Congenital Heart Defects

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Topic Overview

What are congenital heart defects?

Congenital heart defects are problems with how a baby's heart forms. "Congenital" means that the heart problem develops before the baby is born or at birth.

Most congenital heart defects affect how blood flows through the heart or through the blood vessels near the heart. Some defects may cause blood to flow in a pattern that isn't normal. Others can completely or partially block blood flow.

There are many different types of congenital heart defects. They can be fairly simple, such as a hole between the chambers of the heart or a heart valve that has not formed right. Others are more serious and complex, such as a missing heart valve or heart chamber.

Some defects are discovered in the fetus (developing baby) while a woman is pregnant. Others are not found until birth. Still others may not be discovered until your child gets older or even until he or she is an adult.

No matter when a heart defect is discovered, having a child with a heart problem is very stressful. Dealing with the fear and uncertainty may seem overwhelming, especially when you have a fragile newborn. It may help you to learn as much as you can about your child's treatment and to talk to your doctor and other parents who have a child with similar problems.

What causes the defects?

No one knows exactly what causes most congenital heart defects. Genes passed down from a parent are a possible cause. Viral infections also may play a role. For example, if a woman gets German measles (rubella) while she is pregnant, it may cause problems with how her baby's heart develops. Women who have diabetes have a greater chance of having a child with a congenital heart defect.

Congenital heart defects are more common in babies who are born with genetic conditions such as Down syndrome.

Taking some prescription or other medicines during pregnancy may cause congenital heart defects. Women who use illegal "street" drugs or who drink alcohol during pregnancy have a higher risk of having a baby with a congenital heart defect.

What are the symptoms?

Symptoms of congenital heart defects will depend on what problem your baby has. Babies with congenital heart defects may have one or more of these symptoms:

  • Tiring quickly
  • Difficulty breathing
  • Puffiness or swelling
  • Sweating easily
  • Not gaining weight as they should
  • A bluish tint to the skin, lips, and fingernails that becomes worse while eating or crying
  • Fainting or near-fainting spells, especially related to physical activity
  • Having dark, strong-smelling urine or other signs of dehydration

In some cases, your child's congenital heart defect may be so mild that symptoms won't appear until the child is a teenager or young adult.

How are congenital heart defects diagnosed?

In most cases, congenital heart defects are found at birth or during a baby's first few months.

You may find that your baby has trouble eating or isn't gaining weight. Or your doctor may hear abnormal sounds or murmurs in your baby's heart during a routine checkup. The first sign may be a bluish tint to the baby's skin.

After a doctor suspects a heart defect, your baby will probably need several tests, such as blood tests, an echocardiogram, and possibly a heart catheterization. The doctor may use the echocardiogram to check blood flow through your baby's heart and to look at the valves, thickness, and shape of the heart. A heart catheterization measures blood pressure in the heart and heart arteries and can show how well the heart is pumping.

Having your child go through this testing can be very scary. Don't be afraid to ask as many questions as you need to ask to feel comfortable. Talk to your doctor and the nurses. And talk to the people who are doing the testing.

How are they treated?

Some defects get better on their own and may not need treatment. Your baby's or child's treatment will depend on the type of defect.

Medicines may be used to help the heart work better. Medicines may also treat symptoms until the defect is repaired.

Some defects can be fixed by using a thin, flexible tube called a catheter. This procedure doesn't require opening up the chest. The doctor threads the catheter through a blood vessel-typically in the groin-and into the heart. There, he or she uses the catheter to close holes or open narrowed blood vessels or valves.

If a baby has a large or complex defect, the baby may need one or more open-heart surgeries. The surgery may be done right away, done over several steps, or delayed until the baby is stronger. Sometimes surgery is delayed if the baby is premature or until the baby is strong enough to handle the surgery. In some cases, the child may need different types of surgery over time as he or she grows.

In rare cases, a heart defect may be so serious that a heart transplant is needed.

