Pediatric Cardiomyopathy

Pediatric Cardiomyopathy

What is cardiomyopathy?

Cardiomyopathy is a chronic heart condition that involves the deterioration of the heart muscle (myocardium). The heart muscle becomes abnormally enlarged, thickened and or stiffened. Eventually the heart is unable to pump effectively and cannot supply the body with enough blood to function. In severe cases, cardiomyopathy can lead to heart failure or sudden death.

There are 5 forms of cardiomyopathy:

  • Dilated cardiomyopathy (DCM)
  • Hypertrophic cardiomyopathy (HCM)
  • Restrictive cardiomyopathy (RCM)
  • Arrhythmogenic right ventricular cardiomyopathy (ARCM)
  • Left ventricular non-compaction cardiomyopathy (LVNC)

Who gets cardiomyopathy?

Cardiomyopathy predominantly affects adults, but in rare instances affects infants and children. According to the North American Pediatric Cardiomyopathy Registry, one out of 100,000 children in the U.S. is diagnosed with cardiomyopathy each year. However, this is a conservative number because it does not include all types of cardiomyopathies. Cardiomyopathy can occur in any child regardless of age, gender, race or economic background.

Causes of cardiomyopathy in children:

There are many causes of cardiomyopathy in children, including some that are not fully understood. Common causes include:

  • Inheriting the condition from one or both parents
  • A viral infection
  • Toxins affecting other organs
  • Using chemotherapy drugs
  • Metabolic, mitochondrial or systemic diseases in parts of the body other than the heart

Despite advances in genetic testing, the exact cause remains unknown in 2/3 diagnosed children.

Symptoms of pediatric cardiomyopathy:

Occasionally, symptoms of pediatric cardiomyopathy can be mistaken as a cold, flu, asthma, or stomachache. Some affected children have no symptoms while others may experience any of the following symptoms:

  • Shortness of breath or rapid breathing
  • Dizziness and fainting
  • Irregular or rapid heartbeat
  • Abdominal bloating or swelling
  • Chest pain
  • Extreme fatigue

Infants may experience poor weight gain, extreme sleepiness, difficulty feeding or excessive sweating.

How is pediatric cardiomyopathy diagnosed?

A pediatric cardiologist confirms the diagnosis through an echocardiogram (echo) and electrocardiogram (EKG, or ECG). In some cases, other medical tests are necessary. Genetic testing may be recommended to verify the cause of the disease.

Since pediatric cardiomyopathy is rare, it is recommended to locate a children’s medical center with a wide range of experience in managing children with cardiomyopathy. Early diagnosis and treatment of the disease is essential to preventing complications and progression to heart failure.

Treatment options for cardiomyopathy:

There is no cure for cardiomyopathy, but the disease is treatable. Symptoms and complications can be well-controlled by medications, surgery, and implantable devices. The type of cardiomyopathy and its severity determines the treatment plan.

  • Medication is usually the first treatment option to improve the functioning of the heart.
  • An implantable cardioverter defibrillator (ICD) may be needed to regulate heartbeat in children with irregular heart rhythms (arrhythmia).
  • A surgical procedure called a myectomy may relieve some symptoms in children with HCM.
  • A heart transplant may be necessary if medical management is ineffective in controlling symptoms and preventing heart failure.

Genetic considerations:

Cardiomyopathy is often genetic, so it is important to assess a family’s inheritance risk and discuss family screening. A pediatric cardiologist and a pediatric geneticist should work together to form a screening plan and discuss genetic testing options.

How does the diagnosis impact a child’s lifestyle?

Although cardiomyopathy is a chronic condition, many children with cardiomyopathy lead a relatively normal life with few limitations. A diagnosis will likely involve more visits to the cardiologist and taking daily medications. Other adjustments may include restriction from competitive and contact sports, diet changes, and school accommodations.

About sudden cardiac arrest:

Often underdiagnosed, cardiomyopathy is the leading cause of sudden deaths in children under the age of 18. Because the disease can be present without symptoms, families should be aware of any family members who have heart disease or who died unexpectedly before age 50.

Warning signs of sudden cardiac arrest:

Recognizing the warning signs of sudden cardiac arrest is also important. If your child experiences any of the following, he or she should see a pediatric cardiologist for an extensive cardiac evaluation:

  • Chest pain or discomfort when being physically active
  • Unexplained fainting or near fainting
  • Excessive fatigue associated with exercise
  • Unusually fast heartbeat
Last Updated 9/26/2016

Parenting with a Congenital Heart Defect: Why Prioritizing Your Own Health is Important

Parenting with a Congenital Heart Defect: Why Prioritizing Your Own Health is Important

If you are a parent who lives with a congenital heart defect (CHD), it can be challenging to balance the day-to-day demands of being a mom or dad while living with and caring for your own health.

