CA, or stiff heart syndrome, is a condition that affects the heart tissue. There are a few types of amyloidosis, all caused by abnormal proteins in the body.
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CA is a type of heart disease caused by abnormal proteins in the body.
Proteins do many jobs in the body, such as:
Normal proteins "fold" when they do their jobs. When proteins don't fold properly, they clump together and become amyloid proteins. Amyloidosis occurs when these proteins get caught in tissues where they shouldn't be.
Amyloidosis can affect many body parts, including the:
Doctors define CA as a buildup of amyloid proteins in the heart muscle. When amyloid deposits accrue, the heart becomes stiff and can't pump enough blood.
Doctors used to consider CA a rare disease.
Before, they estimated around 200,000 people in the U.S. had amyloidosis. Since CA is now much easier to diagnose with a nuclear cardiology scan, doctors realize it may not be rare at all. And the number of cases may be much higher.
There are many types of amyloidosis.
The two most common types that affect the heart are ATTR and AL.
Other types of amyloidosis, such as AA, rarely affect the heart. Causes of AA amyloidosis include a long-lasting infection or inflammation from conditions like rheumatoid arthritis.
Left untreated, CA can cause:
Some kinds of amyloidosis progress quicker than others, so it's crucial to know which type you have.
The UPMC Cardiac Amyloidosis Center is one of only a few amyloidosis programs in the country.
What sets us apart?
When amyloid proteins build up in the heart, the heart gets stiff and can't pump enough blood. If the amyloid affects the nerves in the heart, the heart won't pump right.
CA can cause symptoms like those of other heart conditions. If you think you're having signs of heart problems, you should always see a doctor as soon as possible.
Some symptoms of CA and other heart problems are:
To know whether you're at risk for CA, our team will talk with about your medical and family history of this condition.
Our doctors will examine you to look for symptoms of CA.
If you have risk factors and symptoms, you may need to have some tests such as:
If your doctor diagnoses you with CA, you may need more tests. These will help your doctor see if you have amyloidosis in any other organs or parts of your body.
Treatment for your CA varies with the type you have. The ultimate goal of treating CA is to reduce your symptoms while making sure the condition doesn't get worse.
Newer drugs have recently become available to treat CA by targeting the abnormal proteins. We use diuretics (water pills) to treat other symptoms of heart problems, like swelling. They help the body get rid of excess fluid.
New ATTR amyloidosis treatments are quickly becoming ready for use. Our team of doctors will talk to you about the latest treatments and research studies.
If you have AL amyloidosis, you may need to have chemo or maybe even a stem cell transplant. These treatments stop the production of the amyloid protein.
Because the AL type of amyloidosis can progress very fast, you need prompt treatment by a hematologist.
The UPMC Heart and Vascular Institute offers resources and videos about CA and other conditions.
Many people find these resources helpful in getting ready for a procedure or diagnostic test and answering their questions about their condition.
The links below will open a new browser window: