Overview, Causes, & Risk Factors
Restrictive cardiomyopathy is a condition in which the walls of the heart become thick and rigid. The heart is then not able to fill with a normal amount of blood.
What is going on in the body?
In a person with restrictive cardiomyopathy, the muscle in
the walls of the heart becomes thickened and less elastic. The heart then
cannot fill with blood properly. Blood can, in effect, "back up" into the lungs and the rest of the body. This can lead to failure of the heart, or congestive heart failure.
What are the causes and risks of the disease?
When the cause of the heart muscle thickening
is unknown, this condition is called primary restrictive cardiomyopathy.
However, other diseases can cause this condition. These cases are called
secondary restrictive cardiomyopathy. Examples of causes include:
amyloidosis, a condition
caused by abnormal protein deposits that can affect the heart and many other areas of the body
sarcoidosis, a condition that
causes inflammation in many areas of the body for unknown reasons
hemochromatosis, a condition
caused by too much iron in the body. The extra iron can get deposited into the heart and other organs.
Loffler's syndrome, a condition caused by an abnormally high number of
certain blood cells. These cells can cause damage to the heart and other
endomyocardial fibrosis, a disease of unknown cause that results in
scarring of the heart
In some cases, the cause cannot be found.
Symptoms & Signs
What are the signs and symptoms of the disease?
Symptoms of restrictive cardiomyopathy
are mostly due to the rise in pressure from blood backing up in the lungs and other parts of the body. These symptoms may include:
shortness of breath or trouble breathing
swelling of the veins in the neck and the legs
swelling in the abdomen, known as ascites
heart murmurs, or abnormal
Diagnosis & Tests
How is the disease diagnosed?
Diagnosis of restrictive cardiomyopathy begins with a
history and physical exam. The healthcare provider may order a variety of tests, including:
an electrocardiogram, or ECG
a chest x-ray
an echocardiogram, which is a test that uses ultrasound waves to view the heart
other imaging tests, such as a CT
scan or MRI
A biopsy of the inside of the
heart is often needed to confirm the diagnosis. A biopsy is a procedure to remove a small piece of tissue from the body. A heart biopsy is done by a procedure called cardiac catheterization.. This involves inserting a tube through the skin and into an artery, usually in the
groin. The tube is moved forward in the artery until it reaches the heart. A
small tool can be passed through the tube to remove a small piece of heart
muscle. This piece can be sent to the lab for further examination and testing.
Prevention & Expectations
What can be done to prevent the disease?
In most cases, nothing can be done to prevent restrictive cardiomyopathy. Prevention is related to the cause. Early treatment of sarcoidosis and hemochromatosis may help prevent some cases.
What are the long-term effects of the disease?
Congestive heart failure almost always occurs in this
condition. Restrictive cardiomyopathy often has a very poor outcome and
commonly causes death.
What are the risks to others?
There are no risks to others.
Treatment & Monitoring
What are the treatments for the disease?
There are few treatments for restrictive cardiomyopathy. Treatment is directed at the cause, when possible, to prevent further heart damage. For example, a person with hemochromatosis often needs to have his or her blood filtered
by a machine to remove excess iron. Medications called diuretics, or "water" pills, and heart medications are used to treat the congestive heart failure.
Medications to suppress the immune system can help delay heart damage in many
cases. This kind of treatment does not offer a cure. In rare cases, surgery to remove some of the scarred heart tissue may improve symptoms. For cases that don't respond to these measures, a heart transplant may be the only treatment.
What are the side effects of the treatments?
Side effects depend on the treatment used. Diuretics and heart medications can cause allergic reactions, salt imbalances, stomach upset, and other effects. Medications that suppress the immune system can result in infections, kidney or liver damage, and other effects. Surgery is associated with bleeding, infection, and allergic reactions to anesthesia. A heart transplant is a high-risk operation and the body may reject the new heart.
What happens after treatment for the disease?
A person with restrictive cardiomyopathy will need lifelong treatment.
How is the disease monitored?
The person will work
together with the healthcare provider to monitor symptoms, the effects of
treatment, and medications. This often involves blood tests and repeated
imaging tests of the heart. Any new or worsening symptoms should be reported
to the healthcare provider.