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Restrictive Cardiomyopathy (RCM)
Children’s heart conditions can’t be prevented, but a lot can be done to improve their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children with a heart problem can be quickly diagnosed and treated. At the Cardiac Center, we specialize in the treatment of heart problems, including cardiomyopathies and heart failure.
If your child’s been diagnosed with restrictive cardiomyopathy (or “RCM,” for short), we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
Restrictive cardiomyopathy (RCM) in children is a disease of the cardiac muscle in which the muscle (or “myocardium”) can’t relax to allow a child’s heart to fill easily. This is often referred to as a “loss of compliance.” It results in something called “diastolic” dysfunction — because the abnormality is present primarily in diastole, which is the period of time between contractions when the heart is relaxed and filling with blood. Because it’s harder for the blood to get into the pumping chambers of the heart (the ventricles) between contractions, blood accumulates within the upper chambers (the atria) causing the atria to enlarge.
Blood also accumulates within the veins draining into the atria, causing higher venous pressures in both the pulmonary veins (draining the lungs) and the systemic veins (draining the body). The higher venous pressures in the pulmonary veins are especially damaging because they can result in damage to the blood vessels within the lungs and, ultimately, to high blood pressure in the arteries of the lungs (called “pulmonary hypertension”). When pulmonary hypertension becomes severe, it may limit the treatment options because heart transplantation may become impossible.
In most cases, the underlying cause is unknown (or “idiopathic”). The heart may also become thickened (or “hypertrophied”). It can be difficult to make the distinction between this disease and hypertrophic cardiomyopathy, especially when the hypertrophic cardiomyopathy is nonobstructive.
How Does the Heart Normally Work?
When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »
Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:
Children usually show signs of restrictive cardiomyopathy in early childhood, but it may be diagnosed from the newborn period through adolescence. Many children are asymptomatic (they don’t have symptoms) but a caregiver may hear a heart murmur. But most children will have symptoms of “diastolic heart failure” (this is heart failure due to the inability of the heart to relax) including:
- difficulty breathing
- fluid retention (which may cause swelling in the extremities)
- exercise intolerance (difficulty exercising)
We can usually diagnose restrictive cardiomyopathy (RCM) in children using an echocardiogram (an “echo”). An echo is a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart.
Children often also need additional testing including cardiac catheterization. Catheterization involves inserting a thin plastic, flexible tube (called a “catheter”) into an artery and vein that lead to the heart. Cardiac catheterization is done under sedation with local anesthesia or under general anesthesia, depending on your child’s age and condition.
It’s especially important to measure the pulmonary artery pressures and evaluate the resistance to blood flow through the lungs. This may help us determine the best treatment strategies.
The only effective treatment for restrictive cardiomyopathy is heart transplantation. The timing of transplantation depends on the individual characteristics of each individual child. Although some may live 10 years or longer after diagnosis without a heart transplant, most don’t survive more than three to five years. There’s an increased risk of both sudden death and gradually worsening heart failure. So heart transplantation is a must.
In children with restrictive cardiomyopathy and elevated pulmonary artery pressures (called “pulmonary hypertension”), at the Nemours Cardiac Center we’ve had success using a ventricular assist device (or “VAD,” a mechanical pump to assist the heart) to lessen the work of the heart and allow the pulmonary arteries to return to more normal pressures and resistance. This has allowed us to transplant children who wouldn’t have been candidates before the VAD was implanted.
Children with restrictive cardiomyopathy will need frequent echocardiograms and physician visits to monitor the changes in their heart muscle. Cardiac catheterization procedures are also often necessary to assess the degree of difficulty in filling the ventricle in between beats. Medications may help treat some symptoms.
When children need a ventricular assist device to assist with the function of their heart, they’ll often need to stay in the hospital until a transplant becomes available. Children need to be on blood thinners while being supported by the ventricular assist device. Most children can function excellently and enjoy a normal quality of life following a heart transplant, but they will need to take medications for the rest of their lives to prevent rejection of the new heart.
If your child has restrictive cardiomyopathy, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart disease journey — and to help your child live the healthiest, most fulfilling life possible.
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Information for Patients
Outpatient Services and Inpatient Units: (302) 651-6660
After 5 p.m. and Weekends:
Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690