Frequently Asked Questions

Learning about congenital heart defects:

Being diagnosed:

Getting treatment:

Ongoing concerns:

Living with congenital heart defects:

Cause

The exact cause of most congenital heart defects is not known. But doctors do know that certain things increase the risk that a baby will have a heart defect. See What Increases Your Risk.

Symptoms

Congenital heart defects cause a wide range of symptoms. Your baby may have only mild symptoms and tire easily, for example. He or she may have life-threatening symptoms, such as severe difficulty breathing. Or your baby may not have any symptoms that you notice at birth but may have them later as he or she grows.

Symptoms usually go away after the defect is corrected. A congenital heart defect that is repaired at the right time is less likely to permanently affect your child's growth and development.

Common symptoms include:

  • Difficult or rapid breathing. You may notice trouble breathing when your baby is active, such as when he or she feeds or cries. Your baby may also breathe fast during rest or activities.
  • Poor weight gain. When most of a baby's energy is spent pumping blood to the body, little is left for eating and growing. Your baby may tire when eating and may take longer than expected to finish feeding.
  • Sweating, especially on the head. You may notice that your baby has damp hair and cool, moist skin.
  • Fatigue and fussiness. Your baby may be too tired to play and may sleep most of the time.
  • Sudden weight gain or puffiness and swelling of the skin, seen most often around the eyes and in the hands and feet. These symptoms may be most noticeable when your baby first wakes up. The weight gain or puffiness can be caused by fluid retention that is related to poor blood circulation.
  • Dehydration. Signs of dehydration include having dry mouth and eyes and having dark and strong-smelling urine.

Symptoms of blood flow problems

Blood flow problems caused by heart defects can mean that your baby gets less oxygen. This happens mostly in children who have cyanotic heart defects ("blue babies").

If a baby has trouble getting oxygen or the heart is working extra hard, symptoms include:

  • A bluish tint (cyanosis) to the skin, lips, and nail beds. This becomes worse when your baby cries or eats.
  • Slower-than-expected growth and development (with more severe congenital heart defects). Your baby may weigh less, be shorter, and take longer than expected to learn skills such as standing and walking.

What Happens

Congenital heart defects happen when the heart doesn't form normally as the developing baby (fetus) grows in the uterus. Heart defects may cause problems with blood flow through the heart after a baby is born. The problems can affect the baby's blood and oxygen supply.

There are many types of congenital heart defects. If the defect lowers the amount of oxygen in the body, it is called cyanotic. If the defect doesn't affect oxygen in the body, it is called acyanotic. Some defects require treatment right away. Other defects get better on their own and don't require treatment.

Only about one-third of full-term babies born with a congenital heart defect have major defects that need surgery or have defects that may cause death during the first year of life.footnote 1

Not all defects are found when a child is very young. Some defects don't cause symptoms and aren't life-threatening. These defects may not be found until the teen years or later.

Some children have a shorter life span than average if their defect is severe or if heart failure or other complications happen.

Long-term problems

Many children and adults with corrected heart defects lead healthy lives. But the treatments they've had or the defect itself can cause or be related to long-term problems. These include:

  • Developmental delays or disabilities or behavior problems.
  • Certain physical traits, such as smaller-than-average adult height and weight, clubbing, or cyanosis (bluish tint to the skin from low blood-oxygen levels). These can present challenges to a person's self-esteem and confidence.

Adults

If you are an adult with a congenital heart defect, see the topic Congenital Heart Defects in Adults.

You may have to make decisions about things like:

  • Pregnancy.
  • Birth control.
  • Type of employment.

What Increases Your Risk

In most cases, the cause of a congenital heart defect isn't known. But certain things increase your baby's chances of having a heart defect. These include:

  • Family history. A child's risk for having a congenital heart defect increases if a brother, sister, or parent has one.
  • Other genetic conditions. For example, Down syndrome has been linked to these defects.
  • Premature birth. Babies born too early have a higher chance of having a heart defect.
  • Chronic conditions. Babies born to women with diabetes or phenylketonuria have a higher chance of having a heart defect.
  • Use of alcohol or drugs during the pregnancy. When a woman drinks alcohol or takes certain drugs while she is pregnant, her baby may develop a heart defect.

