Commonly Asked Questions About Children and Heart Disease

Updated:Jul 6,2016

Answers from the Pediatric Cardiologist

Atrial Septal Defect

Question:
My 4-year-old daughter was recently diagnosed with a hole in her heart. It is called an atrial septal defect. We were told that it should be closed. I heard that there is a new way to close the hole without having surgery. Can this be done any way other than with surgery? My older son had a patent ductus arteriosus surgically repaired 10 years ago when he was 1 year old. Could his patent ductus arteriosus have been closed by this new method that does not require surgery?

Answer:
An atrial septal defect is a hole in the wall that separates the two upper chambers of the heart known as the right atrium and left atrium. The hole can be located in various locations in the wall (the septum). There are new methods of closing this hole without open-heart surgery, but it depends on the location of the hole. Presently, the only holes that can be closed are known as secundum atrial septal defect and patent foramen ovale (PFO). Both of these types of holes have an adequate rim around the hole onto which the device is attached. The non-surgical method of closure is by implanting a device, which resembles a dumbbell into the hole to "plug it up" from within the heart. This is accomplished by passing a small tube (catheter) from a vein located in the groin and up into the heart. The procedure is performed in the cardiac catheterization laboratory and does not require surgery. Several devices are available and the results have been comparable to surgical results. The risks, which are rare, are much the same as for any cardiac catheterization. They include clot formation, bleeding, infection, perforation of the heart, and arrhythmias (heart rhythm disturbances). For device implantation, device embolization is an additional risk (the device could break free and travel to another part of the heart or blood vessels), which may require surgical retrieval. This is an extremely rare risk because most of the devices are designed for safe repositioning and retrieval in the cath lab if necessary. Should embolization occur, the surgeon would simply repair the defect at the time of the retrieval. Another risk is a residual leak across the device, but usually the leak is sealed in two to six months as the body grows new tissue over the device. The cath procedure would also require transesophageal echocardiography, which is an echocardiogram performed with the probe passed down the throat to measure the size of the defect and assist in determining proper position of the device. The entire procedure is performed under general anesthesia. Keep in mind that you will need to be on aspirin for six months after the procedure. The aspirin is used to keep blood clots from forming on the device while the body heals over it. The risks of surgical closure would include most of the cath lab risks such as bleeding, infection, blood clots, arrhythmia and residual leaks. Other inherent risks involving open-chest and open-heart surgery include the need for blood transfusions, prolonged bed rest, lung collapse and lung infections, and fluid forming within the sac of the heart. Overall, both procedures are effective and have low risk. While surgery will be more painful and require longer recovery time and will result in a scar on the chest, it has a proven track record over many decades. The cath lab method is newer and the long-term results are not yet known but appear to be as good as surgery.
 

The patent ductus arteriosus can also be closed without surgery. It is similar to the ASD closure but different devices are available. The most commonly used device is called a coil, which has FDA approval for use in various unwanted blood vessels in the body. The coil has been in use for nearly 30 years and has proven to be quite safe and effective for the smaller PDA's. Other devices are available for larger PDA's, some of which are still investigational. Except for the very small infant, or the patient with a very large ductus, the preferred method of PDA closure in most pediatric cardiology centers is by cardiac catheterization.

Question:
My 4-year-old daughter was recently diagnosed with a hole in her heart. It is called an atrial septal defect. We were told that it should be closed. I heard that there is a new way to close the hole without having surgery. Can this be done any way other than with surgery? My older son had a patent ductus arteriosus surgically repaired 10 years ago when he was 1 year old. Could his patent ductus arteriosus have been closed by this new method that does not require surgery?

Answer:
An atrial septal defect is a hole in the wall that separates the two upper chambers of the heart known as the right atrium and left atrium. The hole can be located in various locations in the wall (the septum). There are new methods of closing this hole without open-heart surgery, but it depends on the location of the hole. Presently, the only holes that can be closed are known as secundum atrial septal defect and patent foramen ovale (PFO). Both of these types of holes have an adequate rim around the hole onto which the device is attached. The non-surgical method of closure is by implanting a device, which resembles a dumbbell into the hole to "plug it up" from within the heart. This is accomplished by passing a small tube (catheter) from a vein located in the groin and up into the heart. The procedure is performed in the cardiac catheterization laboratory and does not require surgery. Several devices are available and the results have been comparable to surgical results. The risks, which are rare, are much the same as for any cardiac catheterization. They include clot formation, bleeding, infection, perforation of the heart, and arrhythmias (heart rhythm disturbances). For device implantation, device embolization is an additional risk (the device could break free and travel to another part of the heart or blood vessels), which may require surgical retrieval. This is an extremely rare risk because most of the devices are designed for safe repositioning and retrieval in the cath lab if necessary. Should embolization occur, the surgeon would simply repair the defect at the time of the retrieval. Another risk is a residual leak across the device, but usually the leak is sealed in two to six months as the body grows new tissue over the device. The cath procedure would also require transesophageal echocardiography, which is an echocardiogram performed with the probe passed down the throat to measure the size of the defect and assist in determining proper position of the device. The entire procedure is performed under general anesthesia. Keep in mind that you will need to be on aspirin for six months after the procedure. The aspirin is used to keep blood clots from forming on the device while the body heals over it. The risks of surgical closure would include most of the cath lab risks such as bleeding, infection, blood clots, arrhythmia and residual leaks. Other inherent risks involving open-chest and open-heart surgery include the need for blood transfusions, prolonged bed rest, lung collapse and lung infections, and fluid forming within the sac of the heart. Overall, both procedures are effective and have low risk. While surgery will be more painful and require longer recovery time and will result in a scar on the chest, it has a proven track record over many decades. The cath lab method is newer and the long-term results are not yet known but appear to be as good as surgery.
 

The patent ductus arteriosus can also be closed without surgery. It is similar to the ASD closure but different devices are available. The most commonly used device is called a coil, which has FDA approval for use in various unwanted blood vessels in the body. The coil has been in use for nearly 30 years and has proven to be quite safe and effective for the smaller PDA's. Other devices are available for larger PDA's, some of which are still investigational. Except for the very small infant, or the patient with a very large ductus, the preferred method of PDA closure in most pediatric cardiology centers is by cardiac catheterization.

Question:
My 11-year-old son was recently diagnosed with a bicuspid aortic valve. What is this? Can he participate in competitive sports? Will he ever need surgery or medical treatment for this?

Answer:
The term bicuspid aortic valve refers to the leaflets of the aortic valve. This is the valve that leads out of the heart to the aorta, the big blood vessel that delivers oxygen-rich blood to the body. Normally, the aortic valve has three leaflets. A bicuspid valve has only two leaflets.

Because of this, patients with a bicuspid valve may develop a narrowing or leakage of the aortic valve. Narrowing or leakage of aortic valves do not necessarily develop in childhood, but may occur in adulthood. On rare occasions these problems require repair, either with a balloon (cardiac catheterization) or surgery. To determine whether narrowing or leakage is present and related to the bicuspid aortic valve, the cardiologist follows the patient with non-invasive tests that may include: electrocardiograms, echocardiograms and stress tests. If narrowing or leakage of the valve becomes severe enough to require intervention, your doctor will explain this to you in more detail. In many cases, bicuspid aortic valves do not require any intervention in childhood and children with them may not need activity restriction. A pediatric cardiologist will make specific recommendations for activity restrictions for each patient based on the severity of the valve narrowing or leakage. Restrictions may be put in place to assure that a normal amount of oxygen gets to the patient's heart muscle at all times. 

