturbances called arrhythmias. Sometimes these may cause dizziness or fainting.

Generally, the long-term outlook is good, but some children may need medicines, heart catheterization or even more surgery.

What about preventing endocarditis?

Children with tetralogy of Fallot are at increased risk for endocarditis. Some children, including those have had a valve replacement, still have a shunt or have leaks around surgical patches, and need to take antibiotics before certain dental procedures to help prevent endocarditis. See the section on Endocarditis for more information.

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Congenital Heart Defect ID Card

Tetralogía de Fallot (TOF)
Soplo normal o funcional del corazón

What causes it?

In most cases, the cause isn't known although in some patients, genetic factors play a role. It's a common type of heart defect. It may be seen more commonly in patients with Down syndrome (in association with AV canal defects) or DiGeorge syndrome. Some patients can have other heart defects along with tetralogy of Fallot.

How does it affect the heart?

Normally the left side of the heart only pumps blood to the body, and the heart's right side only pumps blood to the lungs. In a patient with tetralogy of Fallot, blood can travel across the hole (VSD) from the right pumping chamber (right ventricle) to the left pumping chamber (left ventricle) and out into the body artery (aorta). Obstruction in the pulmonary valve leading from the right ventricle to the lung artery prevents the normal amount of blood from being pumped to the lungs. Sometimes the pulmonary valve is completely obstructed (pulmonary atresia).

How does tetralogy of Fallot affect me?

Most patients are diagnosed with tetralogy of Fallot as infants or young children. Patients with unrepaired tetralogy of Fallot are usually blue (cyanotic). This is true in infants, children and adults with unrepaired tetralogy of Fallot. Most adults with tetralogy of Fallot have had it repaired in childhood. Many people are symptom free but may have residual or recurrent problems. These include valve leakage of blood into the heart's right side, blockage of blood leaving the heart's right side and heart rhythm problems. Some patients with these problems may have limited exercise tolerance and may require medicines, repeat operations and/or a special pacemaker to lower the risk of heart rhythm problems.

What can be done about tetralogy of Fallot?

Tetralogy of Fallot is treated with two kinds of surgery. One provides temporary improvement by a shunt to give more blood flow to the lungs. The other is a complete repair of the two most important abnormalities that make up tetralogy of Fallot. Patients might have one or both surgeries in their lifetime.

Shunt Operation

In some patients, a shunt operation may be done first to provide more blood flow to the lungs. This is not open-heart surgery and doesn't fix the inside of the heart. The shunt is usually a small tube of synthetic material sewn between a body artery (or the aorta) and the pulmonary artery. The shunt is removed when a complete intracardiac repair is done later. This is usually done in babies to allow them grow big enough to have a full repair but is occasionally done in adults if a complete repair isn't an immediate option. Some adults had shunts as children, but never got a complete repair. Those patients may still be able to get a complete repair, even as adults.

Complete Repair

Complete repair tends to be done early in life, but in some cases it can be done in adulthood. The surgeon closes the ventricular septal defect with a patch and relieves the obstruction to blood flow going to the lungs. This may be done by removing some thickened muscle below the pulmonary valve, repairing or removing the obstructed pulmonary valve and, if needed, enlarging the pulmonary artery branches that go to each lung. Sometimes a tube (conduit) with a valve in it is placed between the right ventricle and the pulmonary artery. This is sometimes called a Rastelli repair. It's similar to the type of repair used for some other heart defects.

Repeat Operations

The surgery to open up the pulmonary valve often leads to a leaky pulmonary valve. Although this is better tolerated than the original abnormality, the leaky valve can eventually cause problems. There also can be obstructed pathways that are left behind or develop as the patient grows. Both leaky valves and obstructed pathways can cause problems for adult patients with tetralogy of Fallot. In many cases, another surgery to replace the pulmonary valve may be needed in adolescence or adulthood.

