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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.

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.

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.

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:

  • Myocarditis – an inflammation of the heart muscles – most often caused by a viral infection. You can learn more about myocarditis here.
  • Metabolic disease
  • Chemotherapy

Signs and symptoms

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

Newborns, infants and babies:

  • Difficulty feeding
  • Excessive sweating when feeding
  • Rapid breathing
  • Sweating excessively during activities
  • Slow or poor growth

Toddlers, children, teens and young adults

  • Dizziness or feeling lightheaded
  • Fainting (syncope)
    Shortness of breath
  • Easily tires
  • Feeling fatigued
  • Persistent cough
  • Abnormal heartbeats or palpitations
  • Swelling or unexplained weight gain

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:

  • Medical history – in addition to the medical history you bring with you to your child's appointment, your pediatric cardiologist may have a few more questions in order to more accurately determine needed testing and to help determine the diagnosis.
  • Family medical history – the doctor will want to know about any heart, genetic or metabolic conditions as well as any history of sudden death or unexplained death in a relative.
  • Physical exam – a thorough examination will be performed on your child to determine if there are any cardiac or musculoskeletal issues that may be related to cardiomyopathy.
  • Blood tests – this may include evaluation of the white blood cell count, hemoglobin, electrolytes, chemistry and viral panels.
  • Electrocardiogram
  • Echocardiography
  • Stress test
  • Cardiac MRI
  • Cardiac catheterization
  • Myocardial biopsy – this minimally invasive procedure is done in the cardiac catheterization lab. A very small instrument is inserted into the heart through a main vein in the leg and a sample of tissue is collected and tested for viral or bacterial infection.

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:

  • Medication – your child may be treated with ACE inhibitors, beta-blockers or other types of medication designed to control symptoms and heart rhythms as well as reverse and prevent new or further damage to the heart.
  • Lifetime fitness and lifestyle plan – depending on the cause and level of damage done to the heart, as well as any continued risk, our team of specialists will create a wellness plan to help your child live as active a life as possible. In some cases, your child may not be able to participate in certain types of exercise or competitive sports. Your child's plan may include diet and an exercise program designed to complement his or her treatment plan as a whole. Some children may require careful monitoring of their diet, avoidance of fasting, and certain childhood illnesses may require oversight by your pediatrician and/or your cardiology team.
  • Defibrillator – some children may be at risk for sudden cardiac arrest. If this is a part of your child's diagnosis, the cardiac team at Cook Children's may recommend implanting a defibrillator, and possibly a pacemaker. The defibrillator can deliver a shock to your heart to stop a potentially life-threatening arrhythmia. The pacemaker helps maintain a regular heart rhythm.
  • Mechanical device – In cases where the heart is severely damaged or your child's condition continues to worsen, your child will need to have a mechanical device implanted to help his or her heart function. The type of device selected will depend on the age and size of your child since there are different devices to meet the needs of very young or small children or those of teens or adults.
  • Transplant – in some cases the heart simply cannot be repaired and your child may need a heart transplant. If this is the case for your child, our specialists will work closely with you and your family to determine the next steps and best course of action for you and your child.

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.

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

  • Difficulty feeding
  • Excessive sweating while feeding
  • Unexplained sweating
  • Poor growth

Toddlers, children, teens and young adults

  • Tires easily or fatigued even when not active
  • Shortness of breath
  • Feeling dizzy or lightheaded
  • Fainting (syncope)
  • Abnormal heart rhythm
  • Palpitations
  • Chest pain
  • Sudden cardiac arrest

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:

  • Medical history – in addition to the medical history you bring with you to your child's appointment, your pediatric cardiologist may have a few more questions in order to more accurately determine needed testing and to help determine the diagnosis.
  • Family medical history – the doctor will want to know about any heart, genetic or metabolic conditions as well as any history of sudden death or unexplained death in a relative.
  • Physical exam – a thorough examination will be performed on your child to determine if there are any cardiac or musculoskeletal issues that may be related to cardiomyopathy.
  • Blood tests – this will include determining if the oxygen levels in your child's blood suggest a problem with the heart's ability to properly function.
  • Electrocardiogram
  • Echocardiography
  • Stress test
  • Cardiac MRI
  • Cardiac catheterization
  • Myocardial biopsy – this minimally invasive procedure is done in the cardiac catheterization lab. A very small instrument is inserted into the heart through a main vein in the leg and a sample of tissue is collected and tested for viral or bacterial infection.

