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Dedicated Sibley Cardiomyopathy Program Treats and Manages Complex Heart Muscle Disease

Cardiomyopathy is a broad term that refers to a disease of the heart muscle that impairs its ability to pump blood effectively. Some cases are acquired, such as those resulting from a viral infection or certain medications, and others can be genetic. Cardiomyopathy can be seen in isolation or as a part of Congenital Heart Disease (CHD).

The Children’s Cardiomyopathy Foundation reports that one child out of 100,000 is diagnosed with cardiomyopathy, and that for every diagnosed child, there is likely another undiagnosed child at risk of premature death. Up to 67 percent of cases have no known cause.

Different kinds of cardiomyopathy cause the heart muscle to enlarge, thicken or become stiff, and the disease can lead to irregular heart rhythms or heart failure. Robert Campbell, MD, pediatric cardiologist at Sibley Heart Center Cardiology, said there are five classifications of cardiomyopathy that doctors may encounter in patients sent to the Hypertrophic Cardiomyopathy Program at Sibley Heart Center Cardiology.

Dilated cardiomyopathy

This is the most common form of cardiomyopathy in which the heart muscle becomes enlarged and stretched, causing the heart to become weak and pump inefficiently. Problems that can occur include irregular heart rhythms, risk of blood clots, heart failure and leaky mitral valve. The most common symptoms include pale or ashen skin color, cool, sweaty skin, rapid heart and breathing rate, shortness of breath, fatigue, irritability, chest pain, poor appetite and slow growth.

Hypertrophic cardiomyopathy

The heart muscle becomes thicker than normal, obstructing blood flow to the rest of the body and setting the patient up for arrhythmias and the possibility of sudden death. The thickened muscle can also affect the mitral valve, allowing blood to move backwards from the left ventricle into the left atrium, instead of forward to the rest of the body. Children with hypertrophic cardiomyopathy may have symptoms that increase with exertion or the symptoms may be unpredictable. The most common symptoms are shortness of breath on exertion, dizziness, fainting, chest pain and abnormal heart rhythms.

Restrictive cardiomyopathy

The heart muscle becomes excessively stiff and requires high pressure to fill. This is an uncommon type of cardiomyopathy in the U.S. and occurs rarely in children. Symptoms include exercise intolerance, exercise shortness of breath, and arrhythmia.

Arrhythmic Right Ventricular Dysplasia

ARVD is a rare type of cardiomyopathy that occurs if the muscle tissue in the right ventricle is infiltrated by scar tissue, disrupting the heart’s electrical system and causing life threatening arrhythmias. ARVD usually affects teens and young adults, and it can cause sudden cardiac arrest in young athletes. Symptoms include heart palpitations and fainting after physical activity.

Non-compaction cardiomyopathy (NCC)

This is a rare congenital cardiomyopathy that affects both children and adults. It results from the failure of myocardial development during a baby’s development. In this type of cardiomyopathy, the walls inside the left ventricle look saggy, like the rough edge of a sponge, instead of smooth, Dr. Campbell said, setting up a substrate for arrhythmias.

We now know that many of the cardiomyopathies may be inherited or genetic,” Dr. Campbell said. “It is important to know the details of family history. Cardiomyopathy is often a progressive disorder and the clinical manifestations may worsen over time. Features of the five different cardiomyopathies may coexist in any one patient or family, for example hypertrophic and restrictive.”

Treatments for cardiomyopathy may include medications or surgical interventions like implantation of a pacemaker or defibrillator. Some cases improve over time, while others require the consideration of heart transplant.

Depending on the type, some cases of cardiomyopathy can be managed with activity guidelines and by ensuring that a child’s family and school are prepared to respond to sudden cardiac arrest through a program like When considering activity management, Dr. Campbell says that it’s important to balance the long-term health needs of a child in a society in which 30 percent of children are obese with the conservative national guidelines.

I take the approach of shared decision-making with the family,” Dr. Campbell said. “We sit down together, and I lay out the risks and rewards of staying active. We want to lower or eliminate the risks for their child and come up with a plan that addresses how we can be as safe as possible and as normal as possible.”

For more resources, please visit:

Hypertrophic Cardiomyopathy Association

Children’s Cardiomyopathy Foundation

The Children’s Healthcare of Atlanta article: Cardiomyopathy and Your Child

For more information about Sibley Heart Center Cardiology and our pediatric cardiology specialists, click here.

 

 

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