Question:
What is "cardiac asthma"? I thought asthma was caused by allergies. My son has seasonal asthma and takes an inhaler. Does he have a heart problem also?

Answer:
Asthma is a spasm of small air tubes (bronchioles) in the lungs caused by many factors that affect how air passes through the lungs. These include allergies, pollens, pollutants, exercise, stress and lung disease. Patients usually have difficulty breathing associated with wheezing and coughing. Treatment is directed at opening up the small air tubes with inhalers or other oral medications. Most children with asthma have this type of problem. "Cardiac asthma" mimics lung asthma but is much less common. Patients with heart failure or heart valves that don't open properly can also experience wheezing and coughing that is known as cardiac asthma. However, the bronchial spasm in cardiac asthma is caused by back pressure from the left side of the heart to the lungs. Symptoms usually occur with exercise or at night after going to bed. If a child has a congenital heart defect, coughing and wheezing may be symptoms of heart failure and require evaluation by a congenital heart specialist. Special tests such as an echocardiogram or chest x-ray may be required to determine if a child has a congenital heart defect. Treatment is directed at improving the pump function of the heart with medications. If the asthma is caused by a heart valve that is not working properly, or a hole between the heart chambers, surgery or other procedures may be required.

Question:
I had a child with a congenital heart defect; why did this happen? What is the likelihood of my having another child with the same defect or a different defect?

Answer:
Heart defects, especially those occurring as isolated problems in otherwise healthy infants, do not always have readily identifiable genetic or environmental causes. As the circulatory system is one of the first organ systems to begin functioning, blood flow problems occurring in early fetal life can be compounded as the fetus develops.
 

Slightly less than 1 percent of infants born in the United States (about eight per 1,000 live births) have a congenital heart defect. Depending on the defect, the chances of a sibling also being born with the same defect or other heart defect are about 2 percent to 3 percent. While this is higher than the general population rate of 0.8 percent, it is still a relatively small chance. Some genetic defects such as Down Syndrome are associated with heart defects. Several genetic syndromes have heart defects as well as defects in other organ systems. About 25 percent of children with a heart defect have one or more additional abnormalities in another organ. These facts all point to a genetic component.
 

Numerous environmental exposures are also associated with congenital heart defects. These include using some medications for seizures, acne or bipolar psychiatric disorder, or having some diseases such as rubella (German measles), other viral diseases, diabetes or lupus early in pregnancy. Alcohol and street drugs may increase the risk also, as can exposure to chemicals such as certain pesticides and solvents.
 

Most congenital defects are not that common and not likely to occur in future children. Consultation with a genetics counselor to find out more about your particular risk for future pregnancies is recommended. Remaining healthy and avoiding certain exposures is helpful. Fetal echocardiograms (ultrasounds) may help detect heart problems as early as 12–14 weeks of pregnancy.

Question:
My 12-year-old son complains of chest pain when he plays soccer. Is this serious? Do I need to take him to a heart specialist? What is causing his chest pain?

Answer:
We're aware that heart problems can cause chest pain. It is natural to worry when a child or adolescent complains of chest pain that their heart is the cause. Many parents may even wonder if their child might be having a heart attack. 
 

It's important to realize problems that result in heart disease in adults are rare in children. And, though chest pain is pretty common in youngsters, it is very rare that this pain is related in any way to their heart. Most chest pain in children is not serious. Many times the source of the pain cannot even be determined. 
 

Other causes of chest pain common in children include pains from pulled muscles or sports-related injury, pain related to asthma or cough, and pain due to infections like pneumonia and pleurisy. Usually, if the pain is caused by anything serious, there will be other signs. For example, infections usually cause fever, too. 

Your child’s primary care doctor is the best person to speak with if you have concerns about your child’s pain. If your child’s chest pain was associated with a fainting episode, this may be a sign of a serious problem, and you should seek medical attention right away; meanwhile, your child should refrain from sports.

Question:
My granddaughter was born with coarctation. What is coarctation and how is it treated?

Answer:
Coarctation of the aorta is a narrowing of the aorta, the large blood vessel coming out of the heart that provides oxygen-rich blood to the body. Coarctation of the aorta causes the heart to work harder to generate enough pressure to push the blood through the narrowing. This causes high pressure in the left side of the heart, and in the arms, while the legs will have lower blood pressure. In babies with coarctation of the aorta, the heart may fail to generate the high pressure needed, and the child may show signs of heart failure and need urgent intervention. Overall, the long-term outlook for a child born with this defect is excellent, as long as the defect is repaired in a timely manner.
 

Coarctation of the aorta can be fixed with an operation or an interventional catheterization. There are a few different ways in which surgery can be done. The surgeon can cut out the area of the narrowing and sew the two ends back together or use extra tissue (patch) to make the area bigger. Depending on your granddaughter’s age and anatomic details it may be possible to fix the coarctation with a catheterization using a balloon and possibly a stent. The long-term problems to watch for after surgery or catheterization include the possibility that the narrowing would come back in the area of the operation, usually related to scar tissue, requiring another intervention. In most cases, if the narrowing comes back the narrowing can be stretched open with a balloon via cardiac catheterization with a high success rate. A more rare possibility is that the area of the aorta where surgery was done could weaken over time, resulting in dilation (enlargement) and aneurysm (weakening) of the vessel walls. If this happens, she may also need another operation.
 

Your granddaughter’s doctors will be checking her after the operation as she grows, to make sure those problems are not present. Your granddaughter could have high blood pressure in the future. However, when coarctation of the aorta is fixed early in life, this is less likely to become a problem. There is a small risk of endocarditis (heart infection) after repair of coarctation. Some girls with coarctation may have a chromosome problem called Turner Syndrome and these children may need to have medical evaluation for other possible non-heart problems.
 

As long as there is no significant narrowing left in the aorta and her blood pressure is normal, she should be able to participate in sports without limitations and have a normal life.

Question:
My baby turns “blue” when she cries. Does she have a heart problem?

Answer:
It's very common for babies to develop a “blueness” around the mouth when they are crying or if they seem to be holding their breath. It's also common for babies to get blue hands and feet when they're cold. This is a very common observation made by parents and describes a normal condition termed “acrocyanosis.” It’s a normal response in babies and adults to reflect cool fingers and toes. Even normal room temperature may seem cold to an unclothed baby.
 

A physician should be consulted for persistent blueness (even at rest), blueness involving lips or gums, or blueness associated with difficulty breathing or lethargy and poor feeding.

Question:
What is the cause of an enlarged heart in an 11-year-old child? His chief complaints are chest pain and severe coughing and sometimes vomiting.

Answer:
The finding of an enlarged heart in a child or an adolescent who complains of chest pain, cough and some vomiting should prompt an immediate investigation to determine the precise nature of the problem. Several heart conditions can lead to these symptoms in a child who has been previously in good health. Examples include inflammation of the membrane or sac that surrounds the heart (a condition called pericarditis), which is often associated with accumulation of fluid in that sac (called pericardial effusion), and inflammation of the heart muscle itself (a condition called myocarditis that may be caused by a virus). Less common conditions that can manifest with these symptoms and an enlarged heart include weakness of the heart muscle (a condition called dilated cardiomyopathy), rheumatic fever (infrequent in the United States), infection of the heart valves (called endocarditis), and a previously unknown congenital abnormality of the heart or blood vessels. Heart attacks, which can occur in adults, are extremely rare in children and most likely not the cause of chest pain and enlarged heart.
 

