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CardiomyopathyCook Children's

Cardiomyopathy

There are many different types of cardiomyopathies and depending on the type, cardiomyopathy disease can cause the heart muscle to thicken, degenerate, enlarge or become rigid. Even though the defects are formed in utero (before the baby is born), some may not be diagnosed until a child is born, or even well after that, into adulthood.

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Cardiomyopathies are a major cause of sudden cardiac arrest and sudden cardiac death. To learn more about what you can do to protect your child at home, at school and during sports, click here.

What they all have in common is that they interfere with the heart's ability to receive and/or pump blood, ultimately weakening the heart and possibly leading to heart failure.

There are multiple signs and symptoms related to cardiomyopathy and some people may experience more than one. Some people never experience any symptoms or complications. However, cardiomyopathy is a chronic—and often progressive—disease. Cardiomyopathy is also one of the leading causes of sudden death in children.

Children diagnosed with a cardiomyopathy disorder will need to be seen by a pediatric cardiologist on an ongoing basis. Because the heart grows as the body grows, our patients at Cook Children’s continue with us into adulthood until their hearts reach full maturity. Not only are we a Heart Center, we are also centered on the well-being of our patients and their families for the long-term.

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Who gets cardiomyopathy?

Cardiomyopathy is diagnosed in people of all races, ages, genders and socio-economic backgrounds, but certain types of the disease may be seen more in specific groups. For example, dilated cardiomyopathy (DCM) is diagnosed more often in African Americans and arrhythmogenic right ventricular dysplasia (ARVD) is most often diagnosed in teens and young adults. Cardiomyopathy typically affects adults, but in rare cases, it also affects infants and children under the age of 18. When all forms are combined, it is estimated that 1 in 30,000 U.S. children is diagnosed with cardiomyopathy. Most children are diagnosed in the first year of life and between the ages of 12 and 18. Children with the highest risk of cardiomyopathy are those with a family history of cardiomyopathy, heart failure and/or sudden cardiac arrest. Diabetes and other metabolic diseases can also put your child at risk, along with diseases that damage the heart, including viral and bacterial infections.

What causes cardiomyopathy?

There are more than 100 known causes of cardiomyopathy in children and in a large percentage of cases, the cause remains unknown. It is estimated that nearly two-thirds of cardiomyopathy cases are the result of an unknown cause. Of those with a known cause, cases are predominantly genetic. Children with a family history of cardiomyopathy are most at risk for developing the disease. Cardiomyopathy may also be the result of other genetic diseases that are not primarily heart-related, such as muscular dystrophy. Other disorders that can cause cardiomyopathy include metabolic and mitochondrial abnormalities, thyroid disorders, anemia and complications of congenital heart disease. Certain viral and bacterial infections can affect the heart and, in rare cases, certain medication and treatments such as chemotherapy drugs and radiation can as well. Nutritional deficiencies can also contribute to certain types of cardiomyopathy.

Testing, diagnosis and treatment

With more than 100 known causes of cardiomyopathy in children and a large percentage of unknown causes, cardiomyopathy can be difficult to diagnose and treat. Many children have no symptoms. In some children symptoms come and go and can be different so they don't always appear to be related. When a child develops cardiomyopathy it can be due to a viral or bacterial infection, a defect in the heart, certain genetic or metabolic conditions, some medications or even certain necessary medical treatments, such as chemotherapy. Depending on the cause, some children will fully recover; others may have a progressive disease and require ongoing medical care throughout their lives. Here at Cook Children's Heart Center, our pediatric specialty team is experienced in the evaluation and treatment of even the rarest forms of cardiomyopathy. Currently, there are five known categories of cardiomyopathy. We have provided a detailed listing here.

Dilated cardiomyopathy (DCM)

Dilated cardiomyopathy (DCM), sometimes called congestive cardiomyopathy, is the most common type of cardiomyopathy diagnosed in children. In this type of disorder, the muscle in one or both of the ventricles becomes very thin. This causes the ventricle to enlarge, which interferes with the heart’s ability to pump blood.

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Causes

There is no single cause for DCM. A large number of cases are genetic and usually run in families, including parents, grandparents, brothers and sisters, aunts, uncles and cousins. In many cases there is no known cause of DCM. When a cause can’t be found, the diagnosis is called idiopathic dilated cardiomyopathy. It is important to remember that even when a cause isn’t found, there is treatment for the disorder.  Known causes of DCM may include:

Signs and symptoms

Many people with DCM never have symptoms until they experience heart failure. These symptoms may include:

Newborns, infants and babies:

Toddlers, children, teens and young adults

Testing and diagnosis

When you come to the Heart Center at Cook Children's, please bring your child's medical records to your appointment. We will create a complete medical history of your child including any past illnesses, environmental and genetic risk factors, what symptoms your child has had, and for how long. Once the history has been taken, the doctor will determine which tests your child may need. Tests may include:

Treatment

The type of treatment your child will need depends upon the cause, diagnosis and extent of muscle disease in your child’s heart. Treatment may include one or a combination of the following:


 

Hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) usually occurs in the left ventricle of the heart. The muscle thickens or enlarges, often affecting the wall between the left and right ventricle. The heart cannot beat properly, interrupting, and in some cases blocking, blood flow into and out of the heart. The cell inside the thickened muscles also become irregular and may interfere with the electrical impulses that signal the heart chambers, causing your child to experience abnormal heart rhythms.

