Amyloidosis occurs when abnormal proteins accumulate where normal proteins should be. When the condition develops in the heart, it is called cardiac amyloidosis. Abnormal protein deposits strain the heart, and it must work harder to function and supply blood to the rest of the body.
Cardiac amyloidosis is a rare but treatable condition. Without treatment, the accumulation of abnormal proteins can cause thickening of the heart walls or narrowing of heart valves, both of which reduce the amount of blood the heart is able to pump. The condition can also lead to problems with the electrical function of the heart, causing arrhythmia such as atrial fibrillation (AFib).
There are three types of cardiac amyloidosis.
One function of proteins in the body is to support the immune system. Part of this type of protein is called the “light chain.” When the body creates too many light chains, they can deposit in parts of the body, including the heart, causing problems for the organ.
Transthyretin is a type of protein that is made naturally and helps the body by transporting vitamin A and thyroid hormone. When this protein functions abnormally, the result is transthyretin amyloidosis (ATTR). ATTR has two sub-types:
Hereditary Amyloidosis – This type is genetic and is passed down through family members. Abnormal proteins affect different organs in the body, including the heart.
Wild-type Amyloidosis – This type is most often seen in adults older than age 60 without a family history of amyloidosis. Abnormal proteins affect the heart’s function.
Long-term dialysis patients may develop amyloidosis. Dialysis treatment can cause some proteins to remain in the system and accumulate, rather than being flushed out. This accumulation of proteins can happen in the heart.
The cause is often unknown. Sometimes cardiac amyloidosis is genetic; however, not everyone who has the genetic code for amyloidosis will actually develop the condition. Certain cancers, including multiple myeloma and lymphoma, can cause amyloidosis.
Some patients experience symptoms, while others do not. When symptoms are present, they may include:
Cardiac amyloidosis cannot be prevented, with the exception of dialysis-related amyloidosis. People who are at an increased risk of inheriting or developing cardiac amyloidosis often have these traits:
If your health care provider suspects cardiac amyloidosis, they may recommend testing to look for abnormal protein accumulation. Early detection is important to prevent prolonged damage to the heart. Testing may include:
Treatment recommendations vary based on the type of cardiac amyloidosis and the extent of heart damage. Some treatment options aim to reduce symptoms, including fluid retention, while others are used to limit the progression of the condition.
Health care providers may recommend treatment to alleviate symptoms, including:
Treatment to slow the progression of cardiac amyloidosis includes:
The success of treatment depends on how quickly cardiac amyloidosis is diagnosed and how much heart damage has been sustained. Without a heart transplant, patients with early-stage disease may live seven years or longer, while those with more advanced illness may have a significantly shorter life expectancy.
Because cardiac amyloidosis is a chronic, progressive disease, it is essential to receive an accurate diagnosis as quickly as possible. The esteemed cardiology specialists affiliated with Memorial Hermann have extensive experience identifying and treating amyloidosis. Our medical teams utilize a comprehensive approach to treat cardiac amyloidosis to optimize results.
Memorial Hermann patients have access to the full range of services to treat cardiac amyloidosis from medication to immunotherapy to highly specialized treatments offered at our academic flagship, the Larry D. Johnson Heart & Vascular Institute at Memorial Hermann-Texas Medical Center including protocols for severe, advanced cardiac amyloidosis to mechanical circulatory support options to heart transplantation.
If you’d like a referral to a heart and vascular specialist or want more information about our services, please fill out the form below or call us at (713) 219-3519. For other inquiries, such as obtaining medical records, imaging reports or test results, please call (713) 222-CARE (2273).