cardiac amyloidosis
Infiltrative cardiomyopathy caused by deposition of misfolded proteins in the myocardium, detectable by advanced cardiac imaging.
Full Definition
Cardiac amyloidosis is a progressive infiltrative cardiomyopathy characterized by the extracellular deposition of insoluble amyloid fibrils in the myocardium. The condition can result from different protein types, with light chain (AL) and transthyretin (ATTR) amyloidosis being the most common forms affecting the heart. Advanced cardiac imaging plays a crucial role in diagnosis, with characteristic features including late gadolinium enhancement patterns, abnormal T1 mapping values, and specific echocardiographic findings such as increased wall thickness with reduced voltage on ECG. Early detection through imaging is essential as treatment options differ significantly between amyloid types.
Usage
Usage note: Specify amyloid type when known (AL, ATTR, etc.).
In Context
- "T1 mapping revealed markedly reduced values suggestive of cardiac amyloidosis." — Differential diagnosis report
- "The pattern of late gadolinium enhancement was typical of cardiac amyloidosis with global subendocardial involvement." — Cardiac MRI interpretation