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What causes it?

The most common causes of HCM are genetic. Of these, a large number of cases can be attributed to an inherited condition that causes a defect in the proteins found in the heart muscles. Other genetic causes include genetic syndromes and metabolic and mitochondrial disorders. Nearly fifty percent of all children diagnosed with HCM have a family history of the disease. In some cases, other siblings may have the disease but have no symptoms or complications. Of course, not all causes are genetic. Some are acquired from other conditions, such as consistently high blood pressure. As with all cardiomyopathies, some causes remain unknown.

Symptoms

Children with HCM may not have any symptoms at all. Some children’s symptoms may occur during physical exercise. Other times, symptoms may come on suddenly without any warning at all. Sudden cardiac arrest is the most critical symptom and may occur without warning. HCM is also the most common cause of sudden cardiac death in people under the age of 30. If your child has any of the risk factors for HCM or the following symptoms, it is recommended that he or she be seen by a pediatric cardiologist as soon as possible:

Newborns, infants and babies

Toddlers, children, teens and young adults

Testing and diagnosis

When you come to the Heart Center at Cook Children’s, please bring your child's medical records to your appointment. We will create a complete medical history of your child including any past illnesses, environmental and genetic risk factors, what symptoms your child has had and for how long. Once the history has been taken, the doctor will determine which tests your child may need. Tests may include:

Treatment

Treatment for HCM is different for each child. The specialty team here in our Heart Center will work with you, your pediatrician and your child to create a treatment plan that offers the best quality of life for your child. Some of the considerations for treatment will be based on your child's age, symptoms, how well the heart is functioning and if there are any defects or damage that will need to be repaired. Our goal is to ease symptoms, prevent complications and reduce life-threatening risks such as sudden cardiac arrest. To accomplish the best quality of life outcomes possible for each child, some of the treatment plans may incorporate:

Left ventricular noncompaction

Left ventricular noncompaction (LVNC) is a rare genetic disease in which muscle doesn't develop properly, forming bundles of muscle that are spongy. In normal development the muscles compact, becoming smooth, but in cases of LVNC the muscles remain spongy and can interfere with the pumping action of the heart, sometimes causing abnormal heart rhythms.

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What causes it?

The cause of LVNC is primarily genetic, and often runs in families, so there may be brothers and sisters, parents or even aunts, uncles, cousins or grandparents who also have the condition. Often times, when a patient is diagnosed with LVNC, it may be recommended that other family members have a screening, especially immediate family, since they may not have any symptoms. LVNC can also be a secondary result of other genetic diseases and metabolic syndromes, neuromuscular conditions and congenital defects, including abnormal development of the heart in the early weeks of pregnancy.

Many people with LVNC will go their entire lives without any symptoms of this disorder. However, children and adults with LVNC have a higher risk of forming blood clots in the heart, as well as heart failure. In addition, LVNC may cause abnormal heart rhythms. There is also an increased risk of stroke and sudden death.

Who gets it?

Because it may be genetic, LVNC often begins in the early weeks of pregnancy as the heart is forming. It may also form in young children and into the early teens. However, because it may not have any symptoms, LVNC might not be diagnosed until much later in life. There is a one in five chance of developing LVNC if a parent, brother or sister has it. If a child is born with LVNC, you may want to consider a cardiac evaluation for immediate family members—it could be a life-saving decision.

Symptoms

Newborns, infants and babies

Toddlers, children, teens and young adults

Testing and diagnosis

When you come to the Heart Center at Cook Children’s, please bring your child's medical records to your appointment. We will create a complete medical history of your child including any past illnesses, environmental and genetic risk factors, what symptoms your child has had and for how long. Once the history has been taken, the doctor will determine which tests your child may need. Tests may include:

Echocardiography and cardiac MRI are often critical diagnostic tools used in the diagnosis of LVNC because the imaging resolution allows the team to see the muscle and extent of the disease and any damage caused in much more detail. This in turn helps to determine the best course of treatment for your child’s particular diagnosis.