Most frequently, the enlarged heart is detected on a chest x-ray, a test that only gives a general idea about the overall size of the heart and blood vessels. The pediatric cardiologist will conduct a thorough investigation of your child, including detailed history, careful physical examination, and an electrocardiogram (ECG). An echocardiogram (cardiac ultrasound) is a key test in evaluating an enlarged heart. This test will provide detailed information about the anatomy, size and function of the cardiac chambers. Sometimes, additional tests, such as cardiac catheterization and heart muscle biopsy, may be required to complete the investigation.

Question:
For the past few months, my daughter has been passing out at school. I have been told that there is nothing to worry about and all I should do is put more salt into her diet. Although she eats lots of pretzels and potato chips, she still keeps passing out. I am afraid! What could this be and can she be treated?

Answer:
Fainting spells are a common problem in children. Most of the time fainting is not a life-threatening problem, but there are some serious causes of fainting that must be ruled out by a specialist in pediatric heart conditions. Assuming that these serious causes of fainting have been ruled out, it sounds like your daughter has simple fainting spells, which has the scientific name of "neurocardiogenic syncope." The important thing to remember is that this condition is not life threatening and it can be treated in many ways. The treatment that you described of increasing salt intake, and/or fluid intake, is usually the first step for treating neurocardiogenic syncope or "fainting spells."  If that doesn't work, you can use several other things. At this time, the best thing for you to do is to call your doctor back and let him know that the current treatment is not working so that other treatments can be considered or other tests can be performed.

Question: 
I am 20 weeks pregnant and thought everything was going fine. My obstetrician said she noticed a “bright spot” in my unborn baby’s heart and told me to go see a pediatric cardiologist for a fetal echocardiogram. My daughter has a heart murmur, but I was told it did not mean anything. What is a fetal echocardiogram and why do obstetricians refer patients for them? Is a “bright spot” on the heart serious? Does my daughter’s heart murmur make my unborn baby more likely to have a heart problem? Does the heart of my unborn baby work the same way before and after the baby is born?

Answer:
Your obstetrician may want to explore the possibility of a heart defect in more detail. Heart problems are the most common birth defect, occurring in about 1 percent of children. A fetal echocardiogram is a specialized ultrasound performed by a pediatric cardiologist that focuses on the fetus’s heart. The machine looks exactly the same as a regular ultrasound machine but images only the heart. It has specialized probes and hardware that allow clear images of the fetal heart. There is no risk to the fetus and you do not have to have a full bladder for the test. If a heart problem is found, the pediatric cardiologist can explain the diagnosis and what it would mean for the baby after birth.

An obstetrician may refer someone to a pediatric cardiologist for a fetal echocardiogram for several reasons. Some examples are

  • suspicion of a heart defect on routine ultrasound
  • heart rhythm abnormality
  • family history of congenital heart disease
  • a problem in another organ of the fetus
  • certain maternal illnesses or medication exposures

In most cases this test is just precautionary and is done to reassure you that everything is normal. This is almost always the case when the reason for referral is a “bright spot.” They almost always go away by the time the baby is born. If you or one of your children has a structural congenital heart defect, then future babies are at slightly increased risk. However, most heart murmurs are innocent and in no way mean that future children are at increased risk.

It is important to know that the fetal circulatory pattern is different than after the baby is born. That's because the placenta, not the lungs, provide oxygen to the baby. There is an extra vessel and hole in the heart that allow blood to bypass the lungs. The fetal circulatory pattern may protect the fetus from getting sick, even if there is a heart problem. This circulatory pattern also makes it impossible for the pediatric cardiologist to rule out small holes in the heart or a blockage in the blood vessel that leads to the lower half of the body. All normal fetal echocardiogram studies have this limitation.

Fetal echocardiography is usually very accurate but can be limited if the baby is not in a good position or there is low amniotic fluid. Sometimes, it is recommended to come back for a second fetal echocardiogram or an echocardiogram on the baby after birth, if a complete study can't be obtained.

Your pediatric cardiologist will communicate with your obstetrician to make sure the results of your fetal echocardiogram are included in your record. Pediatric cardiologists also work closely with pediatricians and neonatologists (newborn specialists) to make sure that all the doctors that may be involved in the care of your baby before and after birth are aware of any issues related to the heart.

Question:
My 15-year-old son had heart surgery when he was 14 months old. He saw his cardiologist once a year until he was 7, and we were given a good report. We moved to a new city seven years ago and have not found a new cardiologist. Other than the scar on the middle of his chest, he seems just like any other kid, but our pediatrician recommended we make an appointment with a local cardiologist. Is this necessary? Does it have to be a pediatric cardiologist?

Answer:
Almost all children who have had heart surgery need regular follow-up by a cardiologist with special expertise in caring for patients with congenital heart defects. For children, this specialist would be a pediatric cardiologist and for adults it would be an adult or pediatric cardiologist who has had extra training in caring for adults with congenital heart defects. The regular follow-up is necessary because even though heart surgery was performed, problems can happen with the heart as the child gets older. These problems can include trouble with the rhythm of the heart, narrowing or leaking of the heart valves, weakness in the heart muscle and other more rare complications.
 

It is important to know your son’s diagnosis. If possible, contact your son’s cardiologist from when he was small and request a copy of his medical records from the time of surgery and all subsequent cardiology visits. This will be very helpful when you go to a new doctor, especially in a new city. If you don’t have these old records, you or your new doctor may be able to call the cardiology department where your son had surgery to get a copy of the records.
 

For most patients who have had congenital heart surgery, follow-up is routine and often recommended yearly. The cardiologist will examine your son looking for sites of old scars and listening to his heart. Electrocardiography (EKG), echocardiography (ultrasound of the heart), 24-hour Holter monitoring, stress testing, and magnetic resonance imaging (MRI) or CT scanning are some of the tests your son’s cardiologist may recommend. The cardiologist will tell you if your child is at risk for needing future procedures (surgery or heart catheterizations) and also will describe any limitations to exercise and other strenuous activity that should be followed. You will also be told whether your child needs to take antibiotics prior to going to the dentist.
 

Given all the different types of congenital heart defects that exist, it is very important that a cardiologist who is knowledgeable about congenital heart disease be involved in your child’s care indefinitely. Specialized centers that provide care to adults with congenital heart defects are available in some places. These centers usually have a team of physicians, nurses and other professionals who have expertise in caring for adult patients who have had heart surgery. Your pediatric cardiologist can guide you on follow-up as your son enters adulthood.

Question:
When my boy was born, he was called a “blue baby.” The doctor said his heart has only one ventricle instead of two and no blood is flowing from the heart to the lungs. He said they will have to do three operations. The last one is called a Fontan Procedure. What does he mean? Can you explain why my baby needs all these surgeries? 

