Cardiac Amyloidosis (CA or Stiff Heart Disorder)

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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On this page

• What Is CA?
• What Are the Symptoms of CA?
• How Do You Diagnose CA?
• How Do You Treat CA?

What is Cardiac Amyloidosis (CA)?

CA is a type of heart disease caused by abnormal proteins in the body.

Proteins do many jobs in the body, such as:

• Fight infection.
• Cause chemical reactions in cells.
• Carry molecules from cell to cell.
• Give structure to cells.

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:

• Liver
• Nerves
• Kidneys
• Digestive tract
• Spleen
• Heart

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.

What are the types of CA?

There are many types of amyloidosis.

The two most common types that affect the heart are ATTR and AL.

ATTR (transthyretin) amyloidosis

• The transthyretin protein makes this type of amyloid. The protein, normally found in the blood, can affect the heart or nerves.
• Amyloid production may be due to a faulty gene (hereditary) or for reasons related to aging that doctors don't fully understand.
• A cardiologist or neurologist diagnoses ATTR of the heart with a nuclear scan. In the past, they could only diagnose it with a heart biopsy.
• Since diagnosis has become easier, doctors now realize ATTR is much more common than they once thought.

AL amyloidosis

• Bone marrow cells (the part inside the bones that makes blood cells) produce this type of amyloid.
• AL amyloidosis can affect the heart, but also the kidneys, skin, nerves, and liver.
• Because bone marrow makes the amyloid cells, doctors regard AL as a blood cancer.
• A hematologist diagnoses and treats AL amyloidosis.

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.

Complications of CA

Left untreated, CA can cause:

• Heart failure.
• AFib.
• Heart block (in which the nerves fail to tell parts of the heart to pump).

Some kinds of amyloidosis progress quicker than others, so it's crucial to know which type you have.

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What Are The Signs And Symptoms Of CA?

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:

• Shortness of breath and tiredness.
• A cough, especially when lying down.
• Dizziness.
• Irregular heart rhythm.
• Swelling in the belly or ankles and legs.

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How Do You Diagnose CA or Stiff Heart Disorder?

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:

• Blood work.
• An electrocardiogram (ECG), which measures electrical impulses in the heart.
• Scans such as echocardiography, nuclear imaging, and MRI.

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.

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How Do You Treat CA?

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.

ATTR amyloidosis treatments

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.

AL amyloidosis treatments

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.

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Why Choose UPMC for CA Care?

The UPMC is one of only a few amyloidosis programs in the country.

What sets us apart?

• Our specially trained team focuses only on amyloidosis of the heart to provide each person the best care.
• We've changed the way we diagnose CA. Recent research now lets us use a non-invasive nuclear heart scan instead of a biopsy.
• Our team includes scientists who dedicate their careers to advancing our knowledge of this disorder and finding new treatments.
• We use the latest CA treatments and drugs as soon as they come to market.

By UPMC Editorial Staff. Last reviewed on 2024-10-01.