The term cardiomyopathy refers to a series of conditions that affect the heart muscle (or myocardium). There are two kinds of cardiomyopathy. Abnormal heart muscles cause primary cardiomyopathy. Secondary cardiomyopathy is caused when other diseases damage the heart muscle.
Cardiomyopathies are more common in children than previously thought. Furthermore, children may not show cardiomyopathy on testing, but may have high risk of developing it based on their family history and genetic predisposition. The most common cardiomyopathies in children are dilated (enlarged and weaken hearts) or hypertrophic (thick and stiff hearts).
Symptoms of Cardiomyopathies
Symptoms vary based on the type of cardiomyopathy. For those with either dilated or hypertrophic cardiomyopathy, the signs are similar to congestive heart failure.
In babies, symptoms may include:
- Breathing problems during feeding
- Lack of energy
- Longer than normal feedings
- Poor weight gain
In older children and teens, common symptoms are:
- Abdominal pain
- Abnormal heartbeat sensations (palpitations)
- Breathing problems
- Lack of appetite
- Passing out and inability to exercise for extended periods of time
Sometimes cardiomyopathy has no symptoms, especially in its earliest stages. The condition may be discovered during tests for another health issue or a sports physical. These patients often benefit from medications and regular visits with a cardiologist who specializes in congenital heart defects.
If cardiomyopathy is secondary and caused by an infection, patients may notice fever, chills and achiness before heart-related symptoms appear.
Diagnosis of Cardiomyopathy
If your doctor suspects your child has a form of cardiomyopathy, diagnosis begins with a health history, a physical examination and possibly blood test. Other tests may include:
- Electrocardiogram (EKG): This simple, painless test is often the first test. It is designed to record the heart’s electrical activity through electrodes attached to the skin like bandages.
- Echocardiography (Echo): This painless ultrasound uses sound waves to create pictures that show the size, shape and function of the heart. Doctors use echo to see the type and severity of the cardiomyopathy.
- Cardiac MRI. Occasionally, an MRI of the heart is needed to get more detail about the heart. Cardiac MRIs are also used to get more information when a patient’s echocardiograms are inconclusive. This test provides information about the structure and function of the heart. Dye may be injected to provide additional information. This test may be done under general anesthesia in young children and without anesthesia in older children.
- Chest X-ray. X-rays can provide yet another set of images, primarily to show whether the heart is enlarged or if the lungs have extra fluid (a sign of heart failure).
- Holter and Event Monitors. Patients wear these small portable devices, which record the heart’s electrical activity during normal daily activities. These are like “take home EKGs” that collect data for 24 to 48 hours.
- Stress Test. A series of tests may be performed while your child’s heart is working hard and beating fast. Usually done in school-age children or older, it involves walking/running on a treadmill or riding an exercise bike while blood pressure, EKG and breathing are monitored. Your child will exercise (or be given medicine) to increase heart rate and activity.
- Genetic Testing. Genetic blood tests may be ordered when a doctor suspects cardiomyopathy, but your child is not showing symptoms. Since some heart issues run in families, your child’s doctor may recommend tests for parents and siblings.
- Cardiac Catheterization. This procedure is typically done under general anesthesia, except in teenagers and adults where local anesthetic may be all that is necessary. A small, flexible tube is threaded through a vein in the arm, upper thigh or neck. As a diagnostic step, special dye is injected, allowing the doctor to see how the blood flows through the heart and blood vessels. Pressure inside the heart can also be measured. Sometimes, tiny pieces of heart muscle are also taken for further study (heart biopsy).
Treatments vary based on the type and severity of the cardiomyopathy. A child without symptoms may be monitored through regular checkups and tests. In more complex cases, several options may be considered:
- Medications. Some medications have been shown to relieve symptoms, regulate heart rate and improve survival in patients with cardiomyopathy. The type and effectiveness of medications depends on the exact type of cardiomyopathy. In dilated cardiomyopathy beta blockers and angiotensin converting enzyme inhibitors (ACE inhibitors) are frequently used. These medicines can help remodel the enlarged and weak heart to make it better at pumping. Other medications may also treat cardiomyopathy. Occasionally, patients may have severe heart symptoms that require them to come into the hospital for short periods to get intravenous (IV) medications. These medications help remove excess water and improve the heart’s pumping to allow it to recover.
- Pacemaker. These small implantable devices send electrical signals to help regulate and monitor heart rates. During a minimally-invasive procedure under local anesthesia, a pacemaker is inserted under the skin.
- Defibrillator. Implantable cardioverter-defibrillators (ICDs) can be a life-saving instrument. This tiny battery-powered device is about the size of a pocket watch. It is placed under the skin to regulate the heart rate and serve as a pacemaker. If an abnormal rhythm occurs, the device delivers an electric shock to restore a normal heartbeat.
- Radiofrequency Ablation. This minimally-invasive procedure uses a small, needle-like probe to burn away scar tissue and improve the heart’s electrical system.
- Ventricular Assist Devices (VADs) and Total Artificial Hearts (TAH). Both devices are advanced therapies to augment or replace the heart’s work when it is very weak. VADs are pumps used to assist the heart. They are placed in the patient’s native heart to remove blood and pump it to the body. VADs are used when only one side of the patient’s heart is weak or failing. TAHs are used when a both sides of a patient’s heart are weak or failing. When a TAH is placed, the patient’s heart is completely removed. The TAH handles the full workload for both sides of the heart. VADs and TAH are only used in children as a temporary measure (bridge) until they receive a heart transplant.
- Heart Transplant. In the most severe cases, a heart transplant may be recommended. While waiting for a donor, your child may be placed on a heart-assist device to support the heart and prepare for transplant.
Key Points To Remember
Key Points To Remember
- The term cardiomyopathy describes several conditions that affect the heart muscle.
- Diagnosis can occur through a physical exam, blood tests and non-invasive procedures.
- Cardiomyopathy is serious, but rare. It is treatable through medical and surgical options.
- If cardiomyopathy is secondary and caused by an infection, the first symptoms may be fever, chills and achiness before heart-related indications appear.
- Sometimes cardiomyopathy has no symptoms and it is discovered during tests for another health issue or a sports physical.
- Cardiomyopathy frequently runs in families. If one family member is diagnosed with a cardiomyopathy, other relatives are usually tested (both genetic and clinical) to determine whether they may have the condition or carry a risk of developing it later in life. The Riley at IU Health Cardiovascular Genetics Program helps families determine how they may be affected by congenital heart defects such as cardiomyopathy.
Support Services & Resources
Support Services & Resources
Visit the websites below to find support groups, services and learn more about cardiomyopathy.
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
This not-for-profit organization supports adults living with congenital heart disease and works with health professionals to distribute helpful information to patients, parents and caregivers.
The American College of Cardiology provides educational resources to support people living with congenital and cardiovascular disease, including cardiomyopathy.
This U.S. government website offers health information to help parents and caregivers research conditions such as cardiomyopathy.
Clinical research is vital to finding new tests to help with diagnosis and new treatments. Our pediatric cardiologists actively participate in many studies at a local and national level, including research funded by grants from the National Institutes of Health (NIH) Program Project—a major institution that studies the cause and treatment of heart conditions. Cardiologists at Riley at IU Health may ask whether you are interested in volunteering your child for participation in clinical studies.
In addition to our primary hospital location at the Academic Health Center in Indianapolis, IN, we have convenient locations to better serve our communities throughout the state.
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