Answer:
Your baby was born with a very rare heart defect called pulmonary atresia. He has only one usable pumping chamber, or ventricle. The baby does have one normal main artery, called the aorta, which gives oxygenated blood to his body and two normal main veins (called the vena cavae) that bring unoxygenated (blue) blood back to the heart. To direct enough blood flow to the lungs, the two large veins that bring unoxygenated or blue blood from the body must eventually be connected directly to the arteries that go to the lungs (the pulmonary arteries). Because the blood pressure in a newborn baby’s pulmonary arteries is high, this vein-to-pulmonary-artery connection can't be done immediately. Therefore, the baby’s surgery will need to be done in three stages. The first operation is a shunt or connection from the main artery, or aorta, to the pulmonary artery. The second operation is a shunt from one large vein to the pulmonary artery (the first shunt is removed at the second operation). In the third operation, the second main vein is connected to the pulmonary artery. This third stage is called a Fontan Procedure. Your baby needs these three operations in separate stages because this helps him adjust gradually to the changes in the direction of blood flow. Doing the Fontan Procedure right away would put too much strain on his heart.

Question:
How do I get a second opinion regarding my child's heart problem?

Answer:
Obtaining more information about your child's heart problem is important. Never be afraid to ask your doctor questions and make sure you understand the answers. Sometimes parents feel that they would like a second opinion regarding their child's problem. Here are some helpful hints on how to get the information you need to make sure that your child is getting the best care possible.

  • First, talk to your child's cardiologist. Heart problems in children can be confusing, the working of the heart complex and the treatment options difficult to explain. It takes medical students many hours of classroom instruction to understand these difficult problems, so don't be embarrassed if you didn't understand or remember it all after your first or second session.
  • Make a list of questions. These can be emotional and difficult discussions, so it helps to have a written list of questions.
  • Ask for diagrams. Again, because of the heart's complexities, pictures can go a long way towards helping you understand the problem and treatment.

Sometimes children's heart problems can be emergencies that require immediate attention and might not allow time for second opinions. However, when time permits, if you still want more information or another point of view, consider the following:

  • Ask your child's cardiologist who to go to for the second opinion. Although this may seem awkward or embarrassing, your doctor should understand and should help you find another pediatric cardiologist in the field relating to your child's problem. If this is not possible, you may be able get a referral from your child's pediatrician. Medical care in the United States has become very subspecialized, especially for some of the complex heart problems of children. In certain circumstances, it might be even more appropriate to speak with a congenital heart surgeon or a pediatric cardiologist who specializes in areas such as cardiac catheterization, arrhythmia (irregular heart rhythm) treatment, heart transplantation, etc.
  • Get the pertinent records from your child's cardiologist. To make the most of your second opinion, it will greatly help the consulting specialist if he or she can have detailed records to review. Such records might include notes of office visits, catheterization reports, operative reports, videotape, CD, or DVD copies of echocardiograms or even copies of pictures obtained during cardiac catheterizations.
  • If possible, contact the consulting physician before the visit. The consulting physician may want to review your child's records before the visit or may ask you to bring them with you. In either case, being prepared will make it more likely that the visit will provide you with the information you are seeking. Remember to bring your list!

Question:
My baby was born with a slow heart rate. We were told that she has "heart block." What is this? Her doctors also told us she needs a pacemaker, but when we hold her and feed her, she seems fine. We're scared. What caused her heart block? I feel guilty. Did I do something that caused my baby to be sick? Will she ever lead a normal life if she has a pacemaker? Will she be able to go swimming and play with other children? Will she be able to have children when she grows up? I'm sorry I have so many questions.

Answer:
The heart is a pump with a built-in electrical system. Normally, electricity starts in the upper chamber and spreads to the lower pumping chambers through special heart tissue called the conduction system or AV node (see Figure 1). This system is like a nerve running through the middle of the heart. It permits the upper and lower chambers to contract one after the other, which permits the best pumping function.
 

Heart block occurs when the spread of electricity from the upper chambers (atria) to the lower chambers (ventricles) is interrupted (Figure 2). This interruption can be partial or complete. If partial, not all electrical signals travel from the upper to lower chambers. The heart rate may be irregular, but this may not cause problems. At other times, however, the interruption is complete and the upper and lower chambers have no electrical connection. The lower chambers pump independently of the upper chambers. This can cause problems if the heart rate of the lower chambers is too slow. Symptoms of tiredness, dizziness, headache and fainting can occur. If the rate is very slow, the heart pump function may not supply enough blood to the body. Then the heart becomes an inefficient pump and congestive heart failure can result.
 

When heart block is detected in a newborn, its exact cause may not be known, as in most types of congenital heart defects. Heart block usually occurs in hearts that are otherwise normal. Surgery on the heart can't fix the problem. However, if the infant's heart rate is too slow and symptoms develop, a pacemaker may be needed. Pacemakers are small battery-powered electronic devices, a little bigger than the size of two silver dollars, that remind the heart to beat at an appropriate rate to prevent symptoms of the very slow heart rate. These devices are surgically implanted under the skin in the upper abdomen or chest. A wire connects the pacemaker generator to the heart itself. The pacemaker will usually be necessary throughout a person’s life and will need to be surgically replaced, usually every five to 10 years as the battery wears down. Infants with pacemakers can develop normally and lead normal lives. Later in life, they'll need to avoid some types of contact sports like football and wrestling to prevent damage to the pacemaker system.
 

Some children do not need pacemakers until they are teenagers. These pacemakers have their wires placed through veins leading to the heart and connected to the pacemaker in the upper chest under the collarbone.
 

At times, a mother with an infant who has heart block may unknowingly have a condition that causes problems with some of her own body's tissues, like skin and joints. An example of one of the types of this condition is called lupus erythematosus. Her condition can affect the developing fetus by interfering with the infant heart's conduction system. The infants themselves don't develop the skin and joint problems. Since mothers having a newborn infant with heart block may develop skin and joint problems themselves later in life, they should notify their physicians and consider having special testing.

Question
I was recently told that my 6-year-old daughter has a murmur but that it is an "innocent murmur" and I have nothing to worry about. My sister was born with a hole in her heart and had open heart surgery and now is on medicines because her heart is weak. I am scared that my daughter has something serious. Is there such a thing as an innocent murmur? If so, what is it? Should I be more worried about my daughter because my sister has congenital heart disease?

Answer:
 

Innocent heart murmurs are sounds made by the blood circulating through the heart's chambers and valves or through blood vessels near the heart. They're sometimes called other names such as "functional" or "physiologic" murmurs.
 

Innocent murmurs, also called functional or normal murmurs, are very common during infancy and childhood. In fact, it is estimated that at some time in their lives most children will have an innocent heart murmur. There are a few types of innocent murmurs, but all represent the normal sound of blood as it flows from the heart into the main blood vessels of the body or lungs. Normal murmurs are usually diagnosed by the typical sound heard with a stethoscope during a physical examination. Some murmurs require additional tests, such as an electrocardiogram (ECG) or echocardiogram (Echo) to distinguish them from much less common abnormal murmurs.


Heart abnormalities can on rare occasions run in the family. However, if you do not have congenital heart disease and your sister is the only family member to be born with a heart problem, then your daughter is not at increased risk of having such a problem.
 

Sometimes, when a doctor first hears the murmur through a stethoscope, he or she may want to have other tests done to be sure the murmur is innocent. After that, there's no need for a cardiac reevaluation unless the patient or doctor has more questions. The child doesn't need medication, won't have cardiac symptoms, and doesn't have a heart problem or heart disease. A parent doesn't need to pamper the child or restrict his or her diet or activities. The child can be as active as any other normal, healthy child.

Question:
When my son is sleeping, his heart rate frequently varies at 80, 60 and 40 with a few seconds pause; no color change is noted. Is this an indication of any potentially serious problems?

