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.
Types, Causes, and Risk Factors for 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.
Why Choose UPMC for CA Care?
The UPMC Cardiac Amyloidosis Center 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.