If you're concerned about having a baby with a congenital heart defect, you can take steps to lower your risk. For healthy pregnancy choices, see the topic Pregnancy.

When To Call a Doctor

Call 911 or other emergency services immediately if your child has severe difficulty breathing, faints, or has seizures.

Call your doctor immediately if your child with a congenital heart defect has:

  • Symptoms of heart failure or cyanosis-the bluish tint that affects skin, lips, and nails because of lack of oxygen-that become significantly worse within a short time period.
  • Symptoms of endocarditis, such as a fever that won't go away.
  • Symptoms of dehydration, such as sunken eyes with few tears, a dry mouth with little or no spit, and little or no urine for 6 hours.

Talk to your doctor if your child with a congenital heart defect has:

  • Moderate difficulty breathing.
  • A poor appetite and isn't eating well, or sweats while eating, or has a rapid heartbeat or rapid breathing while eating.
  • Less energy or seems to be sleeping more than usual.
  • Sudden weight gain or isn't gaining weight.

Who to see

The following health professionals can evaluate symptoms of a congenital heart defect:

To prepare for your appointment, see the topic Making the Most of Your Appointment.

Exams and Tests

Testing a baby for congenital heart defects can be done while a woman is pregnant or after a baby's birth. Children and adults who have a heart defect need regular checkups throughout their lives.

Tests during pregnancy

A fetal echocardiogram, which can be done as early as 16 weeks of pregnancy, is the best test before a baby's birth. The test uses sound waves to take pictures of the baby's heart. The fetal echocardiogram may be done if:

  • The mother or father has a congenital heart defect.
  • During her pregnancy the mother has been exposed to certain chemicals.
  • During her pregnancy the mother has taken medicines that may increase the risk that her baby (fetus) will develop heart defects.
  • Other exams or tests show signs that a baby may have a problem related to the heart's structure.

Tests after your baby is born

Many congenital heart defects are found in the first month after a baby is born. Your doctor may ask about the baby's symptoms, appetite, and other habits. He or she may do other tests to find a heart problem, such as:

More tests may be needed, depending on the symptoms and type of defect. These may include:

  • Chest X-ray, to check the size and blood vessels of the heart.
  • Electrocardiogram (ECG, EKG), to check for irregular heart rhythms (arrhythmias) that may be related to a congenital heart defect.
  • Blood tests, to check for anemia, polycythemia, or levels of certain chemicals in the blood.
  • Oximetry, to see whether oxygen-poor blood is being pumped to the body. The amount of oxygen in the blood can also be measured by an arterial blood gas (ABG).
  • CT scan or MRI of the heart and major blood vessels, to view abnormal heart structures and/or blood vessels. During these tests, your child will probably be given medicine to make him or her relax and sleep.

Tests for children and adults

Children, teens, and adults with congenital heart defects need regular checkups. They might have tests to check the health of the heart and to check for complications.

A stress test (exercise electrocardiogram) can be done to check if a child or an adult with a heart defect needs exercise restrictions or to see if exercise performance is changing over time. This test might be done before a child starts school or before a teen or adult starts a new exercise program.

Adults who have congenital heart defects need routine checkups. Be sure you have a primary care physician. You might also need to see your cardiologist regularly, such as once a year.

Early detection

If you are thinking of becoming pregnant and you or your partner has a congenital heart defect, it's best to plan ahead for a pregnancy. Genetic counseling can help you find out your risk of passing a heart defect to your child.

If you are already pregnant and you or your partner has a family history of congenital heart defects, a fetal echocardiogram or genetic testing may be done during pregnancy.

A baby may be checked for congenital heart defects if:

Treatment Overview

Your child's treatment for a congenital heart defect will be based on the type of problem he or she has. Your child's age, size, and general health also are important.