Answer:
Although you did not mention your son’s age, I am assuming he may be about 7 or 8 years old. If so, a heart rate of 80 beats per minute would be within the realm of normal. The heart rate will go down during sleep and it will vary from time to time, most often with his breathing pattern. However, if you are concerned about this, you should discuss this with your child’s pediatrician. Because he or she will be familiar with your son’s medical history and the results of any further evaluation he may wish to do, the doctor will be able to answer your question and, equally as important, give you peace of mind.

Question:
My sister was told her daughter needs a heart transplant. Can you provide me with information about this?

Answer:
Heart transplantation involves replacing a failing heart with a new one (donor heart). Children undergo heart transplantation for two primary reasons -- a congenital heart defect or severe malfunction of the heart muscle. Heart transplantation is considered when there are no other medical or surgical options. The hearts are donated from someone who has suffered an injury resulting in death of his or her brain, but not the other vital organs and whose family has agreed to make the heart and other organs available for donation. Many children who receive heart transplants live years longer than they otherwise would have and have an excellent quality of life.
 

Children who need heart transplants are placed on a waiting list administered by a national agency called United Network of Organ Sharing (UNOS). The waiting time for a heart can range from days to months (even more than a year) and depends on multiple factors including size, blood type, and whether the patient is hospitalized on intravenous heart medications.
 

Patients who receive a heart transplant need to be on medications to prevent their own body from attacking the heart (rejection). This is because the body’s defense system against infection (immune system) recognizes the heart as a foreign body and tries to damage it. Unfortunately, these medicines have many side effects, including making the body more susceptible to a dangerous infections, damaging the kidneys, and even cancer. Frequent visits (at least every three months) to the transplant cardiology team are required for close monitoring.

Question: 
Please tell me about hypertrophic cardiomyopathy in children. My 8-year-old daughter was just diagnosed with ventricular hypertrophy. When she was 2, she was diagnosed with a heart murmur and I was told that she would probably outgrow it. Can you shed any light on how this could have happened?

Answer:
The murmur your child had at age 2 likely had nothing to do with her current diagnosis. Murmurs, or "extra heart sounds," are very common in children and are usually "benign" or "innocent" murmurs. These extra sounds may arise from the thin chest wall and rapid growth seen in early childhood.
 

You have been told that your daughter has "ventricular hypertrophy," which is a thickening of the heart muscle. This diagnosis can be suspected by an electrocardiogram, but needs to be confirmed by an echocardiogram read by a pediatric cardiologist. Ventricular hypertrophy can occur from stenotic (blocked) heart valves, high blood pressure, or can be the result of a condition called hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy is caused by several genetic defects resulting in an abnormally thick heart muscle. This disease can run in families or be the result of a new genetic mutation. It can result in poor heart function, heart failure, heart rhythm problems and in some severe cases, can cause passing out or cardiovascular collapse. If ventricular hypertrophy is confirmed by further testing, it is important that the cause be determined and the appropriate treatment and medical checkups be followed.

Question:
My son was born with Hypoplastic Left Heart Syndrome. He has had two operations and we have been told that he will need another one. Although his doctor has explained what he has to me, I still don't understand what is wrong with him and why he is blue and can't keep up with other children his age. Will he always be like this?

Answer:
The heart normally has two lower pumping chambers. The right ventricle normally pumps blood to the lungs, while the left ventricle normally does all the work to pump the blood to the rest of the body. In your son's case, the primary problem is that the left ventricle did not develop properly during the pregnancy and the right ventricle has to do all the work. The first two operations result in the blue blood from the upper half of the body going directly to the lungs and the rest of the blood mixing together and going to the body. The blue blood (without oxygen) returning from the body mixes with the red  blood (returning from the lungs). The mixed blood is then pumped to supply the body, causing him to appear blue. Your son’s next operation, known as the Fontan procedure, may result in much improved oxygen levels because all the blue blood will go to the lungs. This may allow him to better keep up when participating in physical activities.

Question:
Our 4-year-old daughter was born with a small hole between the upper two chambers of her heart (it’s called an isolated ASD).  Her cardiologist said it would not need to be repaired.  Recently, I was talking with some parents in a local support group.  They said kids with congenital heart defects need to take antibiotics before going to the dentist to keep them from getting endocarditis.  We’ve never been told that our daughter should get antibiotics, and she is about to go for her first dental visit.  I called her doctor, and she said that our daughter did not need antibiotics.  We are still concerned, because we don’t understand why most of the kids need the antibiotics.  Should I tell her dentist that she needs antibiotics?  What are the antibiotics for?  What is endocarditis?

Answer:
Most children with congenital heart defects are at an increased risk for developing bacterial endocarditis, an infection of the lining of the inside of the heart or the heart valves (this lining is called the endocardium).  However, children with the condition called isolated secundum atrial septal defect (ASD) are not at increased risk, so that’s why your cardiologist did not recommend antibiotics for your daughter.

Bacterial endocarditis (also called infective endocarditis) occurs when bacteria in the bloodstream (bacteremia) lodge on abnormal heart valves or most structural abnormalities of the heart.  Certain bacteria normally live on parts of the body, such as the mouth and upper respiratory system, the intestinal and urinary tracts, and the skin.  Some surgical and dental procedures cause a brief bacteremia.  Although bacteremia is common following many invasive procedures, only certain bacteria commonly cause endocarditis.

Most cases of endocarditis can’t be prevented, because we don’t always know when a bacteremia occurs.  For many years the American Heart Association recommended that children and adults at increased risk for endocarditis take antibiotics before certain dental and surgical procedures.  This was done because it was thought that when someone was having one of the dental or surgical procedures that can cause bacteremia, antibiotics could be given to prevent the bacteria from surviving in the bloodstream.  This is called “endocarditis prophylaxis”.  However, the AHA made major changes to their guidelines in 2007.  After they reviewed all of the scientific evidence on the causes and prevention of endocarditis, they concluded that the prophylactic dose of antibiotics given before dental and surgical procedures prevent very few, if any, cases of endocarditis. There is also some risk involved with taking antibiotics.  Therefore, the new guidelines say that antibiotic prophylaxis should be reserved only for those children and adults who have the greatest risk for a bad outcome if they get endocarditis.  Patients with congenital heart disease who are at the greatest danger of bad outcomes from IE and for whom preventive antibiotics prior to a dental procedure are worth the risks include those with:

  • Unrepaired or incompletely repaired cyanotic congenital heart disease, including those with palliative shunts and conduits
  •  A completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first six months after the procedure
  •  Any repaired congenital heart defect with residual defect at the site or adjacent to the site of a prosthetic patch or a prosthetic device

Except for the conditions listed above, antibiotic prophylaxis is no longer recommended for any other form of congenital heart disease.

Other people who are still recommended to take antibiotics before most dental procedures include those who have a prosthetic heart valve, those who have previously had a case of endocarditis, and a cardiac transplant patient whose heart develops a problem in a heart valve.  Most of these situations would be in adults, but some could be children.

So, while your daughter doesn’t need antibiotics before these procedures, children with any type of congenital heart defects listed above do need them.

Furthermore, the AHA emphasizes that for anyone at increased risk for endocarditis, good oral health and hygiene by regular tooth brushing, etc. is more protective against getting endocarditis than taking the prophylactic antibiotics.

Question:
My daughter was born with an interrupted aortic arch and a double outlet right ventricle. Please explain these heart defects to me. What are my chances of having another child with the same condition?