Treatment

The treatment plan for LVNC is different for each child, depending on age, cause of the disorder and risks associated with your child’s heart health. The specialty team here in our Heart Center will work with you, your pediatrician and your child to create a treatment plan that offers the best quality of life for your child. Some of the considerations for treatment will be based on your child’s age, symptoms, how well the heart is functioning and if there are any defects or damage needing repair. Our goal is to ease symptoms, prevent complications, and reduce life-threatening risks such as sudden cardiac arrest. To accomplish the best quality of life outcomes possible for each child, some of the treatment plans may incorporate:

Arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular (ARVC) is a very rare disorder, especially in children, where the muscle cells in the right ventricle break down and muscle tissue is replaced with fatty or scar tissue. In order to pump blood, the heart has to receive and fill with blood. The scar tissue interrupts the heart's electrical impulses, interfering with its ability to receive and fill with blood.

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What causes it?

While many of the causes of ARVD are unknown, 30 percent to 50 percent of cases diagnosed are genetic, or inherited. ARVD may also be related to congenital heart disorders, and viral or bacterial infection that gets into the bloodstream and affects the heart.

Who gets it?

ARVC affects approximately 1 in 5,000 people across all ages, races and socio-economic barriers. It is extremely rare in younger patients, but does tend to run in families. Children whose parents, grandparents, brothers or sisters are born with ARVC should be screened by a pediatric cardiologist, even if there are no symptoms.

Symptoms

ARVC is very rare in children and signs or symptoms typically don’t occur until early adulthood or later. Sudden cardiac arrest is often one of the first symptoms and is life-threatening. Other common symptoms include:

Testing and diagnosis

When you come to the Heart Center at Cook Children’s, please bring your child's medical records to your appointment. We will create a complete medical history of your child including any past illnesses, environmental and genetic risk factors, what symptoms your child has had and for how long. Once the history has been taken, the doctor will determine which tests your child may need. Tests may include:

A cardiac MRI may be recommended in the diagnosis of ARVC because the imaging resolution allows the team to see fibro-fatty tissue as well as wall abnormalities in the right ventricle, and the extent of the disease in much more detail. This in turn helps to determine the best course of treatment for your child’s particular diagnosis. For patients who already have a pacemaker or defibrillator implanted, a CT scan may be performed instead.

Treatment

The treatment plan for ARVC is different for each child, depending on age, cause of the disorder and risks associated with your child’s heart health. The specialty team here in our Heart Center will work with you, your pediatrician and your child to create a treatment plan that offers the best quality of life for your child. Some of the considerations for treatment will be based on your child’s age, symptoms, how well the heart is functioning and if there are any defects or damage needing repair. Our goal is to ease symptoms, and in the case of ARVC, prevent arrhythmias, sudden cardiac arrest and death. To accomplish the best quality of life outcomes possible for each child, some of the treatment plans may incorporate:

Restrictive cardiomyopathy

Restrictive cardiomyopathy (RCM) is rare, occurring in only three to five percent of children who are diagnosed with cardiomyopathy, or less than one in every 1,000,000 children. RCM causes the walls of the heart to stiffen, making it increasingly difficult for them to expand and contract and restricting the heart's ability to fill with blood. The heart continues to pump normally but the lack of blood supply places increased pressure on the upper heart chambers and, as a result, they become enlarged. This process can cause the blood in the ventricles to flow backward into the lungs and cause heart failure.

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What causes it?

There are multiple causes of RCM, many of which are unknown. In some cases, a genetic mutation occurs when the fetus forms, causing a change in the child’s DNA. The child may also inherit RCM from one or both parents. RCM has also been linked to congenital heart defects and rare disorders such as connective tissue diseases like scleroderma and metabolic disorders.

Who gets it?

Though typically not diagnosed before adulthood, children may also develop RCM. The disorder is diagnosed in all races and socio-economic environments. Children with a family history of RCM in a parent, grandparent, brother or sister and even aunt, uncle or cousin have a higher risk of developing RCM and should be screened, though symptoms may not appear until well into adulthood. It is also recommended that if your child is diagnosed with RCM, your family members should also be evaluated.

Symptoms

Infants, toddlers and young children:

Older children, teens and young adults:

Advanced RCM symptoms:

Testing and diagnosis

When you come to the Heart Center at Cook Children's, please bring your child's medical records to your appointment. We will create a complete medical history of your child including any past illnesses, environmental and genetic risk factors, what symptoms your child has had, and how long. Once the history has been taken, the doctor will determine which tests your child may need. Tests may include:

 

Treatment

The specialty team here in our Heart Center will work with you, your pediatrician and your child to create a treatment plan that offers the best quality of life for your child. Some of the considerations for treatment will be based on your child’s age, symptoms, how well the heart is functioning and if there are any defects or damage needing repair. Our goal is to ease symptoms, prevent complications and reduce the risk of heart failure. To accomplish the best quality of life outcomes possible for each child, some of the treatment plans may incorporate:

Genetic testing

If you or any member of your family has been diagnosed, or if your child has been diagnosed with cardiomyopathy, your family members may be candidates for genetic screening.

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If your child has been diagnosed, you probably have lots of questions. We can help. For resources, education or more information on support, click here. If you would like to schedule an appointment, refer a patient or speak to our staff, please call our offices at 682-885-2140.

 
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