Your child may need:

  • Medicines to help with symptoms. Some medicines can control a heartbeat that isn't regular. Others make the heart stronger until a defect can be fixed. Your child may need some medicines after surgery. To learn more, see Medications.
  • A procedure called heart catheterization to find out details about the heart defect or sometimes to repair the defect. To learn more, see Other Treatment.
  • Surgery to repair the structural defect. If a newborn needs surgery, the surgery may be delayed until the baby is stronger. If the defect threatens the baby's life, surgery will be done right away. To learn more, see Surgery.

Your child will also need regular visits to a pediatric cardiologist.

Coping

Caring for a child who has a heart problem can be hard.

You may need to keep track of medicines and make frequent trips to the doctor. Costs can be high. Try to find support groups and other parents who can help you with the many emotions involved.

What to think about

Some children die from severe congenital heart defects or related complications, such as heart failure. If your baby is born with a severe heart defect, there's a good chance that he or she will survive with treatment. But you must also prepare for the possibility that your child may die. Talk with your doctor about local resources and organizations that can help you manage your emotional and practical struggles when faced with this possibility.

Prevention

Congenital heart defects generally cannot be prevented.

If you or your partner has a congenital heart defect and you are planning to have a baby, see the topic Congenital Heart Defects: Pregnancy.

Women who plan to get pregnant and women who are pregnant can lower their risk of having babies with congenital heart defects by taking steps to have healthy pregnancies. For healthy pregnancy choices, see the topic Pregnancy.

Home Treatment

Congenital heart defects often are repaired with surgery or heart catheterization, but home treatment also plays an important role. Some of the issues you'll face may include:

  • Getting your child to eat enough.
  • Giving medicines.
  • Caring for a child in the hospital.
  • Exercise restrictions.
  • Coping with oxygen problems.
  • Caring for yourself.
  • Dealing with family issues and medical bills.

For more information and help, see Congenital Heart Defects: Caring for Your Child.

Teens and adults who have heart defects may need to think ahead about pregnancy, birth control, employment, and other issues. For help, see Congenital Heart Defects in Adults.

Medications

Medicines often are needed to treat congenital heart defects until the defect can be repaired or corrected. Some children and adults need to take medicine even after the defect is repaired. Children with certain defects that cannot be completely corrected may have to take medicines for a long time.

Treatment with medicines depends on the:

  • Type of defect. Complex cyanotic heart defects usually need treatment with medicines more often than acyanotic heart defects.
  • Size of the defect. Children with large or complex defects are likely to have symptoms and may need medicines to relieve the symptoms.

Medicine choices

Medicines might be used to treat complications, relieve symptoms, or prevent problems. They might not treat the defect itself.

The following are some of the medicines used for heart defects.

To treat complications and relieve symptoms

  • Diuretics lower the amount of extra fluid in the body.
  • Digoxin increases the strength of the heartbeats.
  • Vasodilators widen blood vessels so blood can flow more easily.
  • Antiarrhythmics treat and prevent irregular heartbeats.

To treat a certain defect

To prevent problems

What to think about

Know how to give medicine safely. Your child's heart medicines are very strong and can be dangerous if they aren't given correctly. For help, see the topic Congenital Heart Defects: Caring for Your Child.

Surgery

Surgery is done for more complex defects or when catheterization cannot correct the defect. Or your child might need a combination of surgery and catheterization to fix a defect. The kind of surgery will depend on what defect the child has.

Some congenital heart defects can be completely repaired with one surgery. Defects that are more complex often require several surgeries over time.

Preparing for surgery

Prepare yourself for what to expect at the hospital. It may be shocking to see your newborn or child hooked up to so many machines and tubes.

If your child is older, you can help your child feel more comfortable and secure by preparing him or her for what to expect. Your child's doctor or the hospital staff can help you prepare your child. Encourage your child to ask questions. And let him or her talk to the doctors too.