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:

  • Medication – To prevent complications and ease symptoms, your child may be treated with beta-blockers, calcium channel blockers and other medications designed to control heart rhythm, such as ventricular arrhythmias.
  • Lifetime fitness and lifestyle plan – depending on the cause and level of damage done to the heart, as well as any continued risk, our team of specialists will create a wellness plan to help your child live as active a life as possible. In some cases, your child may not be able to participate in certain types of exercise or competitive sports. Your child's plan may include diet and an exercise program designed to complement his or her treatment plan as a whole. Some children may require careful monitoring of their diet, avoidance of fasting, and certain childhood illnesses may require oversight by your pediatrician and/or your cardiology team.
  • Defibrillator – some children may be at risk for sudden cardiac arrest. If this is a part of your child's diagnosis, the cardiac team at Cook Children's may recommend implanting a defibrillator, and possibly a pacemaker. The defibrillator can "restart" the heart if it suddenly stops, preventing a life-threatening event. The pacemaker regulates blood flow to the body.
  • Corrective surgery – in some cases children may undergo a septal myectomy surgery to remove a portion of the enlarged heart muscle.
  • Transplant – in the event that your child's heart simply cannot be repaired, your child may need a heart transplant. If this the case for your child, our specialists will work closely with you and your family to determine the next steps and best course of action for you and your child.

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.

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

  • Difficulty feeding
  • Excessive sweating while feeding
  • Unexplained sweating
  • Poor growth

Toddlers, children, teens and young adults

  • Shortness of breath
  • Feeling dizzy or lightheaded
  • Fainting (syncope)
  • Abnormal heart rhythm
  • Palpitations
  • Unexplained weight gain or swelling

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:

  • Medical history – in addition to the medical history you bring with you to your child's appointment, your pediatric cardiologist may have a few more questions in order to more accurately determine needed testing and to help determine the diagnosis.
  • Family medical history – the doctor will want to know about any heart, genetic or metabolic conditions as well as any history of sudden death or unexplained death in a relative.
  • Physical exam – a thorough examination will be performed on your child to determine if there are any cardiac or musculoskeletal issues that may be related to cardiomyopathy.
  • Blood tests – this will include determining if the oxygen levels in your child's blood suggest a problem with the heart's ability to properly function.
  • Electrocardiogram
  • Echocardiography
  • Stress test
  • Cardiac MRI
  • Cardiac catheterization

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:

  • Medication – Depending on your child's needs, certain medications may help to prevent or repair damage to your child's heart. Children with LVNC are particularly at risk for developing blood clots so blood thinners, such as aspirin, may be prescribed.
  • Lifetime fitness and lifestyle plan – depending on the cause and level of damage done to the heart, as well as any continued risk, our team of specialists will create a wellness plan to help your child live as active a life as possible. In some cases, your child may not be able to participate in certain types of exercise or competitive sports. Your child's plan may include diet and an exercise program designed to complement his or her treatment plan as a whole. Some children may require careful monitoring of their diet, avoidance of fasting, and certain childhood illnesses may require oversight by your pediatrician and/or your cardiology team.
  • Defibrillator – some children may be at risk for sudden cardiac arrest. If this is a part of your child's diagnosis, the cardiac team at Cook Children's may recommend implanting a defibrillator, and possibly a pacemaker. The defibrillator can "restart" the heart if it suddenly stops, preventing a life-threatening event. The pacemaker regulates blood flow to the body.
  • Mechanical device – In cases where the heart is severely damaged or your child's condition continues to worsen, a mechanical device may be implanted to help his or her heart function. The type of device selected will depend on the age and size of your child since there are different devices to meet the needs of very young or small children or those of teens or adults.
  • Transplant – in some cases the heart simply cannot be repaired and your child may need a heart transplant. If this the case for your child, our specialists will work closely with you and your family to determine

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.

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:

  • Shortness of breath
  • Palpitations (fluttering of the heart)
  • Irregular heart rhythms
  • Tachycardia (rapid heartbeat)
  • Fainting (syncope)
  • Swelling of neck veins
  • Pain or discomfort in the abdomen
  • Malfunction or failure of kidneys or liver
  • Swelling of the liver, legs or in the gastrointestinal tract
  • Enlargement of the heart
  • Lung congestion

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:

  • Medical history – in addition to the medical history you bring with you to your child's appointment, your pediatric cardiologist may have a few more questions in order to more accurately determine needed testing and to help determine the diagnosis.
  • Family medical history – the doctor will want to know about any heart, genetic or metabolic conditions as well as any history of sudden death or unexplained death in a relative.
  • Physical exam – a thorough examination will be performed on your child to determine if there are any cardiac or musculoskeletal issues that may be related to cardiomyopathy.
  • Blood tests – this will include determining if the oxygen levels in your child's blood suggest a problem with the heart's ability to properly function.
  • Electrocardiogram
  • Echocardiography
  • Stress test
  • Cardiac MRI
  • Cardiac catheterization

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:

