When Your Child Has Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is a problem with the heart muscle. It may not cause symptoms that bother your child. But it can lead to serious problems over time. The good news is that it can usually be managed. Your child’s healthcare provider will talk with you about treatment options. This sheet tells you more about this problem and how it is treated.

The normal heart

The heart is divided into 4 chambers that hold blood as it moves through the heart. The 2 upper chambers are called atria. The 2 lower chambers are called ventricles. The heart also contains 4 valves between the chambers. The valves open and close to keep blood flowing forward through the heart.

What is hypertrophic cardiomyopathy?

With this condition, the heart muscle thickens. Heart muscle that is too thick can’t pump blood normally. It may affect both ventricles. Or it may affect only 1, most often the left ventricle. In some cases, it may just affect the wall that separates the ventricles (ventricular septum).

Why is hypertrophic cardiomyopathy a problem?

A thickened heart muscle can:

  • Impair blood flow leaving the heart. The thickened wall can block the outflow of blood from the heart to the body. Or it can force the valves to close too soon due to changes in pressure. This forces the heart to work harder than normal to pump blood. If the heart no longer pumps blood well, a condition called congestive heart failure (CHF) can develop. In CHF, the inability of the heart to push blood forward appropriately can cause a backup of blood and fluid in your lungs, abdomen, liver, and limbs. This can also weaken the heart muscle more. Over time other organs in the body will also suffer from a lack of adequate blood flow.

  • Damage heart valves. The mitral valve is most likely to be affected. This valve is found between the left atrium and the left ventricle. Blood may leak backward through the valve. This is called valve insufficiency. Excess blood leakage through the valve puts undue strain on the ventricle. It has to pump extra hard to get all the blood out.

  • Damage electrical cells in the heart. The thickened heart can change the way electricity is conducted through the heart and can affect the cells that control the beating of the heart. Damage to these cells can cause abnormal heart rhythms (arrhythmias). In rare cases, a severe arrhythmia can lead to sudden death.

What causes hypertrophic cardiomyopathy?

This condition can occur in your child by chance. A cause may not always be found. But hypertrophic cardiomyopathy is one of the most common inherited (genetic) diseases of the heart muscle. It may be passed from a parent to child in some families. If you or your child has this condition, each close family member should be tested for it. It is important to let your healthcare provider know if there is a history of sudden cardiac death in the family or death at a young age.

What are the symptoms of hypertrophic cardiomyopathy?

Most children with this condition have no symptoms. If they do occur, they often show up when children are active. Symptoms may include:

  • Lightheadedness, dizzy spells, or fainting

  • Rapid, pounding heartbeat

  • Shortness of breath or tiredness

  • Tightness or pressure in the chest

  • Fatigue or having little energy for usual activities

How is hypertrophic cardiomyopathy diagnosed?

Hypertrophic cardiomyopathy symptoms may be nonspecific. Heart problems in children are managed by a pediatric cardiologist. The pediatric cardiologist will do a physical exam to check for a heart problem. Several tests may be done to help confirm a diagnosis or tell more about the heart problem. These tests may include:

  • Echocardiogram (echo). Sound waves (ultrasound) are used to make a picture of the heart. This helps find problems with heart structure or function.

  • Electrocardiogram (ECG or EKG). This test records the electrical activity of the heart. It helps find arrhythmias or some problems with heart size or structure.

  • Holter or event monitor. This test records the electrical activity of the heart over time, usually from a few days to a few weeks. It can help detect problems with the heart rhythm.

  • Exercise stress test. This records the electrical activity of the heart while your child exercises. It helps detect problems with the heart that may be noticed when the heart beats faster.

How is hypertrophic cardiomyopathy treated?

Treatment is aimed to reduce symptoms and help prevent CHF. It can also reduce the risk of severe arrhythmia and sudden death. Your child’s treatment plan may include:

  • Medicines. Beta-blockers and calcium channel blockers may be prescribed. These medicines can lower blood pressure and slow heart rate. They help to prevent arrhythmias. They may also improve the heart’s pumping action.

  • Surgery. This is done in severe cases. Surgery removes a portion of the heart muscle that is abnormally thickened. Surgery is typically performed when there is obstruction of blood flow out of the heart because of the thickened muscle. Surgery improves blood flow from the heart to the body.

  • Implantable cardioverter defibrillator (ICD). Sometimes having hypertrophic cardiomyopathy places you at higher risk of a dangerous arrhythmia. An ICD is a device that is placed in the chest. It tracks the heart rate. When needed, it delivers an electric shock to the heart to stop a life-threatening heart rhythm.

  • Heart transplant. In rare cases, a heart transplant may be needed. Your child’s cardiologist will discuss this with you.

How shared decision-making can help

With hypertrophic cardiomyopathy, testing and treatment can vary. What might be the correct option for one child may not be a good choice for another. The American College of Cardiology advises shared decision-making for children with or at risk for hypertrophic cardiomyopathy. Shared decision-making is when patients, caregivers, and healthcare providers work together to make care decisions. It includes talking about all testing and treatment options; their risks, benefits, and expected outcomes; and the child's and family's own values, goals, and preferences. Shared decision-making improves health outcomes. It can help you work with your child's care team to personalize your decisions and find the right option for your child.

What are the long-term concerns?

  • With treatment, most children with this condition can be active. But they may have to limit certain sports or physical activities. Talk with your child's healthcare provider about activities that are safe for your child.

  • Your child needs to have regular visits with the cardiologist for the rest of their life. This is to check that the heart is working correctly. If an ICD is placed, this needs regular checks.

  • Your child may need to take medicine for the rest of their life.

When should I call my child's healthcare provider?

Contact your child's provider if your child has any of the following:

  • Fainting or dizzy spells

  • Trouble breathing

  • Tightness or pain in the chest

  • Irregular, rapid heartbeats (palpitations)

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