What is restrictive cardiomyopathy?

Restrictive cardiomyopathy, the least common type of cardiomyopathy in the U.S., occurs when the myocardium of the ventricles becomes excessively rigid, and the filling of the ventricles with blood between heart beats is impaired. This condition occurs rarely in children.

Restrictive cardiomyopathy usually results from an underlying condition that affects the rest of the body. However, there are idiopathic (cause unknown) occurrences as well. Restrictive cardiomyopathy does not appear to be inherited, but some of the diseases that lead to the condition are genetically transmitted.

Conditions that may be associated with the development of restrictive cardiomyopathy include, but are not limited to, the following:

  • Scleroderma (a chronic, degenerative disease that affects the joints, skin, and internal organs)

  • Amyloidosis (a rare disease which causes the buildup of amyloid, a protein and starch, in tissues and organs)

  • Sarcoidosis (a rare inflammation of the lymph nodes and other tissues throughout the body)

  • Mucopolysaccharidosis (a condition in which mucopolysaccharides, or carbohydrates that bond with water to form a thick, jelly-like substance, accumulate in body organs)

  • Cancer

Radiation therapy for cancer treatment has also been associated with restrictive cardiomyopathy.

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

  • Shortness of breath on exertion

  • Chest pain

  • Weakness

  • Swelling of the extremities

Specific treatment for restrictive cardiomyopathy will be determined by your child's health care provider 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 health care provider may recommend medications to accomplish the following:

  • Decrease the workload of the heart

  • Decrease the oxygen requirements of the heart

  • Prevent blood clots from forming

  • Regulate irregular heartbeats

Surgical treatment may include:

Consult your child's health care provider for more information regarding the specific outlook for your child.

For more information or to schedule an appointment, call 314.454.5437 or 800.678.5437 or email us