For more tips, see:

Surgery choices

In general, the types of surgery are:

  • Closing holes or blood vessels that have either formed or not closed. Usually, a patch or stitches are used.
  • Widening arteries.
  • Repairing or replacing valves that are too tight or that leak too much.
  • Returning the aorta or pulmonary arteries to the right position.

In rare cases, a heart transplant may be needed.

The type of surgery depends on the type of defect and the surgeon's preference.

What to think about

If a young baby (for example, newborn to age 3 months) has a life-threatening defect, surgery may be needed right away. For some defects, the best time for surgery is before the child is 2 years old. For other defects, the best time may be between the ages of 2 and 4.

In some cases, surgery may be done when a child is older. Surgery may be delayed if the defect is likely to heal on its own.

Some types of surgery are more invasive and take longer to recover from than others. Even after surgery, your child may still have symptoms such as weakness and a bluish tint (cyanosis) to the skin, lips, and nail beds.

After surgery, it's possible for symptoms to return or for complications to develop later. In these cases, more surgeries also may be needed.

Other Treatment

Heart catheterization may be used to correct certain congenital heart defects. Children typically recover more quickly from a heart catheterization procedure than from a surgery.

With heart catheterization, a doctor threads a thin, flexible tube called a catheter through a blood vessel-typically in the groin-and into the heart. This lets the doctor check the heart and treat a defect.

Other Places To Get Help

Organization

National Heart, Lung, and Blood Institute (U.S.)
www.nhlbi.nih.gov

References

Citations

  1. Brown DW, Fulton DR (2011). Congenital heart disease in children and adolescents. In V Fuster et al., eds., Hurst's The Heart, 13th ed., pp. 1827-1883. New York: McGraw-Hill.

Other Works Consulted

  • Brickner ME (2007). Congenital heart disease. In EJ Topol, ed., Textbook of Cardiovascular Medicine, 3rd ed., pp. 502-536. Philadelphia: Lippincott Williams and Wilkins.
  • Brown DW, Fulton DR (2011). Congenital heart disease in children and adolescents. In V Fuster et al., eds., Hurst's The Heart, 13th ed., pp. 1827-1883. New York: McGraw-Hill.
  • Feltes TF, et al. (2011). Indications for cardiac catheterization and intervention in pediatric cardiac disease: A scientific statement from the American Heart Association. Circulation, 123(22): 2607-2652.
  • Giglia TM, et al. (2013). Prevention and treatment of thrombosis in pediatric and congenital heart disease: A scientific statement from the American Heart Association. Circulation, 128(24): 2622-2703. DOI: 10.1161/01.cir.0000436140.77832.7a. Accessed March 25, 2014.
  • Madriago E, Silberbach M (2010). Heart failure in infants and children. Pediatrics in Review, 31(1): 4-12.
  • Marino BS, et al. (2012). Neurodevelopmental outcomes in children with congenital heart disease: Evaluation and management. A scientific statement from the American Heart Association. Circulation, 126(9): 1143-1172.
  • Sable C, et al. (2011). Best practices in managing transition to adulthood for adolescents with congenital heart disease: The transition process and medical and psychosocial issues: A scientific statement from the American Heart Association. Circulation, 123(13): 1454-1485.
  • Warnes CA, et al. (2008). ACC/AHA 2008 Guidelines for the management of adults with congenital heart disease: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation, 118(23): 2395-2451.
  • Webb GD, et al. (2015) Congenital heart disease. In DL Mann et al., eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 10th ed., vol. 2, pp. 1391-1445. Philadelphia: Saunders.

Credits

ByHealthwise Staff

Primary Medical ReviewerJohn Pope, MD - Pediatrics

Martin J. Gabica, MD - Family Medicine

E. Gregory Thompson, MD - Internal Medicine

Specialist Medical ReviewerLarry A. Latson, MD - Pediatric Cardiology

Current as ofSeptember 21, 2016