Cardiomyopathy

Care for children with cardiomyopathy is complex and requires the expertise of a pediatric cardiologist, and possibly a heart surgeon, electrophysiologist or a geneticist. Treatment depends on the type of cardiomyopathy and how soon it is detected. At early stages, medications and a pacemaker or defibrillator may be recommended. Having an experienced physician team such as the one at Herma Heart Center is critical since children with advanced cardiomyopathy may require surgery or heart transplantation. Read more.

What is cardiomyopathy?

Cardiomyopathy is any disease of the heart muscle in which the heart loses its ability to pump blood effectively. In some instances, heart rhythm is disturbed, leading to irregular heartbeats, or arrhythmias. There may be multiple causes of cardiomyopathy, including viral infections. Sometimes, the exact cause of the muscle disease is never found.

How does cardiomyopathy differ from other heart disorders?

Cardiomyopathy differs from many of the other disorders of the heart in several ways, including the following:

  • Cardiomyopathy can, and often does, occur in the young.
  • The condition is fairly uncommon, affecting only about 50,000 Americans (adults and children).
  • Cardiomyopathy is a leading cause for heart transplantation.
  • The condition tends to be progressive and sometimes worsens fairly quickly.
  • It may be associated with diseases involving other organs, as well as the heart.

Why is cardiomyopathy a concern?

Cardiomyopathy prevents the heart muscle from pumping enough blood to meet the body's needs.

What causes cardiomyopathy?

Viral infections that infect the heart are a major cause of cardiomyopathy. In some instances, cardiomyopathy is a result of another disease or its treatment, such as complex congenital (present at birth) heart disease, nutritional deficiencies, uncontrollable, fast heart rhythms, or certain types of chemotherapy for childhood cancers. Sometimes, cardiomyopathy can be linked to a genetic abnormality. Other times, the cause is unknown.

What types of cardiomyopathy commonly affect children?

The following types of cardiomyopathy commonly affect children:

Dilated cardiomyopathy

Dilated cardiomyopathy is the most common form of cardiomyopathy. The heart muscle is enlarged and stretched (dilated), causing the heart to become weak and pump inefficiently. Other problems that may occur with dilated cardiomyopathy include the following:

  • Irregular heart rhythms. 
  • Risk of blood clots. 
  • Congestive heart failure.

Various infections (including viral) which lead to an inflammation of the heart muscle (myocarditis) can cause this type of cardiomyopathy.

Contact with toxins or very powerful therapeutic drugs, such as certain types of chemotherapy given to fight cancer, have been known to cause dilated cardiomyopathy. Heredity can also be a factor. Twenty percent of people with dilated cardiomyopathy have a parent or sibling with the disease. In many cases, a specific cause for this type of the disease is never identified.

Because the heart muscle is weak and unable to pump enough blood to meet the body's demands, the body tries to preserve blood flow to essential organs such as the brain and kidneys by reducing blood flow to other areas of the body, such as the skin and muscles.

The following are the most common symptoms of dilated cardiomyopathy. However, each child may experience symptoms differently. Symptoms may include: 

  • Pale or ashen skin color. 
  • Cool, sweaty skin. 
  • Rapid heart rate. 
  • Rapid breathing rate. 
  • Shortness of breath. 
  • Fatigue. 
  • Irritability. 
  • Chest pain. 
  • Poor appetite. 
  • Slow growth.

Specific treatment for dilated cardiomyopathy will be determined by your child's physician based on: 

  • Your child's age, overall health, and medical history. 
  • Extent of the disease. 
  • Your child's tolerance for specific medications, procedures, or therapies. 
  • Expectations for the course of the disease. 
  • Your opinion or preference.

Your child's physician may recommend medications to accomplish the following: 

  • Help the heart beat more effectively.
  • Decrease the workload of the heart. 
  • Decrease the oxygen requirements of the heart. 
  • Prevent clots from forming.
  • Decrease inflammation of the heart.
  • Regulate irregular heartbeats.

In some cases, dilated cardiomyopathy due to viral causes improves. In other cases, the condition worsens and transplantation of the heart may be considered. Consult your child's physician for more information regarding the specific outlook for your child.

Hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy is also known as hypertrophic obstructive cardiomyopathy, HOCM; asymmetric septal hypertrophy, ASH; or idiopathic hypertrophic subaortic stenosis, IHSS.  In this type of cardiomyopathy, the muscle mass of the left ventricle of the heart is larger than normal, or the wall between the two ventricles (septum) becomes enlarged. These abnormalities obstruct the blood flow from the left ventricle.

The thickened muscle or septal wall can also affect one of the leaflets of the mitral valve, which separates the left atrium and the left ventricle. The valve leaflet becomes leaky, allowing blood to move backwards from the left ventricle into the left atrium, instead of forward to the body.

Hypertrophic cardiomyopathy is often hereditary. One-half of children with the disease have a parent or sibling with varying degrees of left ventricular muscle or ventricular wall enlargement, although relatives may or may not have symptoms.

Children with hypertrophic cardiomyopathy may have symptoms that increase with exertion or symptoms may be unpredictable.

The following are the most common symptoms of hypertrophic cardiomyopathy. However, each child may experience symptoms differently. Symptoms may include:

  • Shortness of breath on exertion.
  • Dizziness.
  • Fainting.
  • Chest pain.
  • Abnormal heart rhythms.

Specific treatment for hypertrophic cardiomyopathy will be determined by your child's physician based on:

  • Your child's age, overall health, and medical history.
  • Extent of the disease.
  • Your child's tolerance for specific medications, procedures, or therapies.
  • Expectations for the course of the disease.
  • Your opinion or preference.

Your child's physician may recommend medications to accomplish the following:

  • Decrease the workload of the heart.
  • Decrease the oxygen requirements of the heart.
  • Regulate irregular heartbeats.

Surgical treatment may include:

  • Removal of part of the enlarged muscle.
  • Artificial pacemaker.
  • Heart transplantation.