Who to Put First?

It may be difficult some days to figure out who to put first – your children or yourself. When it is hard to find the energy to even get out of bed, it is important to take care of your own physical and emotional needs first. As a parent, this may not sound “right,” but the best gift you can give your child is to also care for yourself.

A CHD can be unpredictable, as can your child’s needs. Plan ahead.

  • Ask for help. When a CHD overrules parenthood, call friends, family members or a sitter to help. Parenting changes with older children, but you still need to be okay with reaching out to others.
  • Find time to relax. Whether visiting with friends, seeing a movie, meditating, or just reading, it is essential to make relaxation a priority.
  • Lower your expectations. Your parenting style may be different from your sister’s or your friend’s, but that does not mean you are not a good parent. Lowering your expectations will help make meeting the challenges of parenting with a CHD seem easier.

Planning for the Future

Every person with a CHD should have a living will. It not only notifies your family members about your wishes, but it also guides them in decision making if you are unable to speak for yourself.

Once you have a living will, be sure to:

  • Store it in a safe place in your home and tell your family members where it is located. This is not a document to keep in the bank safety deposit box. It should be located in a secure but easily accessible spot in your home.
  • Talk to your family about the instructions included in your living will. Family members are more accepting of a loved one’s wishes if they are aware of what those are ahead of time.
  • Bring it to the hospital if you are admitted. Living wills are now required by hospitals so be sure to bring it with you if you are being admitted to the hospital

Additional information regarding congenital heart defects and lifelong cardiac care is available at the Congenital Heart Public Health Consortium (CHPHC) website, www.chphc.org. The CHPHC is housed at the American Academy of Pediatrics through a grant from the Centers for Disease Control and Prevention in an effort to utilize public health principles to affect change for those whose lives are impacted by a CHD. Organizational members of the Consortium represent the voice of providers, patients, families, clinicians and researchers.

Last Updated 11/7/2016

Irregular Heartbeat (Arrhythmia)

Irregular Heartbeat (Arrhythmia)

My child’s heart seems to beat very fast. Does she have an irregular heartbeat?

Your child’s heart rate normally will vary to some degree. Fever, crying, exercise, or other vigorous activity makes any heart beat faster. And the younger the child, the faster the normal heart rate will be. As your child gets older, her heart rate will slow down. A resting heart rate of 130 to 150 beats per minute is normal for a newborn infant, but it is too fast for a six-year-old child at rest. In a very athletic teenager, a resting heart rate of 50 to 60 beats per minute may be normal.

The heart’s regular rhythm or beat is maintained by a small electrical circuit that runs through nerves in the walls of the heart. When the circuit is working properly, the heartbeat is quite regular; but when there’s a problem in the circuit, an irregular heartbeat, or arrhythmia, can occur. Some children are born with abnormalities in this heart circuitry, but arrhythmias also can be caused by infections or chemical imbalances in the blood. Even in healthy children, there can be other variations in the rhythm of the heartbeat, including changes that occur just as a result of breathing. Such a fluctuation is called sinus arrhythmia, and requires no special evaluation or treatment because it is normal.

So-called premature heartbeats are another form of irregular rhythm that requires no treatment. If these occur in your child, she might say that her heart “skipped a beat” or did a “flip-flop.” Usually these symptoms do not indicate the presence of significant heart disease.

If your pediatrician says that your child has a true arrhythmia, it could mean that her heart beats faster than normal (tachycardia), very fast (flutter), fast and with no regularity (fibrillation), slower than normal (bradycardia), or that it has isolated early beats (premature beats). While true arrhythmias are not very common, when they do occur they can be serious. On rare occasions they can cause fainting or even heart failure. Fortunately, they can be treated successfully so it’s important to detect arrhythmias as early as possible.

Signs and symptoms

If your child has a true arrhythmia, your pediatrician probably will discover it during a routine visit. But should you notice any of the following warning signs between pediatric visits, notify your doctor immediately.

  • Your infant suddenly becomes pale and listless; her body feels limp.
  • Your child complains of her “heart beating fast,” when she’s not exercising.
  • She tells you she feels uncomfortable, weak, or dizzy.
  • She blacks out or faints.


It’s unlikely that your child will ever experience any of these symptoms, but if she does, your pediatrician will perform additional tests and perhaps consult with a pediatric cardiologist. In the process the doctors may do an electrocardiogram (ECG), to better distinguish a normal sinus arrhythmia from a true arrhythmia. An ECG is a tape recording of the electrical impulses that make the heart beat, and it will allow the doctor to observe any irregularities more closely.

