If your child is born with a heart defect, chances are better than ever that the heart defect can be overcome and that your child will grow and develop normally. We have made many advances in not only diagnosing congenital heart disease, but we have made great strides in perfecting the surgical repair of these defects. In defects once thought to be hopeless, we now have excellent surgical options. It is always best to have your child continue with routine well child visits with his or her primary care physician and if any cardiac or heart abnormalities are detected, your pediatrician will arrange for appropriate follow-up by pediatric cardiology. As parents, we have the responsibility of helping our children lead active healthy lifestyles. Encouraging our children to be active in physical activities and to encourage them to have healthy eating habits would be two excellent lifestyle changes that they can carry out into adulthood. By leading active healthy lives, we hopefully will see less acquired disease - obesity, high blood pressure, diabetes - which can affect the heart.

What heart conditions are found most often in children?

The most common heart conditions found in children are structural heart defects. Congenital heart defects are those that are present at birth. Of all structural heart defects, ventricular septal defects or communications between the bottom chambers of the heart are most common. As a matter of fact, structural heart defects occur in roughly 8 per 1,000 live births.

What are the first steps that should be taken after diagnosis?

The initial step following diagnosis of structural heart disease depends on clinical findings, findings that the pediatric cardiologist recognizes. Sometimes no follow-up or further testing is necessary. Other times, a more aggressive workup is needed. Often if we suspect congenital heart disease, we will request that a child undergo a chest x-ray to evaluate heart size and configuration as well as pulmonary blood flow. In addition, we will obtain an electrocardiogram to evaluate cardiac rhythm, and many times the EKG will give us information regarding heart size as well. The EKG and chest x-ray are the most common testing that we will obtain.

Is high blood pressure a problem found often in children?

It is not often found in children. Sometimes when we do evaluate a child for hypertension, we find that there is an associated structural or heart disease problem. Most specifically it is coarctation of the aorta. In this condition, the aorta, which is the major blood vessel which leaves the heart flowing to the body, is constricted or pinched. This obstructs blood flow from the heart to the rest of the body. This is where hypertension can be detected above the level of narrowing. With this condition, congestive heart failure as well as high blood pressure may develop. This diagnosis is specifically considered in a patient who is in the immediate newborn period.

What are the most severe or serious defects?

The most serious or severe congenital heart defects would include a significant left-sided heart obstruction. More specifically, this would be hypoplastic left heart syndrome. In hypoplastic left heart syndrome, or HLHS, the left side of the heart is under developed. The left side of the heart, the left ventricle, has the primary role of pumping blood from the heart to the rest of the body. When we se an underdeveloped left side, we often see a small aorta as well. We have gained knowledge over the years in terms of addressing infants with hypoplastic left heart syndrome. We have a three-stage procedure which infants undergo in attempts to repair this disease entity. This condition is fatal without either surgical repair or heart transplantation.

I am a father of a 5-year-old girl who has to go to the cardiologist today. She has had chest pains, and her pediatrician said for her to see a cardiologist. She is nervous about what is going to happen during the exam. Can you help me explain to her what is going to happen?

The cardiologist may be ordering an EKG and/or chest x-ray. The cardiologist will spend a significant amount of time obtaining a thorough history in terms of the chest pain. The cardiologist will ask frequency of chest pain, ask questions in terms of exercise and chest pain, and also obtain a family history, which may be helpful in this evaluation of chest pain. She need not be nervous for the primary cause of chest pain in children of her age is non-cardiac. In other words, statistically, we would expect her heart to be healthy and the chest pain may be related to musculoskeletal problems. You can encourage your daughter to be optimistic that this will be a good visit, and I assure you it will be very painless. He also will examine her heart carefully and will conclude by reviewing with you his or her impression.

What measures can be taken when a fetus is discovered to have heart defects while in the mother's womb?

The most important role I have as a pediatric cardiologist when I detect fetal congenital heart disease is to counsel the parents and explain to them congenital heart defects their child will be born with and also the plan for the most appropriate postnatal care. In other words, will it be safe for this fetus to be delivered locally, by the local physician, or will it be necessary to have this infant born at a tertiary care center? At the tertiary care center, there are not only pediatric cardiologists and cardiac surgeons, but also a sophisticated intensive care unit for infants.

My daughter had the DKS procedure performed at 9 months and has had the second stage glynn. Would you suggest we avoid daycare until she's old enough to go to preschool? Will she have a difficult time with viruses is she's not exposed to other children at a later age?

I suggest that you discuss this with your local pediatric cardiologist as well as your daughter's primary care physician. I do not know enough about your daughter's overall general health to be able to make an accurate recommendation or opinion about this.

What percentages of babies are born with heart defects?

In 8 per 1,000 live births there is congenital heart disease. Many more fetuses are found to have congenital heart disease, perhaps three to four times the incidence just stated. However, many of these fetuses succumb to the congenital heart defect or perhaps other abnormalities such that the overall incidence in heart disease in live births drops to 8 per 1000.