Answer:
Your daughter was born with several complicated heart defects that involve problems with the heart pump as well as both main blood vessels that supply the body and lungs. In this condition, the main blood vessel to the body (aorta) is interrupted in the area of the blood vessels that supply the upper body. In addition, instead of the lung artery (pulmonary artery) and aorta coming out of separate chambers, both arise from the right-sided ventricle. There is also an associated hole (ventricular septal defect or VSD) between the pumping chambers. This heart defect requires surgery during infancy. After surgery, your child will need to be seen by a congenital heart disease specialist for the rest of her life.
 

This combination of heart defects may be associated with a genetic syndrome called "DiGeorge Syndrome," which may also include other defects such as abnormal function of the immune system as well as the gland that controls calcium in the bloodstream. This syndrome is caused by a genetic defect on one of the chromosomes, (chromosome 22q11). The heart defects may also occur separately and can also be associated with a similar defect on the same gene. This gene defect can be detected by a specialized genetic test (FISH or fluorescent in situ hybridization). This test can determine if one of the parents also has this genetic defect or if this is a new defect (gene mutation). The results of this testing will help determine the risk of having another child with the same or a related heart defect. If neither parent has this gene defect, the risk of having another child with congenital heart disease is 4 percent to 6 percent. But if one parent has the gene defect then the odds of having a child with a similar gene defect may be 50 percent. These issues should be discussed with your cardiologist or genetics specialist.

Question:
My obstetrician detected an irregular heart rhythm during a regular visit today. I am 24 weeks pregnant and everything else has been fine. What is an irregular heartbeat and what does it mean for my unborn baby? Will this problem go away before my baby is born? Do I need to take medication?

Answer:
An irregular heart rhythm is usually benign, but your obstetrician may refer you to a pediatric cardiologist for a fetal echocardiogram to better define the heart rhythm. 
 

The normal fetal heart rate is between 120 and 160 beats per minute. The heartbeat is usually regular with only a little variation in the heart rate. Each normal heartbeat begins from the top part of the heart (atrium), followed by the bottom part of the heart (ventricle). The bottom part of the heart is what causes blood to leave the heart and make a sound on the heart monitor.
 

The most common cause of an irregular heart rhythm is when the top part of the heart beats too early (premature atrial contraction, or PAC). Sometimes this premature beat is transmitted to the bottom part of the heart; other times the communication between the top and bottom part of the heart is blocked after a premature beat.
 

An early beat that is transmitted or blocked will cause an irregularity in the heart's rhythm. These premature atrial contractions are very common in the second half of pregnancy and usually don't cause any problems. They may persist after the baby is born, but usually go away by one month of life. Medication is typically not necessary. 
 

Irregular heart rhythms can cause problems in a small percentage of cases if multiple consecutive premature beats occur. When this happens for a long enough period, it's called tachycardia (fast heart rate). If the heart rate stays above 200 beats per minute for a long period of time (hours or days), it could damage the baby’s heart and other organs. If tachycardia occurs, your pediatric cardiologist and obstetrician will recommend medicine for you to take to help regulate your fetus’s heart rhythm. If there are any signs of damage to the fetal heart, it may be recommended that you be hospitalized while beginning the medicine. If you are put on medicine, it is likely the baby will need to take a similar heart rhythm medicine after birth. 
 

Another cause of slow fetal heart rate is a condition called heart block, or atrioventricular (AV) block. This is more commonly seen in mothers with lupus and other related autoimmune conditions. Heart block may also be seen in fetuses with structural congenital heart disease, and may lead to excessive fluid buildup around the heart and the fetus in general – a condition called “hydrops.”
 

Most fetuses with tachycardia have a good prognosis if the heart is structurally normal. Some fetuses have a pattern of frequent blocked premature contractions resulting in a low heart rate (bradycardia). This does not usually cause any problems to the baby as long as the heart rate remains above 60–70. Some fetuses with premature atrial contractions can have bradycardia on one visit and develop tachycardia later on. An irregular heart rhythm is not usually worrisome in the fetus, but should be followed closely by your obstetrician and pediatric cardiologist.

Question:
I was told that my daughter has Kawasaki Disease. What is this? Is it serious? Will she get better and lead a normal life?

Answer:
Kawasaki Disease (KD) is an inflammatory disease of unknown cause. Some evidence suggests it may be caused by an infection. KD usually occurs in children, and is very rare in adults. Children with KD have high fevers lasting at least five days, along with a variety of other symptoms. These may include conjunctivitis (inflammation of the inner eyelid and whites of the eyes), rash, swollen lips and tongue, enlarged lymph nodes, and reddened, peeling hands and feet. There is no specific test to prove that a child has KD. The diagnosis is made based on a characteristic illness pattern, and the exclusion of other diseases, such as strep throat. Most children with KD are very irritable.
 

Children with uncomplicated KD generally recover without treatment. However, serious complications can develop if the disease involves the coronary arteries. The coronary arteries, which supply the heart muscle with blood, can become enlarged, forming “coronary artery aneurysms.” Mild degrees of enlargement may not cause problems, but significant enlargement, known as “giant aneurysms,” can result in coronary occlusion (blockage). If the enlarged coronaries become blocked from blood clots, then the heart muscle supplied by the coronary artery suffers from a lack of oxygen. This can cause chest pain (known as angina) or a heart attack. Some children require medicine to dissolve or prevent blood clots. Some require urgent coronary artery bypass surgery. The dilation of the coronary arteries and the risk for blockage can be permanent.
 

Fortunately, a child’s risk for coronary artery aneurysms can be reduced. Treatment with an intravenous medicine known as gamma globulin can help reduce this risk. Children with known or suspected KD are generally admitted to the hospital and treated with intravenous gamma globulin. They are also usually given high doses of aspirin until their fever goes away. Afterward, a low dose of aspirin is continued to prevent blood clots. An echocardiogram is performed to find out if there is any involvement of the coronary arteries. Children who remain with ongoing fevers, or who have coronary involvement, are treated more aggressively. These children may receive additional doses of gamma globulin or be treated with steroids or other medicines.
 

Whether your daughter will get better and lead a normal life depends partly on how well she responds to treatment. Most importantly, in depends on whether she develops coronary artery aneurysms. Most children who are treated with gamma globulin within 10 days of illness recover completely. Those without giant aneurysms usually have normal health after they recover. However, children with giant aneurysms usually require ongoing treatment to prevent blood clots. They often have significant physical limitations. These children require close medical follow-up and may require surgery.

Learn more about Kawasaki Disease.

Question:
My pediatrician suspects that my son has Marfan syndrome and has referred him to a pediatric cardiologist for evaluation. What exactly is Marfan and how does it affect the heart?

Answer:
Marfan syndrome is a rare disorder; it affects one in every 5,000-10,000 people. It usually "runs in the family," but your son could also be the first in your family with Marfan syndrome. Almost all body parts and organs function normally, and the brain is not affected. Many historians believe that Abraham Lincoln had Marfan syndrome. People with Marfan syndrome are tall and thin, have long arms, legs and fingers, and tend to be double-jointed. They also are likely to have curvature of the spine and an unusually shaped breastbone. Patients with Marfan syndrome tend to be near-sighted, and some can develop more severe vision problems due to shifting in the position of the lens of the eye.
 