Sometimes your child’s unusual heartbeats may occur at unpredictable times, often not when the ECG is being taken. In that case the cardiologist may suggest that your child carry a small portable tape recorder that continuously records her heartbeat over a one- to two-day period. During this time you’ll be asked to keep a log of your child’s activities and symptoms. Correlating the ECG with your observations will permit a diagnosis to be made. For example, if your child feels her heart “flutter” and becomes dizzy at 2:15 P.M. and the ECG shows her heart suddenly beating faster at the same time, the diagnosis of tachycardia will probably be established.

Occasionally irregular heartbeats will occur only during exercise. If that’s the case with your child, the cardiologist may have your youngster ride a stationary bicycle or run on a treadmill while her heartbeat is being recorded. When your child is old enough to participate in sports, ask your pediatrician if any special tests or restrictions are necessary.

Last Updated 11/21/2015

High Blood Pressure in Children

High Blood Pressure in Children

Can children get high blood pressure?

We usually think of high blood pressure, or hypertension, as a problem that affects adults. But, in fact, this condition can be present at any age, even in infancy. About 5 of every 100 children have higher than normal blood pressure, although fewer than 1 in 100 has medically significant hypertension.

How blood pressure is measured?

The term blood pressure actually refers to two separate measurements:

  • Systolic blood pressure is the highest pressure reached in the arteries as the heart pumps blood out for circulation through the body.
  • Diastolic blood pressure is the much lower pressure that occurs in the arteries when the heart relaxes to take blood in between beats.

If either or both of these measurements are above the range found in healthy people of the same age and sex, it’s called hypertension.


In many cases, hypertension seems to develop with age. As a result, your child may show no signs of high blood pressure as an infant, but may develop the condition as she grows.

Children who are overweight are much more prone to have hypertension (as well as other health problems). By age 7, more than 50% of hypertension is due to obesity and this rises to 85-95% by adolescent years. Thus, good eating habits (without overeating and without emphasizing high-fat foods) and plenty of physical activity are important throughout the early years of childhood (and for the rest of her life).

When hypertension becomes severe in children, it’s often a symptom of another serious problem, such as kidney disease or abnormalities of the heart or of the nervous or endocrine (gland) system.

Early detection is key

Fortunately, high blood pressure can be controlled through dietary changes, medication, or a combination of the two.

However, if hypertension is allowed to continue or become worse over many years, the prolonged extra pressure can lead to heart failure or stroke in adulthood. Also, long-term hypertension causes changes in blood vessel walls that may result in damage to the kidneys, eyes, and other organs. For these reasons it’s important for children with hypertension to have their blood pressure checked regularly by their pediatrician, and for you to follow the doctor’s treatment advice carefully.

Signs and symptoms

In most routine physical examinations, your child’s blood pressure will be measured. This is how hypertension is usually discovered. Most often this condition causes no noticeable discomfort, but any of the following may indicate high blood pressure:

  • Headache
  • Dizziness
  • Shortness of breath
  • Visual disturbances
  • Fatigue


If your child is found to have high blood pressure, your pediatrician will order tests to see if there is an underlying medical problem causing it. These tests include studies of the urine and blood. Sometimes special X-rays are used to examine the blood supply to the kidneys. If no medical problem can be found, your child will be diagnosed with essential hypertension. (In medical terms, the word essential refers only to the fact that no cause could be found.)

Managing your child’s high blood pressure

What will the doctor tell you to do? If obesity is the cause, the first step will be to have your child lose weight. This will need to be very closely monitored by your pediatrician. Not only will weight loss lower blood pressure, it can provide many other health benefits as well.

The next step toward reducing our child’s blood pressure is to limit the salt in her diet. Giving up the use of table salt and restricting salty foods can reverse mild hypertension and will help lower more serious blood pressure levels. You’ll also have to be cautious when shopping for packaged foods; most canned and processed foods contain a great deal of salt, so check labels carefully to make sure the items have little or no salt added.

Your pediatrician also may suggest that your child get more exercise. Physical activity seems to help regulate blood pressure and thus can reduce mild hypertension.


Once your pediatrician knows your child has high blood pressure, he’ll want to check it frequently to make sure the hypertension is not becoming more severe. Depending on how high the blood pressure is, the pediatrician may refer the child to a child hypertension specialist, usually a pediatric nephrologist (kidney specialist) or pediatric cardiologist (heart specialist). If it does become worse, it may be treated with medication as well as diet and exercise.

Many types of medications are available, which work through different parts of the body. When your child’s blood pressure is brought under control with diet or medication it is important to continue the treatment according to your doctor’s recommendations, including changes in diet, or the hypertension will return.


It’s very important to detect hypertension early. Uncontrolled long-standing hypertension can have damaging ef­fects on several other organs in the body such as the heart, kidneys, and brain.

It is now recommended that all children have their blood pressure checked beginning at age three, sooner for those at high risk. These include infants who were preterm, or of low birth weight, who had a difficult or prolonged hospital stay. It also includes children who have congenital heart disease, who are receiving medications that might increase blood pressure, or who have any other condition that might lead to high blood pressure.

