Read about restrictive cardiomyopathy, a condition in which the heart muscle becomes very stiff and cannot pump blood effectively.
Key points
Restrictive cardiomyopathy is the most serious type of cardiomyopathy.
In restrictive cardiomyopathy, the heart muscle becomes very stiff and does not stretch in the relaxation phase when it needs to fill with blood.
Children with this condition will have symptoms of congestive heart failure.
This condition is difficult to treat with medication and may require a heart transplant.
Introduction
Normal left heart function1) Oxygenated blood is pumped into the left atrium from the lungs. The atrium contracts (pumps), pushing blood into the left ventricle. 2) The ventricles contract. 3) This contraction pumps the oxygenated blood out of the left ventricle and into the aorta. The aorta delivers oxygenated blood to the rest of the body. Deoxygenated blood is pumped into the pulmonary artery, to be taken to the lungs.Restrictive cardiomyopathy1) The heart muscle becomes very stiff and does not stretch to allow the ventricles to fill . As a result, the left ventricle cannot handle the amount of blood being pumped in from the left atrium. 2) The stiff heart muscle causes higher pressure in the ventricle. 3) Higher pressure causes blood to back up in the atrium. 4) This results in increased pressure in the atrium which can cause a buildup of pressure and fluid in the lungs.
In restrictive cardiomyopathy (RCM), the
heart muscle becomes very stiff. This means the heart muscle does not stretch in the relaxation phase when it needs to fill with blood. Although the heart keeps pumping, it does not pump enough blood to meet the body’s needs.
Restrictive cardiomyopathy is rare in children, accounting for between 2-5% of all paediatric cardiomyopathy.
More information
What is cardiomyopathy
Cardiomyopathy is a disorder affecting the heart muscle. The heart may have a normal structure but there are problems in the way it develops or functions. Cardiomyopathy usually results in the heart being unable to pump properly, also known as
heart failure.
Cardiomyopathy can be caused by a number of factors, including infections, conditions affecting the body’s metabolism and genetics.
There are several different types of cardiomyopathy. The four main types are:
Children with RCM will have symptoms of congestive heart failure, including:
irritability
difficulty feeding
faster and harder breathing
slow weight gain
sweating with crying or feeding
poor energy
exercise intolerance
poor appetite
palpitation
swelling of ankles and abdomen (rare)
Causes, risk factors and prevalence
Causes of RCM in children
Similar to other types of cardiomyopathy, RCM can be inherited from a parent, meaning there is a genetic cause. If your child is diagnosed with RCM, it is usually recommended that parents, siblings and other close relatives be tested for a heart condition.
Treatment
Treatment of RCM
RCM is the rarest type of cardiomyopathy. However, it is also the most serious type of cardiomyopathy and has the fewest treatment options.
It is difficult to treat this condition with medications. A heart transplant may be needed.
Complications
Complications of RCM
RCM can result in "back pressure" into the lungs and right side of the heart which can cause shortness of breath or fluid in your child’s lungs and/or belly. There is also a higher risk of abnormal heart rhythms with this condition and many children will require blood thinners (anti-coagulation) to reduce the risk of blood clot formation.
