Cardiomyopathy and Heart Failure
What is heart failure?
Heart failure is a condition caused by the heart's inability to circulate the blood well enough to meet the demands of the body. It is typically considered a disease that affects adults, often after a heart attack. Children may also develop poor heart function but for different reasons. If your child has heart failure, our experienced team will determine the causes of your child's heart problem and develop the best treatment plan for him or her.
What causes heart failure in children?
Your child may have developed heart failure as the result of infection (such as myocarditis), congenital heart disease or cardiomyopathy, a primary disease of the heart muscle. We thoroughly evaluate children with cardiomyopathy to determine if there are muscular, infectious, metabolic and/or genetic reasons for the condition.
Some of the underlying causes of cardiomyopathy and heart failure, such as carnitine deficiency (a metabolic disorder), are reversible when treated with medications. Myocarditis, an inflammation of the heart muscle, which is a common cause of heart failure in children, can often be treated with aggressive therapies to support the heart - even mechanical devices when necessary - that can result in complete or near-complete recovery of heart function.
Other causes of heart failure include systemic diseases that affect more than one organ system. These include:
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Barth's syndrome and Noonan's syndrome (genetic)
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Long chain fatty acid deficiency (metabolic)
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Duchenne's muscular dystrophy (neuromuscular)
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Mitochondrial
In some cases, a child's cardiomyopathy is "idiopathic," meaning that there is no identifiable cause.
Cardiomyopathy explained
Your child may have a cardiomyopathy, abnormal heart muscle, without symptoms of heart failure. There are four types of cardiomyopathy: dilated, hypertrophic, restrictive or noncompaction.
Dilated cardiomyopathy is the most common type in both adults and children. It results when the pumping chambers (ventricles) of the heart are abnormally enlarged and weakened. Some children can have a dilated heart with no symptoms while others develop heart failure (see above). There are many different causes of dilated cardiomyopathy. Some families have many members, across many generations, with this type of cardiomyopathy, which is known as familial dilated cardiomyopathy.
Hypertrophic cardiomyopathy occurs when one or more pumping chambers (ventricles) in your child's heart become unusually thickened or "muscle-bound." This may affect the left ventricle, which pumps blood to the body, or both the left and right ventricles (the right ventricle pumps blood to the lungs). This type of cardiomyopathy is relatively rare in infants and children, but more common in adolescents. It is often associated with abnormal heart rhythms, which can lead to sudden death. Hypertrophic cardiomyopathy may run in families; members of affected families are encouraged to undergo screening with echocardiography.
Restrictive cardiomyopathy is the rarest type of cardiomyopathy in children. The chambers of the heart become stiff, resulting in improper heart muscle relaxation, so that your child's heart cannot fill with blood adequately. Abnormal heart rhythms may also occur with this disease.
Noncompaction cardiomyopathy is characterized by muscle-bound (hypertrophic) pumping chambers and poor pumping function. Because of the "primitive" appearance of the heart, it is sometimes called "spongiform" or "embryonal" cardiomyopathy, and is thought to be caused by incomplete heart cell maturation. This condition is often diagnosed in infancy. Noncompaction cardiomyopathy is a rare disease, not commonly seen by the general pediatrician or family physician. It can exist alone or along with structural heart disease; it can be part of an underlying syndrome or occur idiopathically (with no identifiable cause).
How is cardiomyopathy treated?
Diagnosing and successfully treating cardiomyopathy is challenging and requires an experienced healthcare team, such as the one at The Children's Hospital of Philadelphia. Your child may do well with proper medication, combined with outpatient follow-up. This combination can slow the progression of your child's heart failure and improve his or her activity level and quality of life. In some cases, your child's heart disease may progress to the point that heart transplantation is required.
If your child has any type of cardiomyopathy, it is essential that he or she have careful, expert evaluation and precisely tailored therapy. Our team is among the best in the world and can help you through the process.
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