Because overweight children are more likely to develop hypertension (as well as other health problems), watch your child’s caloric intake and make sure she gets plenty of exercise. Even relatively small decreases in weight or small increases in physical activity may prevent hypertension in overweight children.

Last Updated 11/21/2015


Heart Murmur

Heart Murmur

What is a heart murmur?

Technically, a heart murmur is simply a noise heard between the beats of the heart. When a doctor listens to the heart, she hears a sound something like lub-dub, lub-dub, lub-dub. Most often, the period between the lub and the dub and between the dub and the lub is silent. If there is any sound during this period, it is called a murmur. Although the word is unsettling, murmurs are extremely common, and usually normal (that is, the sounds are causes by a healthy heart pumping blood normally).

“Innocent” murmurs

In preschool and school-age children, heart murmurs are almost always not a concern; the children require no special care, and the sound eventually disappears. These children have “normal” or so-called functional or innocent heart murmurs.

If your child has such a murmur, it probably will be discovered between the ages of one and five during a routine examination. The doctor then will listen carefully to determine if this is a “normal” heart murmur or one that might indicate a problem. Usually, just by listening to its sound, the pediatrician will be able to tell if a murmur is innocent (normal). If necessary, she will consult a pediatric cardiologist to be certain, but additional tests are usually not necessary.

On rare occasions, a pediatrician will hear a murmur that sounds abnormal enough to indicate that something might be wrong with the heart. If the doctor suspects this, your child will be referred to a pediatric cardiologist to enable a precise diagnosis to be made.

Heart murmurs and infants

When do heart murmurs become a concern? When they occur very early at birth or during the first six months of life. These murmurs are not functional or innocent, and most likely they will require the attention of a pediatric cardiologist immediately. They may be due to abnormal connections between the pumping chambers (septal defects) or the major blood vessels coming from the heart (e.g., transposition of vessels). Your infant will be observed for changes in skin color (turning blue), as well as breathing or feeding difficulties. He also may undergo additional tests, such as a chest X-ray, electrocardiogram (ECG), and an echocardiogram. This echocardiogram creates a picture of the inside of the heart by using sound waves. If all of these tests prove normal, then it is safe to conclude that the baby has an innocent murmur, but the cardiologist and pediatrician may want to see him again to be absolutely certain. The cardiologist and pediatrician together will make a decision as to next steps depending on the results of these tests.

When a specific condition called patent ductus arteriosus (PDA) occurs, it is often detected shortly after birth, most commonly in premature babies. It is a potentially serious condition in which blood circulates abnormally between two of the major arteries near the heart, due to the failure of a blood vessel (the ductus arteriosis) between these arteries to properly close. In most cases, the only symptom of PDA is a heart murmur until the ductus closes on its own shortly after birth, which often happens in otherwise healthy, full-term newborns. Sometimes, especially in premature babies, it may not close on its own, or it may be large and permit too much blood to pass through the lungs, which can place extra strain on the heart, forcing it to work harder and causing a rise in blood pressure in the arteries of the lungs. If this is the case, a medication or, rarely, surgery may be needed to help close the PDA.


Innocent heart murmurs  are normal and therefore require no treatment. Children with these innocent heart murmers do not need repeated evaluation or long-term follow-up care from cardiologist, nor do they require restrictions on sports or other physical activities.

Innocent heart murmurs generally disappear by midadolescence. Cardiologists don’t know why they go away, any more than we know why they appear in the first place. In the meantime, don’t be discouraged if the murmur is softer on one visit to the pediatrician and loud again on the next. This may simply mean that your child’s heart is beating at a slightly different rate each time. Most likely the murmur will go away eventually.

Patent ductus arteriosus is a selfcorrecting problem in some cases, or medications can be used to close a PDA. But if the ductus arteriosus remains open, it may need to be corrected surgically or with a catheter.

If other, more serious, heart conditions are diagnosed from birth or shortly thereafter, and the evaluation reveals more serious defects, the pediatric cardiologist and pediatrician will consult a pediatric cardiac surgeon at a regional Pediatric Cardiac Center where complete pediatric cardiac diagnostic and intervention capabilities exist.

Last Updated 11/21/2015

Genetics and Congenital Heart Defects

Genetics and Congenital Heart Defects

A congenital heart defect (CHD) is the most common type of birth defect. It is estimated that 2 to 3 million people in the United States have a CHD.

Over the last few decades, treatments for CHDs have improved—meaning more children born with a CHD are growing up to have children of their own.

If you have a CHD, you might wonder what the risk is for your child to also have a CHD. You might also wonder whether pregnancy will affect your health. These are very important questions to discuss with your congenital cardiologist and perhaps other specialists, such as a maternal-fetal-medicine specialist and a geneticist or genetic counselor.

