Cardiac Center

Cardiomyopathy

Before you begin reading about cardiomyopathy, please read the explanation of how the normal heart works for a basic understanding of its structure and function.

What is cardiomyopathy?

Cardiomyopathy is a disease of the heart muscle (myocardium). The muscle becomes abnormally thick, stiff or enlarged, affecting the heart’s ability to fill or pump blood and maintain its rhythm.

Sometimes, particularly when adults develop cardiomyopathies, the disease is caused by factors such as diabetes, a previous heart attack or certain chemotherapy drugs. However, in many cases, in both adults and children, doctors don’t know what causes cardiomyopathies.

When children develop cardiomyopathies, there is often not an identifiable factor such as another illness. Sometimes the cardiomyopathy is inherited (passed down through families because of a genetic difference).

More rarely in children, the cause of cardiomyopathy is identifiable. A heart infection called myocarditis, a protein abnormality in the heart muscle, chemotherapy drugs, metabolic disorders or muscle disorders are among the factors that can cause cardiomyopathy in a child or teenager.

There are different types of cardiomyopathies, including:

In some cases the heart of a child who has a cardiomyopathy will become progressively weaker and the child will end up in a stage of heart failure (difficulty breathing, difficulty eating, excessive sweating and poor growth) advanced enough to require transplantation. However, in some cases, cardiomyopathies can be treated with medications and the child’s health may improve.

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What are the symptoms of cardiomyopathy?

Symptoms of cardiomyopathy vary widely in type and severity.

Sometimes infants have symptoms of cardiomyopathy shortly after birth. They can include:

Sometimes symptoms don’t appear until the child is older. They can include:

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How is cardiomyopathy diagnosed?

Diagnosis or cardiomyopathy may include:

During cardiac catheterization, the doctor might take biopsies (tiny pieces of heart tissue), which will be evaluated in a laboratory.

If one person in a family is diagnosed with a cardiomyopathy, it may become important that a cardiologist evaluates other family members to assess their risk. The Children’s Hospital of Philadelphia and the University of Pennsylvania Health System have created The Familial Cardiomyopathy Program to evaluate and care for children and adults with cardiomyopathies.

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What are the treatment options for cardiomyopathy?

The treatment options for cardiomyopathy vary widely depending on the type and severity of the cardiomyopathy.

Medicines

Depending on the type of cardiomyopathy, doctors may prescribe medicines that help decrease the size of a dilated heart, relax the heart, increase the pumping strength, regulate the rhythm of the heart, or help in some other way. Often several medications are used in combination.

Pacemakers and implantable defibrillators

These devices help control the heart rhythm so the heart doesn’t beat too slowly or in an irregular pattern. The devices are implanted under the skin and connected to the heart with tiny wires called leads.

Septal myectomy

In patients with hypertrophic cardiomyopathy (HCM), sometimes the septum (the wall of tissue that divides the two sides of the heart) bulges into the left ventricle (the lower, pumping chamber). If the person with HCM is having symptoms that are not helped by medications, a surgeon can remove the bulging portion of the septum through a surgery called septal myectomy. This surgery is rarely used.

Heart transplant

When an advanced stage of heart failure (symptoms that interfere with daily activities or growth in infants, and physical changes) results from a cardiomyopathy, heart transplant may be considered.

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What kind of follow-up care is required for cardiomyopathy?

Children and teenagers with cardiomyopathies will continue to see a pediatric cardiologist regularly. The level of care will vary depending on the severity of the cardiomyopathy. Many benefit from consultation with a specialist in cardiomyopathy and may be referred to other specialists in metabolism, genetics, and or neurology, if an inherited or systemic disorder related to cardiomyopathy is suspected.

If a genetic cause of cardiomyopathy is suspected, it is important for other close family members (parents, siblings, children) to have their hearts checked by a cardiologist, to ensure that they do not have the same or a related heart condition (even if they do not have any symptoms of heart problems). The Children’s Hospital of Philadelphia and the Hospital of the University of Pennsylvania have a joint program, the Familial Cardiomyopathy Program, to ensure that family members of patients with cardiomyopathies have the proper cardiac screening and genetic testing.

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Contact us

The Cardiac Center at CHOP has an experienced team of cardiologists and nurses who specialize in the treatment and care of patients with cardiomyopathy and heart failure.

Joseph Rossano, MD, MS, FAAP, FAAC
Robert E. Shaddy, MD
Kimberly Y. Lin, MD
Matthew J. O’Connor, MD
Elizabeth Goldmuntz, MD, FAAP, FACC
Kelley Miller, MSN, CPNP
Ashley Lebo, NP

Contact us to schedule an appointment with one of our specialists.

Reviewed by: Joseph Rossano, MD, MS, FAAP, FAAC
Date: September 2013

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Familial Cardiomyopathy Program

Learn about the CHOP-Penn Familial Cardiomyopathy Program - care for your whole family.