cardiac amyloidosis
Infiltrative cardiomyopathy caused by deposition of misfolded proteins in the heart muscle, requiring specific imaging techniques for diagnosis.
Full Definition
Cardiac amyloidosis is a progressive infiltrative disease characterized by the deposition of insoluble amyloid fibrils in the myocardium, leading to restrictive cardiomyopathy. The two main types affecting the heart are light chain (AL) and transthyretin (ATTR) amyloidosis. Cardiac imaging plays a crucial role in diagnosis, with characteristic findings including increased left ventricular wall thickness, granular sparkling appearance on echocardiography, and specific patterns on cardiac MRI and nuclear imaging. Early recognition is essential as treatment options vary significantly between subtypes.
Usage
Usage note: Specify the amyloid subtype (AL or ATTR) when known, as treatment approaches differ significantly.
In Context
- "Cardiac amyloidosis was suspected based on the characteristic granular sparkling pattern seen on echocardiography." — Echocardiography report
- "Technetium-99m pyrophosphate scintigraphy confirmed transthyretin cardiac amyloidosis with grade 3 uptake." — Nuclear cardiology report