Restrictive Cardiomyopathy

Restrictive cardiomyopathy is the least common form of cardiomyopathy. The heart's ventricles become rigid because abnormal tissue, such as scar tissue, replaces the normal heart muscle. As a result, the ventricles can't relax normally and fill with blood, and the atria become enlarged. Blood flow in the heart is reduced over time. This can lead to problems such as heart failure or arrhythmias.

Other types of restrictive cardiomyopathy

  • Non-infiltrative cardiomyopathy
  • Infiltrative cardiomyopathy
  • Storage diseases
  • Endomyocardial disorders

What causes restrictive cardiomyopathy?

Certain diseases, conditions and factors can cause restrictive cardiomyopathy, including:

  • Hemochromatosis: A disease in which too much iron builds up in your body. The extra iron is toxic to the body and can damage the organs, including the heart.
  • Sarcoidosis: A disease that causes inflammation and can affect the body's organs. Researchers believe that an abnormal immune response may cause sarcoidosis. The abnormal response causes tiny lumps of cells to form in the body's organs, including the heart.
  • Hypereosinophilic syndrome: A condition in which white blood cells may accumulate in the heart muscle.
  • Amyloidosis: A disease in which abnormal proteins build up in the body's organs, including the heart.
  • Scarring of the heart for unknown reasons, may be due to accumulation of abnormal substances in body. This may be from medications used to treat diseases.

Restrictive cardiomyopathy in children

The first symptoms of RCM in children often seem related to problems other than the heart. Children with RCM may have a history of repeated lung infections or asthma.

Restrictive cardiomyopathy is among the rarest of childhood cardiomyopathies. Its diagnosis is difficult to establish early in the condition due to the lack of symptoms. In many cases the diagnosis of RCM is made only after the presentation of symptoms, such as decreased exercise tolerance, new heart sound (gallop), swelling, syncope (passing out) or chest pain with exercise. Once suspected, certain tests can help confirm this diagnosis.

The diagnosis of RCM affects several areas of a child’s life. Specific recommendations should be developed by the child’s health care team.