What causes a congenital heart defect?

CHDs are caused by genetics, the environment, or a combination of both. Some known genetic causes include:

  • Chromosome abnormalities: Extra or missing chromosomes.
  • Gene mutation: A change to the DNA sequence or order that makes up a gene.

About genetic testing:

There are two types of tests used with genetic testing: chromosomal and DNA. Recent advances in these tests have made it more possible to find the genetic cause of CHDs for many patients. Sometimes, however, a genetic change cannot be identified, but the cause can still be genetic.

How to reduce your baby’s risk of a congenital heart defect:

If you have a CHD, your baby has a higher risk of being born with a CHD. In fact, there is about a 5% chance your child will have a CHD, too. A geneticist or genetic counselor can help you determine if genetic testing might be helpful for you prior to becoming pregnant. See Preconception Counseling for Women with a Congenital Heart Defect for more information.

You might be asking yourself:

  • What can I do to lower my baby’s chance of being born with a CHD?
  • How might I be able to improve my baby’s outcome if he or she has a CHD? 

These are important questions to ask your medical care team, but some research suggests taking folate prior to conception and during pregnancy may decrease the risk of a CHD.

How a congenital heart defect is diagnosed in an unborn baby:

A CHD can be diagnosed very early in pregnancy (around 18 to 20 weeks) via an ultrasound of the baby’s heart, called a fetal echocardiogram.

If you have a CHD caused by a genetic change, your maternal-fetal-medicine specialist can perform either an amniocentesis or chorionic villus sampling to determine whether your unborn baby has the same genetic change. Both of these tests are done by inserting a needle into the womb to get a small tissue sample for testing

Additional information regarding congenital heart defects and lifelong cardiac care is available at the Congenital Heart Public Health Consortium (CHPHC) website, www.chphc.org. The CHPHC is housed at the American Academy of Pediatrics through a grant from the Centers for Disease Control and Prevention in an effort to utilize public health principles to affect change for those whose lives are impacted by a CHD. Organizational members of the Consortium represent the voice of providers, patients, families, clinicians and researchers.

Last Updated 11/7/2016

Common Heart Defects

Common Heart Defects

Congenital heart defects are structural problems arising from abnormal formation of the heart or major blood vessels. At least 1% of all live-born infants have a heart defect. The cause of a heart defect is most often unknown. Some defects may be small and may cause your child few problems; others are life-threatening.

Types of Congenital Heart Defects

There are 18 types of congenital heart defects recognized and many additional anatomic variations. The more common heart defects are listed below:

  • Atrial Septal Defect (ASD)
  • Atrioventricular Canal Defect
  • Coarctation of the Aorta (CoA)
  • Hypoplastic Left Heart Syndrome (HLHS)
  • Pulmonary Atresia
  • Pulmonary Stenosis (PS)
  • Tetralogy of Fallot
  • Total Anomalous Pulmonary Venous Connection (TAPVC)
  • Transposition of the Great Arteries
  • Tricuspid Atresia
  • Truncus Arteriosus
  • Ventricular Septal Defect

Outlook for Children Born with a Heart Defect

Even if your baby is born with a heart defect, it is very likely nowadays that he or she can live a normal adult life and overcome the problem. The many advances in the diagnosis and treatment have made it possible to correct defects via surgery and heart catheterization. Even the most difficult cases have hope now.

The diagnosis and treatment for other heart defects will continue to improve with new advancements and research. Talk with your child’s pediatric heart surgeon if you have specific questions about your child’s condition, treatment options, and expected outcomes.

Last Updated 11/21/2015

Challenges Faced by Parents of Children with Congenital Heart Disease

Challenges Faced by Parents of Children with Congenital Heart Disease

The first thing most parents want to know about their unborn baby is whether the baby is healthy. From the moment parents hear the words, Your child has a heart defect,” they are thrust into a world they were not expecting. 

Even before the child is born, the joy of giving birth is often coupled with so many unique and complicated feelings—ranging from fear, guilt, sadness, shame, and confusion. Many feel ashamed of these feelings and try to suppress them, but it is important to remember these feelings are common and expected.

About Congenital Heart Defects

  • Congenital Heart Disease (CHD) is the most common type of birth defect affecting 8 out of every 1,000 newborns.
  • Each year, about 35,000 babies in the Unites States are diagnosed with CHD. Nearly 25% of those are critical congenital heart defects—ones that require surgery or other interventions within the first year of life to survive. Parents of these children often sit by their child’s side in the hospital hoping and praying their child survives.
  • As a result of dramatic advances in the medical and surgical management of CHD, 85% of infants with CHD are now expected to survive to adulthood, and CHD is regarded as a chronic disease rather than a terminal one. However, survival rates for children with critical congenital heart defects is lower; they often need specialized medical care throughout their lifetime.