Your son was referred to a pediatric cardiologist because 60 percent to 80 percent of people with Marfan syndrome develop enlargement of the first portion of the aorta -- the main artery that takes blood from the heart and distributes it to all parts of the body. For this reason, an echocardiogram helps to confirm the diagnosis of Marfan syndrome. 
 

Problems from enlargement of the aorta in children or teenagers are very rare. Later in life, many people with Marfan syndrome develop so much enlargement of the aorta that open-heart surgery may be needed to replace the first portion of the aorta before it ruptures. Therefore, if your son is diagnosed as having Marfan syndrome, echocardiograms will probably be needed every year to check on the growth of his aorta.
 

Some doctors will prescribe a medication called a beta-blocker for patients with Marfan syndrome. By relaxing the heart and slowing the heart rate, beta-blockers may slow down the rate at which the aorta dilates. A new drug treatment for Marfan syndrome is currently being tested in children and adolescents with Marfan syndrome and dilated aorta. This study is sponsored by the National Institutes of Health and information about it can be found through the Pediatric Heart Network website and at www.clinicaltrials.gov.

You can find a lot more information at the National Marfan Foundation website.

Question:
My 10-year-old daughter has been complaining of a headache and dizziness for a week. The pediatric cardiologist diagnosed it as mitral valve prolapse (MVP) and explained there was not much to be concerned about. Please tell me what this condition is. What should my daughter avoid?

Answer:
MVP is usually a very benign condition that occurs in children and adults. The mitral valve prevents blood from going backwards by closing when the main pumping chamber of the heart (left ventricle) is squeezing or contracting. Some times the leaflets of the mitral valve moves backwards a little during the contraction of the left ventricle. In most patients this causes a minor and insignificant amount of leakage and does not affect the function of the heart.
 

Some patients with MVP may have a variety of symptoms such as palpitations, headaches, dizziness, or even fainting. This aspect of MVP is not related to the severity of the valve leakage; in fact, it is most common in patients with minimal valve leakage. This condition is rarely dangerous but can be uncomfortable. Rare disorders such as thyroid disease, inflammatory diseases, and defects in the strength of heart vessels and valves may all be associated with MVP and can usually be easily diagnosed when they are present, because of other signs and symptoms.
 

Reduction or elimination of caffeine containing food and drinks (coffee, soda, chocolate) is often helpful in making the headaches, dizziness and palpitations go away. Many patients improve with an increase in their daily fluid and/or salt intake. Some patients may require a medication to offset the effect of the adrenalin. These medicines, called beta-blockers, will lower the heart rate and blood pressure, and help to reduce or eliminate symptoms. Almost all patients with MVP can participate in sports without restrictions. 
 

Although many physicians may suspect MVP when listening to the heart, echocardiography (ultrasound of the heart) is the only way to definitively diagnose MVP. It is also important to know that MVP is the most over-diagnosed heart condition and should only be made and treated by an experienced pediatric cardiologist.

Question:
I have been told that my 6-year-old daughter has pulmonary hypertension. What does this mean? Is it the same as the hypertension that has been diagnosed in my mother?

Answer:
Pulmonary hypertension is a condition in which pressure in the blood vessels in the lungs is higher than normal. This is different from systemic hypertension where pressure in the blood vessels to the body is higher than normal. Systemic hypertension is a common condition especially in people as they age. It is diagnosed by taking a blood pressure in the arm. Systemic hypertension is usually successfully treated by oral medications. In contrast, pulmonary hypertension is a rare condition in both children and adults. Patients usually present to the doctor with symptoms of fatigue, chest pain, difficulty breathing with activity and episodes of fainting or nearly fainting. As you can imagine, these symptoms are difficult to get a handle on, especially in infants. Thus, pulmonary hypertension is often diagnosed late, after the patient has had vague symptoms for some time. Pulmonary hypertension can be diagnosed by echocardiography (cardiac ultrasound), although cardiac catheterization is often recommended to confirm the diagnosis and as a way to better understand the cause.

There are two broad classes of pulmonary hypertension: those without any obvious cause (termed idiopathic) and those caused by or occurring with other diseases. Diseases that can cause pulmonary hypertension include:

  • lung disease (such as immature lungs in premature babies or airway obstruction)
  • some congenital heart diseases with excessive blood flow to the lungs or blockage of blood flow from the lungs back to the heart
  • diseases of the clotting system of the body
  • diseases of inflammation (such as systemic lupus erythematosus, SLE)
  • some drugs and toxins
  • HIV infection
  • certain blockages in the drainage of the liver (portal hypertension)

When possible, the best treatment for pulmonary hypertension is treating the underlying disease. When this is possible, the pulmonary hypertension may resolve or at least improve. In situations where treatment of the underlying disease does not improve the pulmonary hypertension and in situations of idiopathic pulmonary hypertension, medications are available. In the past, these medications were only intravenous and often involved long-term intravenous therapy. Inhaled and oral medications have more recently become available. Although rare, lung transplantation has been used successfully in severe cases.

For more information on pulmonary hypertension, visit the Pulmonary Hypertension Association's website.

Question:
We were told that our child has pulmonary valve stenosis. His heart valve doesn't open completely. What can be done to fix the valve?

Answer:
The pulmonary valve is like a one-way swinging door on the right side of the heart that prevents back flow of blood from the lungs to the heart. Sometimes, during heart development, the valve does not form properly. If the valve is tight (stenosis), the right side of the heart needs to work harder to push blood through the valve to the lungs. Depending on how tight the valve is, it may or may not need to be fixed. Your congenital heart specialist can discuss this with you. If the valve is only mildly tight, it can be left alone and may get better as your child gets older. However, if the valve is tight and causes the heart to work harder than it should, it may need to be opened with a small balloon using a special tube (catheter) placed into the heart from a blood vein as part of an interventional catheterization procedure. If the valve is very tight or malformed, it may need to be surgically opened or replaced.

Question:
My baby was just diagnosed with a single ventricle. What does this mean?

Answer:
The normal heart has two pumping chambers or ventricles. "Single ventricle" refers to congenital heart defects where one of these chambers is absent or very small, or defects where the partition between the two pumping chambers is missing. These defects are often called hypoplastic left heart or hypoplastic right heart. Hypoplastic left heart includes both aortic and/or mitral atresia (absence of the valves on the left side of the heart). Hypoplastic right heart includes a wide range of defects with a small or absent tricuspid valve or pulmonary valve (the valves on the right side of the heart). In addition, a single ventricle occurs if there is no partition between the two pumping chambers. These defects can result in severe heart failure (not enough blood to the body) or severe cyanosis (not enough blood to the lungs). All of these defects require a common treatment designed to ensure blood flow to both the lungs and the body. Sometimes this requires limiting blood flow to the lungs and creating a new blood vessel to provide blood flow to the body. Other times, operations are required to ensure blood flow goes to the lung arteries. Ultimately, most babies with single ventricle need two or three operations that result in the channeling of blue blood (unoxygenated) to the lungs and red blood (oxygenated) to the body. There are good surgical options for most babies with single ventricle and many children with this condition can do most normal childhood activities. Your pediatric cardiologist can give you the best information on the specifics of your child's condition.

Question:
My 10-year-old daughter had a hole in her heart repaired when she was 1. She has been healthy since then. Will she be able to participate in sports?

