Introduction:

Restrictive Cardiomyopathy (RCM) is a rare disease where the heart muscle becomes rigid or stiff.  This makes it difficult for the ventricles to relax and a properly fill with blood.

Some cases may be due to an underlying disease, but many have no identifiable cause.

 

Signs and Symptoms:

Typical symptoms are symptoms of heart failure. Symptoms may include:

• shortness of breath (starts with exercise, but over time occurs at rest)
• shortness of breath when lying flat
• fatigue
• persistent cough
• swelling in face, abdomen, legs or feet
• unexplained weight gain
• dizziness or lightheadedness
• fainting or passing out (syncope)
• abnormal heartbeats
• nausea and vomiting
• decreased appetite

Diagnosis

RCM may be suspected due to symptoms of heart failure. Diagnosis is typically made by echocardiogram, but may also require cardiac catheterization to differentiate it from other conditions.

Additional testing may include chest X-ray, ECG, exercise stress test, MRI and Holter monitor.

Treatment:
RCM in children is typically managed by a pediatric heart failure specialist.

The treatment for RCM is focused on minimizing or preventing symptoms, protecting children from abnormal heart rhythms and delaying disease progression.
Medications are used to treat heart failure symptoms.

If a child is identified to be at increased risk for sudden cardiac arrest, preventative treatments using medications to protect against abnormal rhythms (antiarrhythmic medications) or an implantable cardioverter defibrillator (ICD) may be recommended.
If the condition is severe, a heart transplant may be considered.

 

Lifestyle changes:
Some children with RCM may be restricted from certain sports and activities. “Intense” physical activity may be a risk for a child with a weakened heart muscle. Children with RCM should never “push through” a symptom if they are feeling tired, dizzy, or have difficulty breathing with an activity. They should remain well hydrated at all times and rest when needed. It will be important to discuss the sports and activities that are safe and appropriate for your child and to focus on what CAN be done and how to stay safe when participating in activities. Children with RCM should also follow a healthy, well balanced diet.