Ongoing Care:

Medical

If you've had tetralogy of Fallot repaired, you'll need regular follow-up with a cardiologist who's had special training in congenital heart defects. You may need to take medicine before or after your operation to help your heart muscle contract or to control heart rhythm abnormalities. Your cardiologist will follow your progress with various tests. These include electrocardiograms, Holter monitors, exercise stress tests, echocardiograms and cardiac MRIs. This will help determine if you need another procedure, such as a cardiac catheterization or more surgery. You should also consult a cardiologist with expertise in caring for adults with congenital heart disease if you're undergoing any type of non-heart surgery or invasive procedure.

Activity Restrictions

If tetralogy of Fallot has been repaired with surgery, and there's no obstruction or leak in the pulmonary valve, you may be able to participate in normal activities without much increased risk. You may need to limit your activity if there is leftover obstruction or a pulmonary valve leak, which is common after repair. This limitation may be especially necessary for competitive sports. Patients with decreased heart function or rhythm abnormalities may need to limit their activity more. Your cardiologist will help decide if you need limits. See the Physical Activity section for more information.

What will I need in the future?

If you've had tetralogy of Fallot repaired, you'll need regular lifelong regular follow-up with a cardiologist who's had special training in congenital heart defects.

Some long-term problems can include leftover or worsening obstruction between the right pumping chamber and the lung arteries. Another problem can be a leaky pulmonary valve and enlargement of the heart's right side. Patients with repaired tetralogy of Fallot have a higher risk of heart rhythm disturbances called arrhythmias. Sometimes these may cause dizziness or fainting.

Generally, the long-term outlook is good, but some patients may need medicines, heart catheterization or even more surgery.

Endocarditis Prevention

People with tetralogy of Fallot are at increased risk for developing endocarditis. People with unrepaired and partially repaired tetralogy will need antibiotics to prevent endocarditis before certain dental procedures. If your tetralogy of Fallot has been repaired, your cardiologist will let you know if you need to continue to receive these routine antibiotics. See the section on Endocarditis for more information.

Problems You May Have:

Heart Function

In the long-term postoperative period, your heart muscle function may decrease and you'll need medication. This usually reflects weakening function of the right side of the heart, but in rare instances decreased function of the left ventricle may also occur. Medicines that may be needed include diuretics (water pills), drugs to help your heart pump better and drugs to control your blood pressure.

Heart Rhythm Disturbances

People with repaired tetralogy of Fallot have a higher risk of heart rhythm disturbances called arrhythmias. These can originate from the atria or the ventricles. Sometimes they may cause dizziness or fainting. You may need medicine to control them. Sometimes patients also need blood thinners to reduce the risk of stroke related to the arrhythmias. In rare cases, you may need a procedure in the cardiac catheterization laboratory or the operating room to eliminate these arrhythmias and control symptoms. See the Arrhythmias section for more information. Some patients need pacemakers or implantable defibrillators to treat their arrhythmias.

Pregnancy

In many cases, women with repaired tetralogy of Fallot have successful, full-term pregnancies. Some woman with significant residual heart problems may be at risk for a difficult pregnancy. See the section on Pregnancy for more information. Once tetralogy of Fallot has been repaired, the pregnancy risk is low unless the woman has some side effects or residual effect like irregular heart rhythms or persistent narrowing of the pulmonary valve. Any woman considering pregnancy should have a complete physical examination by her cardiologist in consultation with a multi-disciplinary team before becoming pregnant to find out what, if any risk there might be with the pregnancy. Pregnancy is considered to be high-risk and not recommended for women with unoperated tetralogy of Fallot who remain "cyanotic" (blue).

Will I need more surgery?

After the first complete repair, residual problems may require you to have more open-heart surgeries or procedures in the cardiac catheterization laboratory. In some cases, a procedure done in the cardiac catheterization laboratory using a balloon-tipped catheter to dilate and/or place an expandable stent in narrowed areas may be needed.

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