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What Is Cardiomyopathy?
 

The Centers for Disease Control and Prevention (CDC) estimated about 2000 Americans under the age of 25 could be the victims of sudden cardiac arrest in the coming year as schools open across the country. Sudden cardiac arrest is the leading cause of death on school property and 25 percent of the cases simply occur during sports.

Autopsy of victims revealed that many of them had cardiomyopathy. According to the Children’s Cardiomyopathy Foundation (CCF), cardiomyopathy affects about 30,000 children in the United States. Cardiomyopathy is a heart disease of abnormal heart muscle. Heart muscle of a patient with cardiomyopathy becomes enlarged, thick or rigid. Some patients might have their muscle tissue in the heart being replaced with scar tissue, though this is very rare.

If cardiomyopathy worsens, the heart becomes weaker and it might have difficulty in pumping blood through the body to maintain a normal electrical rhythm. The weakening of the heart can lead to other complications, including heart failure, arrhythmias (irregular heartbeats), blood clots, valve problems, cardiac arrest and sudden death. There are 3 main types of cardiomyopathy, namely dilated, hypertrophic and restrictive.

Dilated cardiomyopathy is the most common one, in which the pumping ability of the heart’s main pumping chamber (left ventricle) becomes enlarged (dilated) and cannot effectively pump blood out of the heart. It might occur as a result of infection, chemotherapy or alcohol use. Some people with dilated cardiomyopathy have a family history.

Hypertrophic cardiomyopathy involves abnormal growth or thickening of the heart muscle, particularly affecting the muscle of the heart's left ventricle. As the thickening occurs, the heart tends to stiffen and the size of pumping chamber might shrink and so interfere with the heart’s ability to deliver blood to the rest of the body. It can develop at any age, but it tends to be more severe if it becomes apparent during childhood. Most affected people have a family history of the disease though some might be linked to genetic mutations.

People with restrictive cardiomyopathy have heart muscle that is rigid and less elastic. This type of heart cannot properly expand and fill with blood between heartbeats. It can happen to people of any age, but it tends to affect older people. It is rather rare and can occur for unknown reason. It might also be caused by diseases elsewhere in the body that affect the heart, like disorder that causes the buildup of abnormal proteins (amyloidosis) or a disorder that causes abnormal blood cells to damage the heart (eosinophilic heart disease).

Cardiomyopathy can strike in infancy, too. Recent studies funded by CCF showed that children are 10 times more likely to get the disease during the first year of life than those aged between 2 and 18 combined. Affected infants might experience poor weight gain, breathing difficulty, excessive sweating or lethargy.

Some symptoms of cardiomyopathy include shortness of breath, rapid breathing, fainting, chest pain, dizziness, heart palpitations and fatigue. But children with the underlying disease can be asymptomatic, and they might face higher risk of sudden death if they participate in competitive sports. Children at risk should have an EKG or echocardiogram to ascertain if their heart muscles are enlarged. The screening must be repeated every 5 years until adulthood and is not always conclusive. Many doctors do not recommend genetic testing because there are more than 1,000 genes linked to cardiomyopathy, making the test become so complex.

Treatments might include taking medications such as statins, beta-blockers and calcium channel blockers, undergoing surgery to burn away the thick part of the heart muscle, and implanting defibrillator devices. Those with hypertrophic cardiomyopathy should not compete in sports at all.

Since cardiomyopathy is a silent killer, raising the awareness about the disease is necessary. CCF is the only organization that advocates exclusively for pediatric cardiomyopathy. To protect children against sudden cardiac death, CCF has already introduced legislation to include measures in schools like requiring automatic external defibrillators (AEDs) in the schools and requiring staff to know where these life-saving devices are located and how to use them.

 

 

 

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