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Review
. 2022 Jul;18(3):479-488.
doi: 10.1016/j.hfc.2022.02.005.

Cardiac Amyloidosis

Morie A Gertz  1
Affiliations
Review

Cardiac Amyloidosis

Morie A Gertz. Heart Fail Clin. 2022 Jul.

Abstract

Amyloid deposits are defined by their tinctorial properties. Under the light microscope amyloid deposits are eosinophilic and amorphous when stained with hematoxylin and eosin. With Congo red staining the deposits are positive and under polarized light will exhibit green birefringence. Sixty years later electron microscopy demonstrated that all deposits were fibrillar. All amyloid deposits are protein derived. The clinical characteristics will be driven by the nature of the protein subunit. In cardiology, the 2 most common subunits accounting for well more than 90% of cardiac amyloidosis are either immunoglobulin light chain, amyloid light-chain (AL) amyloidosis, or transthyretin; transthyretin (TTR) amyloidosis. Although 70% of patients with systemic amyloidosis have cardiac involvement the diagnosis is made by cardiologists only 20% of the time, suggesting significant gaps in knowledge in how to establish a workflow to arrive at a diagnosis in everyday practice.

Keywords: Amyloid echocardiography; Amyloidosis; Cardiac transplantation; Chemotherapy; Light chains; Stem cell transplantation; Transthyretin.

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Figures

Figure 1:
Figure 1:
Courtesy of Mayo Clinic
Figure 2:
Figure 2:
Classic amyloid purpura
Figure 3:
Figure 3:
2D echo demonstrating marked thickening of the left ventricular septum and free wall
Figure 4:
Figure 4:
Technetium pyrophosphate scan showing 3+ uptake in the myocardium with a SPECT image demonstrating the radionucleotide localizes to the ventricle
See this image and copyright information in PMC

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