Understanding Unique Parenting Challenges

All parents are caregivers, but parents of children with CHD have extra challenges.

Learning “normal” and more specialized parenting skills:

  • Aside from the typical stresses and adjustments new parents face, in most cases, parents who have a baby with CHD are also struggling to care for a baby who may have spent months in the Neonatal Intensive Care Unit (NICU) and may have tubes or attachments to his or her tiny body.
  • When babies have open-heart surgeries, their ability to learn to eat can be compromised. Some babies have to be fed through a feeding tube. In addition to feeding issues, many children with CHD have trouble gaining weight and must go on special high-calorie diets.

Becoming an expert and advocate.

  • Knowledge is power. Parents often immerse themselves into learning as much as they possibly can about their child’s diagnosis, medication, and treatment plan. It is important to avoid “internet overload” by sticking to reputable websites. See the additional resources at the end of this article. 

Making changes to safeguard their child’s health:

  • Infants and children with CHD have weaker immune systems than the average child. They can end up in the hospital for illnesses that others recover from on their own. Thus, many parents take extra precautions to try to keep their child healthy. This may mean having serious talks with friends and family about getting their annual flu shot and a Tdap shot to prevent whooping cough. See How to Cocoon a Newborn: Only an E-Mail Away! for more information and tips.
  • As their child grows, parents must strike a balance between letting their child live “normally” and protecting them. Walking the line between healthy limits and over-protectiveness is an ongoing concern.

Struggling with the financial component of care:

  • For a child with CHD, surgical costs can be astronomical, even with health insurance. The costs of medicines and doctor appointments add up quickly, too. Many children with CHD are on multiple medications, some taken several times a day. It’s not unusual for children who’ve had open-heart surgery to come home on six or more different medications.

Caring for their other children:

  • If there are siblings in the family, parents must also take their needs into consideration. Young children, especially, may have fears and anxieties about their brother or sister’s condition that they aren’t voicing. It is important to spend one-on-one time with each child and time together as a family that isn’t focused on the CHD.

Educating the school and others:

  • Often, sending a child with CHD to preschool or school, or even having siblings in school, can pose challenges for parents. Again, avoiding major illnesses is a concern. Educating school staff about CHD and their child’s limitations can also be difficult. Sometimes, parents get everyone together who will be working with their child, so that they all understand the condition or defect.

Living with persistent uncertainty:

  • Even if their child doesn’t need any more procedures, there will always be doctor appointments that will inevitably dredge up some old memories and new fears. As children grow, it is also natural for parents to feel fear about the inherent risks of CHD and the knowns and unknowns about their child’s lifelong care. The journey is never over, so support plays a big role in these parents’ lives.

Why Support is So Important

Parents need for all kinds of support – informational, emotional, and instrumental – to be able to face the stress of caring for a child with CHD.

Connecting with other parents of children with a CHD:

  • Parents and other family members are encouraged to connect with others who also have a child in their family with a heart defect. Other parents, for example, can provide a credible model of how to cope in a positive way with exceptional life circumstances.

Talking to a trained mental health professional:

  • Parents who seek the assistance of mental health professionals typically experience reductions in stress, depression and anxiety, as well as improved sleep and life satisfaction.

The Silver Lining

Life with a child diagnosed with CHD can be overwhelming, stressful, and in some cases tragic. However, some families have recognized a silver lining in having a family member with a complex medical condition like CHD. For example, siblings can be more resilient—demonstrating increased compassion, a greater sense of wonder and joy, and a greater appreciation for life. Many parents’ outlook on life and priorities can change, as well, to appreciate the “little things in life” a lot more.

Additional information regarding congenital heart defects and lifelong cardiac care is available at the Congenital Heart Public Health Consortium (CHPHC) website, www.chphc.org. The CHPHC is housed at the American Academy of Pediatrics through a grant from the Centers for Disease Control and Prevention in an effort to utilize public health principles to affect change for those whose lives are impacted by CHD. Organizational members of the Consortium represent the voice of providers, patients, families, clinicians and researchers.

Last Updated 5/19/2016

Cardiac Conditions in Teens

Cardiac Conditions in Teens

Teenagers frequently experience chest pain. Rarely, though, is the symptom related to the heart. Nevertheless, such complaints should be brought to the attention of your pediatrician, who will diagnose the problem through process of elimination.

“Usually, just asking the patient questions points us to the source of the pain,” says Dr. Reginald L. Washington, a pediatric cardiologist from Denver. Among the red flags he looks for are chest pain upon physical exertion and chest pain accompanied by dizziness. “Those situations would warrant further evaluation,” he says, “but most of the time the cause turns out to be anxiety, too much caffeine, asthma, muscle strain or costochondritis.” The latter condition, an inflammation of the chest wall, is treated with rest, over-the counter anti-inflammatory medications and heat.