Answer:
Questions about athletic participation in patients with heart problems are frequent. There are a wide variety of cardiac defects, surgical repairs, and sports. It is best for you to discuss with your pediatric cardiologist as to whether your daughter needs any restrictions from sports. This is particularly true if she wants to eventually participate at the high school or college level. Most children who have relatively simple cardiac defects, and who have had a good result after catheter or surgical repair can participate in at least some, if not all, athletic activities. Other children may have heart conditions that warrant some restrictions. Patients with pacemakers or implantable cardioverter/defibrillators (ICDs) may have restrictions not required for other patients with heart defects. Patients that are taking anticoagulants (drugs that decrease blood clotting) should also use caution and check with their physician about sports participation

Question:
We have a 1-year-old son who is doing well after surgery for transposition of the great arteries (DTGA). We'd like to have another child. Will our second child also have congenital heart disease?

Answer:
Congenital heart disease is the most common birth defect. About eight out of every 1,000 babies (or 0.8 percent) will be born with some form of heart problem. Most of these problems, however, are milder than your son’s. For a couple who's had one child with DTGA and has no other family members with transposition, the chance of having another child with DTGA increases slightly to about 18 in 1,000 or 1.8 percent. This is also about the same chance of this baby being born with a form of congenital heart disease other than DTGA. This gives a greater than 98 percent chance that the baby will be born with a normal heart. 

If, however, other family members (first-degree relatives) have DTGA, other forms of transposition, or other forms of congenital heart disease, the chance that the baby will be born with DTGA may be higher. Transposition rarely runs in the family.

We don't know why congenital heart defects like DTGA occur. The reason is presumed to be genetic, although a specific gene for DTGA hasn't been identified yet. With regard to DTGA, some evidence exists that multiple genes as well as other factors may be involved.

Because you've had one child with a congenital heart disease, it may be helpful for you to have a fetal echocardiogram between 18 and 20 weeks gestation during your next pregnancy. This specialized ultrasound is done by a pediatric cardiologist and shows the developing heart. It should be able to exclude most forms of significant heart disease. Most often the fetal ultrasound is a reassurance that the baby’s heart is developing normally.

Question:
My 4-year-old son was evaluated for a heart murmur today and underwent an echocardiogram. The doctor told me he had mild tricuspid valve regurgitation. What does this mean? Should I be worried?

Answer:
The tricuspid valve is one of four heart valves in the heart. This valve prevents blood from leaking backwards from the right ventricle (the chamber of the heart that pumps blood to the lungs) when this chamber squeezes.

Almost all children undergoing echocardiography (ultrasound of the heart) have a little bit of tricuspid valve leakage or regurgitation. This is often detected because newer echocardiography machines have technology sensitive enough to pick up a wide variety of normal signals from blood moving through the heart. Mild tricuspid valve leakage, in the absence of any other heart problems, is considered to be a normal finding and nothing to worry about.

Question:
My 5-day-old son has been diagnosed with ventricular septal defect (VSD). The size of the hole is about 2 mm. Is this considered large or small, and what are long-term and short-term effects of VSD?

Answer:
A ventricular septal defect (VSD) 2 mm in diameter is considered small. Because it is small, one would not expect your baby to have any problems. This VSD will likely close completely as your baby grows, depending somewhat on the precise location of the hole. This could occur quite soon (within months) or take a few years. In general, a VSD that has not closed by about age 5 will not close spontaneously. VSDs that are very small do not become big as babies grow.
 

Two complications from small VSDs are worth knowing about, both relatively rare. First, there is a small risk that a child or adult with a small VSD will develop an infection in the heart (endocarditis). These infections are bacterial and can occur after events when bacteria are in the blood.
 

The other complication from a small VSD depends on the precise location of the hole. People with a VSD immediately below the aortic valve (the valve at the exit from the left side of the heart to the body) have a small chance that one of the leaflets of that valve may start to sag towards the hole over time. This rarely happens, but when it occurs, can lead to leakage of the valve. For the rare occasion that it does, your physician might recommend surgical closure of the small VSD to prevent further problems with the aortic valve.

Question:
My 8-year-old son complained of chest pain and rapid heartbeat while taking a shower. This happened twice. He was on Flovent and Singulair for asthma. His doctor assures me that the medication had nothing to do with the chest pain. My son had an EKG and the pediatric cardiologist said it showed a wandering pacemaker and no further evaluation is necessary. Should I get a second opinion?

Answer:
The diagnosis of wandering pacemaker refers to a type of heart rhythm with normal variations in heart rate. The pacemaker is an area of the heart responsible for initiating the heartbeat. It's normally located in the upper part of the right atrium (the upper right chamber of the heart). Sometimes the wandering pacemaker can move from the high right atrium to the low right atrium.
 

Chest pain can be associated with an asthmatic condition. So, in your son’s case, chest pain alone would not be a cause for concern about his heart. Some asthma medications can also cause a transient increase in heart rate, but the medicines your son is taking generally don't do this. Rarely, patients may experience a heart rhythm problem called tachycardia (an abnormally fast heart rate). 
 

If your son’s perceived fast heart rate persists, your doctor may want to consider more testing. A transtelephonic EKG monitor is a device that can be used when symptoms occur. The patient presses a button on the recorder and the heartbeat tracing is saved in the monitor’s memory. This tracing can then be sent over the telephone to a cardiologist. Another test is called a Holter monitor. It records an EKG continuously for 24 to 48 hours, while the child is at home, both active and asleep. Either of these two tests can then be analyzed to detect any abnormal heartbeats and determine whether further testing or treatment is needed. 
 

Your son’s symptoms are most likely related to his asthma, but if these continue your doctor may want to consider more testing, including the two tests just described. In addition, if your son experiences any dizziness or fainting, your doctor should be notified at once.
 

It's important that you're comfortable with the diagnosis of the first pediatric cardiologist who examined your child. If you aren't, then seek another opinion

Question: 
My son was diagnosed with WPW. What is this? Will he need treatment? Will he need surgery or a pacemaker?

Answer:
Wolff-Parkinson-White Syndrome is a heart rhythm problem that can usually be detected on an electrocardiogram (ECG or EKG).


Drs. Wolff, Parkinson and White published the first article about this type of rhythm in 1930. Wolff-Parkinson-White (WPW) means that there is an extra electrical connection from the top chambers of the heart (atria) to the bottom chambers (ventricles)  

Sometimes, an abnormal rhythm occurs when heart electrical signals travel in the wrong direction, from the ventricles to the atria, across the extra electrical connection. This heart rhythm causes the heart to beat too fast (tachycardia). Sometimes patients may feel dizzy or nauseous during tachycardia, and fainting may occasionally occur. Medication may be used to slow the heart rate. In rare patients, the condition can be life-threatening.
 

If medication fails to control the tachycardia, or in patients at higher risk of life-threatening events, or if patients are averse to medical therapy, a procedure called catheter ablation may be used. It's done in the cardiac catheterization laboratory under sedation or anesthesia. It involves inserting several specialized catheters into the blood vessels in the groin. These catheters can locate the origin of the abnormal heartbeats in the atrium. They abolish the extra electrical connection using radiofrequency waves (heating) or cryoablation (freezing). Since the 1980s, the procedure has been performed in tens of thousands of children safely and effectively, and provides a permanent cure more than 90 percent of the time. Some patients and physicians prefer ablation over medications as first-line therapy for WPW.
 

It's important for each patient to discuss questions and concerns directly with their cardiologist to understand how these recommendations apply to their specific circumstance.