26
Nov

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
26
Nov

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
26
Nov

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.

Diagnosis

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