High Blood Pressure (Hypertension) and High Blood Cholesterol (Hypercholesteremia)

Doctors once believed that virtually all high blood pressure in youngsters was a complication of another medical condition, such as kidney disease. We now know that even infants can develop primary hypertension, also referred to as essential hypertension. Abnormally elevated levels of the fatlike substance cholesterol in the circulation are also sometimes seen in teenagers. Either problem can pave the way for a future heart attack, cerebral stroke, renal failure, or blindness, among other conditions.

Symptoms that Suggest Primary Hypertension or Hypercholesteremia Include:

  • Dizziness and/or headache from high blood pressure
  • High cholesterol does not cause symptoms in teenagers

How Hypertension and Hypercholesteremia are Diagnosed

Diagnostic measures include physical examination and thorough medical history, plus one or more of the following procedures: (1) multiple blood pressure readings (2) cholesterol blood test.

In teenagers, an abnormally high blood-pressure measurement calls for a second reading, then several additional readings over the course of other office visits before a definitive diagnosis is made. The first number represents the systolic pressure: the peak pressure within the blood vessels when the heart contracts. The second number expresses the diastolic blood pressure reached when the heart relaxes between beats. If the blood pressure remains elevated, more extensive laboratory tests would be  ordered, along with an electrocardiogram (EKG).

Memo to Mom and Dad: Stress can contribute to hypertension. As parents of a teenager, have you had your blood pressure taken lately?

How Hypertension and Hypercholesteremia are Treated

Improved diet and exercise: Both hypertension and hypercholesteremia are uncommon among teenagers. Often, studying the family tree reveals a genetic thread entwined throughout its branches. About half of all youngsters diagnosed with hypertension and two-thirds of those with high cholesterol have a hereditary predisposition to the disorder. “The rest of the cases are due to poor dietary practices and a lack of physical activity,” says Dr. Washington. “In general, those are the easiest to treat. If they learn to eat healthily and engage in aerobic exercise, almost all of them can get their blood pressure and cholesterol down into a normal range.” Even if your youngster’s blood pressure and cholesterol level are normal, we advise following the sensible eating plan. Getting into the habit of choosing foods that are low in saturated fat, cholesterol and salt may help prevent cardiovascular disease later in life.

Drug therapy: Adolescents who fall into the high-risk categories for hypertension or hypercholesteremia may require medication in addition to modifying their diet and lifestyle. Agents typically used to bring down the concentration of cholesterol in the blood include cholestyramine, clofibrate and statins.

To lower blood pressure, a pediatrician might prescribe a diuretic in combination with an agent from one of four families: beta-blockers, angiotensinconverting-enzyme inhibitors or ACE inhibitors; calcium-channel blockers; and alpha-beta blockers. Diuretics, also known as “water pills,” act on the kidneys to flush excess water and sodium (salt) from the body. The other medications reduce blood pressure through various mechanisms.

Helping Teenagers to Help Themselves

Youngsters with or without hypertension or hypercholesteremia should be encouraged to:

    • Maintain a healthy weight.
    • Engage in aerobic exercise for at least thirty minutes per day.
    • Abstain from using tobacco or alcohol.
    • Learn techniques for relieving stress, be it exercise, deep breathing or meditation.
    • Consult the pediatrician if considering taking birth-control pills. Although neither condition is common in healthy teens, oral contraceptives can worsen both high blood pressure and high blood cholesterol.

Mitral Valve Prolapse (MVP)

Four flap like valves regulate the flow of blood through the heart’s four chambers. The mitral valve is situated in the left side of the heart, the side that receives freshly oxygenated blood from the lung, then pumps it out to the circulation. Under normal circumstances, the valve opens to let blood pass from the upper left chamber (the left atrium) into the lower left chamber (the left ventricle). Approximately one in eight otherwise healthy adolescents and young adults are found to have mitral valve prolapse. For reasons that remain unclear, one or both flippers billows out (prolapses) into the upper chamber instead of snapping shut. This can produce a clicking sound, audible through the pediatrician’s stethoscope. Occasionally, blood may leak backward into the atrium, causing a murmur.  “The sounds usually come and go,” says Dr. Washington. Some youngsters with mitral valve prolapse have both a click and a murmur, while others have no abnormal heart noises at all.

Symptoms that Suggest Mitral Valve Prolapse May Include:

  • Abnormal heart murmur
  • Fluttering sensation in the chest, as if the heart were racing or skipping a beat
  • Shortness of breath
  • Headache
  • Sharp, fleeting chest pain

Nineteen out of twenty people with mitral valve prolapse do not exhibit any symptoms; the condition is discovered during a routine physical.