Are children with family history of heart disease more likely to suffer heart disease as a child?

We first need to define heart disease. If we are talking about structural or congenital heart disease, that is heart abnormalities that are found at birth, yes, sometimes there is a higher incidence in family members of families with heart disease. Specifically, left sided heart abnormalities have a higher incidence of recurrence than other congenital heart defects. However, if we are defining heart disease as myocardial infarctions or heart attacks, then we need to evaluate if there are cardiovascular risk factors in these families such as cardiovascular disease at a young age, strokes occurring in people under the age of 50, or elevated cholesterol. If we identify family members with what we call early cardiovascular disease risk factors, then it is important to screen family members. Typically, this is for cholesterol, which sometimes is associated in family members under the age of 50.

What are the newest advances of Pediatric Cardiology?

We are now able to correct some congenital heart defects which in the past required open-heart surgery. The FDA has now approved a device, which can be used through a cardiac catheterization procedure to close small either upper or lower chamber holes of the heart. A cardiac catheterization is a study where a catheter is placed in the child's groin, and the catheter is directed up to the heart and the device is placed at the tip of the catheter. The device can then safely be placed in the area of the heart defect to close the defect in question. We feel it is a very safe procedure, and it works well. There are also valve narrowing or stenoses, which in the past required open-heart surgery in order to relieve the valve narrowing. We now can take these children to the cardiac catheterization laboratory and perform balloon angioplasty of the narrowed valve. Another cardiac catheterization process to address a cardiac defect would be a placing coil into the patent ductus arteriosus (PDA) in a child who has this diagnosis. The coil is placed in this tiny vessel and when the coil is released, it occludes this extra connection.

How common is it for a child to have Shone's Complex? Are my chances of having another child with this condition higher than other heart defects?

The incidence of Shone's complex is very low. However, once you have had a child with left-sided heart abnormalities, such as a child with Shone's complex, the incidence of recurrence in subsequent pregnancies is slightly increased. Again, we have quoted the incidence of congenital heart disease as 8 per 1000 live births. The incidence of having a child with a left-sided lesion once there is a family history of left sided heart disease may go up to as high as 5 to 12 percent in subsequent pregnancies. You may want to discuss this with your child's pediatric cardiologist and he or she will be able to provide you with more details in terms of recurrence of congenital heart disease. He or she will also perhaps suggest a fetal echocardiographic examination at roughly 20 to 24 weeks of pregnancy to evaluate the fetal heart.

My niece (15) has been diagnosed with Wolf's Parkinson's White.... can you explain this condition to me?

Supraventricular tachycardi or SVT is the most common abnormal tachycardia (fast heart rate) in children. SVT in childhood is usually associated with abnormal electrical conduction pathways. In the condition called Wolf-Parkinson-White, or WPW, there is an abnormal conduction pathway, which runs between the upper chambers of the heart or atria, and the lower chambers, or ventricles. This electrical signal can arrive at the ventricles sooner than normal, and this is how your niece can develop the WPW or tachycardia. This condition is actually named after the three people who first described it. We can recognize WPW by characteristic changes on an EKG. Many people with WPW have no symptoms at all, and many have no episodes of tachycardia. However, if the WPW is associated with tachycardia, we have medication that can improve the child's episodes of SVT. If medication is not successful, there is a procedure called radio frequency ablation which can actually eliminate the abnormal pathway by passing energy through a catheter. This procedure is done in the pediatric cardiology catheterization laboratory.

If one child is born with a heart condition, what is the likelihood that the next child will be born with it as well?

The incidence of congenital heart disease is roughly 8 per 1000 live births. The risk of recurrence of congenital heart disease in subsequent pregnancies is slightly increased once there is a child or family member who has congenital heart disease. The recurrence risk is quite low, perhaps 3 to 5 times higher.

Is congenital heart disease more frequent in one sex more than the other?

We have learned that if there is a maternal history of congenital heart disease, then the risk of recurrence in the family is slightly higher than if there is a paternal history of congenital heart disease. Overall, congenital heart disease does not seem to be more frequent in one sex than the other.

My doctor says my child has a heart murmur, and he will grow out of it. Can you explain this to me?

Most likely your physician is describing an innocent heart murmur. There is no need to be alarmed about this diagnosis. An innocent heart murmur, just as the name implies, is innocent or normal. With an innocent murmur the blood circulating through the heart chambers and valves make sounds. These sound or murmurs are detected in your child through the doctor using a stethoscope. Innocent murmurs are very common in children, especially between the ages of 3 and 7. They are quite harmless, and children with innocent heart murmurs do not need to be restricted from any activities. Most often these murmurs will disappear and then reappear during childhood, but become more difficult to detect when the child reaches near adulthood. During times of illness or fever when the heart rate changes these innocent murmurs can become more prominent or louder. The child's heart is entirely normal if they have been diagnosed with an innocent heart murmur, and no further follow-up in pediatric cardiology is necessary.