  • Medication – Children with ARVC are particularly at risk for prolonged or sustained arrhythmias which, if untreated, can result in sudden cardiac death. Antiarrhythmic drug therapy is the first step toward accomplishing this goal.
  • Radio frequency ablations – For arrhythmias that do not respond to medication, or if the medication is not well tolerated, radiofrequency ablation may be performed. This nonsurgical, minimally invasive procedure uses a high-energy source to destroy a tiny amount of tissue that conducts the electrical impulses causing the arrhythmia. A catheter with an electrode at its tip is inserted into a vein and guided to the area of heart muscle in the right ventricle. Then, a mild, painless dose of radiofrequency energy is transmitted to the pathway where the arrhythmia occurs, causing the impulses that conduct the arrhythmias to stop.
  • Lifetime fitness and lifestyle plan – Depending on the cause and level of damage done to the heart, as well as any continued risk, our team of specialists will create a wellness plan to help your child live as active a life as possible. Because of an increased risk for sudden cardiac death, your child may not be able to participate in certain types of exercise or competitive sports. Your child's plan may include diet and an exercise program designed to complement his or her treatment plan as a whole. Some children may require careful monitoring of their diet, avoidance of fasting, and certain childhood illnesses may require oversight by your pediatrician and/or your cardiology team.
  • Defibrillator – Some children may be at risk for sudden cardiac arrest. If this is a part of your child's diagnosis, the cardiac team at Cook Children's may recommend implanting a defibrillator, and possibly a pacemaker. The defibrillator can "restart" the heart if it suddenly stops, preventing a life-threatening event. The pacemaker regulates blood flow to the body.
  • Mechanical device – In cases where the heart is severely damaged or your child's condition continues to worsen, a mechanical device may be implanted to help his or her heart function. The type of device selected will depend on the age and size of your child since there are different devices to meet the needs of very young or small children or those of teens or adults.
  • Transplant – in some cases the heart simply cannot be repaired and your child may need a heart transplant. If this the case for your child, our specialists will work closely with you and your family to determine the next steps and best course of action for you and your child.

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.

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:

  • Irritability
  • Poor appetite
  • Slow weight gain
  • Poor development

Older children, teens and young adults:

  • Dizziness or feeling lightheaded
  • Fainting (syncope)
    Shortness of breath
  • Easily tires or
  • Feeling fatigued
  • Nausea
  • Persistent cough or wheezing
  • Abnormal heartbeats or palpitations

Advanced RCM symptoms:

  • Fluid build-up
  • Swollen or bulging neck veins
  • Enlarged liver
  • Swelling in the feet, legs or abdomen
  • Pulmonary hypertension

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:

  • Medical history – in addition to the medical history you bring with you to your child's appointment, your pediatric cardiologist may have a few more questions in order to more accurately determine needed testing and to help determine the diagnosis.
  • Family medical history – the doctor will want to know about any heart, genetic or metabolic conditions as well as any history of sudden death or unexplained death in a relative.
  • Physical exam – a thorough examination will be performed on your child to determine if there are any cardiac or musculoskeletal issues that may be related to cardiomyopathy.
  • Blood tests – this will include determining if the oxygen levels in your child's blood suggest a problem with the heart's ability to properly function.
  • Electrocardiogram
  • Echocardiography
  • Stress test
  • Cardiac MRI
  • Cardiac catheterization
  • Myocardial biopsy – this minimally invasive procedure is done in the cardiac catheterization lab. A very small instrument is inserted into the heart through a main vein in the leg and a sample of tissue is collected and tested for viral or bacterial infection.

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:

  • Medication – Children with RCM may be given low doses of diuretics to reduce excess fluid in the body and lungs. To prevent the development of blood clots in the heart, blood thinners, or anticoagulants, may be given. Children with abnormal rhythms may be prescribed antiarrhythmic medicines to regulate the heart rate. And in some cases, anti-inflammatories may also be prescribed.
  • Lifetime fitness and lifestyle plan – depending on the cause and level of damage done to the heart, as well as any continued risk, our team of specialists will create a wellness plan to help your child live as active a life as possible. In some cases, your child may not be able to participate in certain types of exercise or competitive sports. Your child's plan may include diet and an exercise program designed to complement his or her treatment plan as a whole. Some children may require careful monitoring of their diet, avoidance of fasting, and certain childhood illnesses may require oversight by your pediatrician and/or your cardiology team.
  • Defibrillator – some children may be at risk for sudden cardiac arrest. If this is a part of your child's diagnosis, the cardiac team at Cook Children's may recommend implanting a defibrillator, and possibly a pacemaker. The defibrillator can "restart" the heart if it suddenly stops, preventing a life-threatening event. The pacemaker regulates blood flow to the body.
  • Mechanical device – In cases where the heart is severely damaged or your child's condition continues to worsen, a mechanical device may be implanted to help his or her heart function. The type of device selected will depend on the age and size of your child since there are different devices to meet the needs of very young or small children or those of teens or adults.
  • Transplant – in some cases the heart simply cannot be repaired and your child may need a heart transplant. If this the case for your child, our specialists will work closely with you and your family to determine the next steps and best course of action for you and your child.

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.

Resources

We're here to help.

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