How Mitral Valve Prolapse is Diagnosed

Physical examination and thorough medical history, plus one or more of the following procedures:

  • Stethoscopic exam
  • echocardiogram
  • chest X ray

How Mitral Valve Prolapse is Treated

“The vast majority of people with mitral valve prolapse can go about their lives without having to worry about the condition or restrict athletic activities,” explains Dr. Washington. Under certain circumstances, however, a leaky mitral valve can become infected. To reduce the risk of endocarditis, rare though it may be, the current recommendation is for antibiotics to be taken just prior to dental appointments and surgical procedures.

Helping Teenagers to Help Themselves

Reducing caffeine consumption can help alleviate heart palpitations. Remember that caffeine is present not only in coffee but in soda, chocolate and tea.

Heart Murmur: Language of the Heart

“A heart murmur,” explains Dr. Washington, “is merely the sound of the heart vibrating slightly or valves opening and closing as blood flows through it. There are probably fifty different types, each with its own distinctive sound. Most of them,” he emphasizes, “are perfectly normal.” As many as four in five young people have these “innocent” murmurs now and then. Unless the murmur occurs during a doctor’s visit, it generally goes undetected. An abnormal murmur, which a pediatrician can identify with a stethoscope, indicates a heart condition that may require further diagnostic testing, possibly by a pediatric cardiologist. Possible causes include infections of the heart (endocarditis, myocarditis), faulty valves and cardiac defects that had been present since birth. Normal heart murmurs don’t call for limits on activities. With abnormal murmurs, says Dr. Washington, “the underlying cause will determine whether restrictions are necessary.”


Birth Control for Young Women with a Congenital Heart Defect

Birth Control for Young Women with a Congenital Heart Defect

Understanding the risks and benefits of different birth control methods is important for all women. For women with a congenital heart defect (CHD), choosing the right birth control is critical to maintaining a healthy heart.

Questions to Ask Yourself:

Selecting birth control when you have a CHD requires you to think about all aspects of your life.  Some typical questions might be:

  • Do I ever want to become pregnant? If so, when might that be?
  • Will pregnancy be too risky with my heart condition? 

Some women with a CHD have a very high risk for medical complications if they become pregnant. If this is you, it is important to use a very effective form of birth control or to consider permanent sterilization.

If pregnancy could be dangerous to your health but you would like to have a family, talk to your doctor about other options, such as adoption or surrogacy. Deciding not to have children is a perfectly acceptable option, too, if that is what you choose.

Types of Birth Control:

Women with a CHD who are able to have a safe or low risk pregnancy but are not ready to become pregnant yet have many options for birth control.

  • Some methods are more effective than others and some also last longer than others.
  • Some methods contain the hormones estrogen and/or progesterone (e.g. oral medications, skin patches, vaginal rings, implants).
  • Some forms of birth control use a “barrier” method to block sperm from entering the uterus (e.g. condoms, copper intrauterine devices or IUDs, diaphragms).

Benefits & Risks of Birth Control Methods:

There are different risks and success rates for the various forms of birth control.

  • Condoms have little physical risk to women. They are also highly effective at protecting against sexually transmitted diseases. However, they do not always work well at preventing pregnancy. In fact, they have a failure rate as high as 30%.
  • Birth control pills are usually reliable at preventing pregnancy. However, some forms bith control pills contain estrogen which can increase the risk of stroke and other medical complications related to the increased risk of blood clots. Estrogen is not recommended if you have a CHD and are at increased risk of forming blood clots, especially if you have an artificial heart valve or have had a Fontan operation.
  • Progesterone-only birth control methods have a lower risk of blood clots. Progesterone can be taken as a pill, placed as an implant under the skin, given as monthly injections or used in an IUD. A progesterone pill must be taken at the same time every day. Progesterone forms of birth control are often good choices if you have a CHD. They can be highly effective when they are used the right way.
  • Progesterone IUD is a very effective method if you do not want to become pregnant for at least a few years.
  • Two forms of birth control are recommended if you are sure you never want to become pregnant or are at very high risk for a dangerous pregnancy. You may consider a tubal ligation or other permanent form of birth control, as well. If you have a long-term male partner, you may consider asking him to have a vasectomy.

Talk to your gynecologist about which form of birth control is best for you based on the recommendations of your congenital cardiologist.

Additional information regarding congenital heart defects and lifelong cardiac care is available at the Congenital Heart Public Health Consortium (CHPHC) website, www.chphc.org. The CHPHC is housed at the American Academy of Pediatrics through a grant from the Centers for Disease Control and Prevention in an effort to utilize public health principles to affect change for those whose lives are impacted by a CHD. Organizational members of the Consortium represent the voice of providers, patients, families, clinicians and researchers.