Cardiomyopathy in Children and Teens

​​Rare and often underdiagnosed, cardiomyopathy is the leading cause of sudden cardiac death 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.

Read on for frequently asked questions from the American Academy of Pediatrics ​about cardiomyopathy in children. 

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.

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 during physical activity
Fainting or near fainting without warning or during physical activity 
Seizure without warning or during physical activityExcessive fatigue associated with exercise
Unusually fast heart rate or rhythm with an unknown cause

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.


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.  ​