source: Journal of Nuclear Cardiology

year: 2019

authors: Sharmila Dorbala, Rodney Falk

Amyloidosis is a disease that develops when abnormally folded proteins collect together to form amyloid fibrils, which deposit in various organs and cause organ damage. Amyloidosis in the heart can be identified by using various imaging tests. This patient page provides an overview of imaging in cardiac amyloidosis.

Cardiac amyloidosis is caused by the deposition of amyloid fibrils between the heart muscle cells. The two most common types of protein fibrils depositing in the heart are light chain (AL) and transthyretin (ATTR) proteins. While light chain amyloidosis results from an excess of abnormal light chain proteins produced by cancerous plasma cells in the bone marrow, transthyretin amyloidosis may result from misfolding healthy TTR proteins produced by the liver, as in wild type amyloidosis of aging. Alternatively, a genetic mutation of the TTR gene can result in altered TTR protein that aggregates and causes hereditary ATTR amyloidosis. Whatever the cause, a build-up of amyloid fibrils in the heart may keep the heart muscle from relaxing between heartbeats, causing high pressure in the heart chambers and blood vessels. Eventually, the heart may not be able to squeeze blood adequately to supply oxygen to the body.

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