Overview

What is cardiomyopathy?

Cardiomyopathy is a very rare form of heart disease, affecting one in every 100,000 children in the United States. If a child has cardiomyopathy, the heart muscle can’t pump blood normally – which can lead to heart failure.

There are several forms of cardiomyopathy:

• Dilated cardiomyopathy is the most common form of cardiomyopathy in children. Children with dilated cardiomyopathy have enlarged chambers of the heart that can’t pump a normal amount of blood out of the heart. The heart is weakened because the muscle cells that make up the wall of the two lower chambers (ventricles) have stretched, enlarged and become weakened.
• Hypertrophic cardiomyopathy is a condition of abnormally thickened heart muscle in one or more of the ventricles. Sometimes the wall of the heart can become so thick it blocks blood flow out of the heart. The muscle cells are also arranged in a disorganized manner, which leads to abnormal relaxation and abnormal heart rhythms.
• Left ventricular non-compaction is a condition of abnormal organization of the heart muscle in which the heart muscle appears “spongy.” A heart with this condition can have difficulty squeezing, relaxing, or both. Abnormal heart rhythms are also common with this condition.
• Restrictive cardiomyopathy prevents the muscle cells of the heart from relaxing, so they remain rigid, making it difficult for the heart to fill with blood returning from the body and lungs. Children with restrictive cardiomyopathy often develop high blood pressure in the arteries that deliver blood to the lungs and must be followed closely.
• In arrhythmogenic right ventricular cardiomyopathy, muscle cells degenerate and are replaced by fat cells. The right ventricle cannot pump blood normally and patients can suffer from abnormal heart rhythms.

Although all forms of cardiomyopathy can occur in children, restrictive and arrhythmogenic right ventricular cardiomyopathy are extremely rare, especially in children.

In all forms of cardiomyopathy, abnormal and sometimes lethal heart rhythms can occur.

Who gets cardiomyopathy?

There are a variety of causes and reasons a child might develop cardiomyopathy such as:

• A viral infection
• A child may be born with an abnormal heart structure that is not repaired in a timely fashion
• Certain metabolic conditions, which are related to how the body creates and uses energy
• Unrecognized, long-standing abnormal heart rhythms
• Some medications
• Some genetic conditions and conditions associated with syndromes
• Nutritional deficiencies
• Exposure to toxins, such as some types of chemotherapy for cancer

Some cardiomyopathies are familial, which means they may occur in the same family. But often, despite extensive evaluation, an underlying cause of cardiomyopathy cannot be identified.

Learn more about the Cardiomyopathy Program at Children's Hospital Colorado.

Programs & Treatments

How can cardiomyopathy be treated?

Treatment usually tries to correct the underlying cause of the cardiomyopathy. Treatment could include surgical correction of a heart defect, control of an abnormal heart rhythm, or replacement of nutritional deficiencies.

At the Cardiomyopathy Program at Children’s Hospital Colorado, one of our most important goals of treatment is maximizing our patients' quality of life.

Medication

Medical therapy varies with the different forms of cardiomyopathies. Patients may need no medication, or they may need several medications to best manage their symptoms and disease depending on the specific type of cardiomyopathy.

Medical devices

If a child is considered to have a high risk for a life-threatening abnormal heart rhythm known as arrhythmia, placement of an internal cardiac defibrillator (ICD) may be necessary. In those patients who develop life-threatening abnormal heart rhythms, an ICD is the only modality known to prevent sudden cardiac death.

Mechanical circulatory support with artificial heart pumps such as ventricular assist devices (VAD) and extracorporeal membrane oxygenation (ECMO) are utilized in children with severe heart failure as a bridge to transplantation.

Heart transplant

Frequent cardiac catheterizations are necessary to monitor the blood pressure in the lungs. For some children, there is no repair or medication that can help the failing heart or rising blood pressure. In these cases, a heart transplant may be an option. A transplant occurs when a failing heart is replaced by a healthy heart from an organ donor. One out of five kids with cardiomyopathy requires a transplant within a year of their diagnosis.

Although doctors have not yet found a cure for pediatric cardiomyopathy, they continue to find new ways to help patients manage this condition.

Learn why our Heart Institute is one of the top pediatric heart transplant centers in the nation.

Want to learn more about cardiomyopathy?

Check out these websites for more information:

• The Cardiomyopathy Program at Children's Hospital Colorado
• Children’s Cardiomyopathy Foundation
• The American Heart Association
• The Cardiomyopathy Association
• Hypertrophic Cardiomyopathy Association

Signs & Symptoms

What are the signs and symptoms of cardiomyopathy?

Because this disease varies from child to child, the signs of cardiomyopathy can be hard to predict. Children with cardiomyopathy may have swelling of the extremities or around the eyes. This happens when body fluids build up because the heart is not pumping hard enough or because the heart is stiff, making it difficult for blood to return from the body or lungs to the heart.

Infants may breathe heavily and rapidly while feeding, sweat excessively (especially when feeding), have a poor appetite and gain weight slowly. Older children may become out of breath easily, have decreased exercise endurance, or have abdominal pain or chest pain. Coughing, fainting, easy fatiguability, rapid breathing, rapid heart rate or pale appearance may also occur.

Diagnosis & Tests

How is cardiomyopathy diagnosed?

In order to treat your child’s condition, your pediatric cardiologist at Children’s Hospital Colorado will want to determine the cause, type and extent of your child’s cardiomyopathy. There are several tests routinely used in diagnosis and to plan the best course of treatment:

• Blood test
• Chest x-ray
• EKG
• Holter and event monitors
• ECHO
• Exercise or stress test
• Cardiac catheterization
• Cardiac MRI