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Related Concept Videos

Amyloid Fibrils03:03

Amyloid Fibrils

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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
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Visualization of Amyloid β Deposits in the Human Brain with Matrix-assisted Laser Desorption/Ionization Imaging Mass Spectrometry
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Alterations in multi-layer strain in AL amyloidosis.

Shanthosh Sivapathan1, Paul Geenty1,2, Tejas Deshmukh1,2

  • 1Westmead Clinical School, University of Sydney, Sydney, Australia.

Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis
|February 21, 2022
PubMed
Summary

Cardiac strain analysis using 2D-speckle tracking echocardiography (2D-STE) reveals early myocardial involvement in AL amyloidosis. Reduced basal segmental longitudinal strain is a key indicator of this condition.

Keywords:
AL amyloidosiscardiac amyloidechocardiographyglobal longitudinal strainspeckle trackingstrain

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Area of Science:

  • Cardiology
  • Medical Imaging
  • Biophysics

Background:

  • Cardiac involvement in AL amyloidosis is associated with poor prognosis.
  • Subclinical cardiac changes in AL amyloidosis can be detected using 2D-speckle tracking echocardiography (2D-STE) strain analysis.
  • This study investigated multilayer and multiplanar 2D-STE myocardial strain.

Purpose of the Study:

  • To evaluate the utility of multilayer and multiplanar 2D-STE strain analysis in identifying cardiac involvement in AL amyloidosis.
  • To compare myocardial strain patterns in AL amyloidosis patients with hypertensive patients and healthy controls.
  • To identify specific strain parameters indicative of early-stage AL amyloidosis.

Main Methods:

  • Compared 75 AL amyloidosis patients with 49 hypertensive patients and 49 healthy controls.
  • Acquired longitudinal strain data from epicardial, mid-myocardial, and endocardial layers.
  • Measured segmental strain from basal, mid, and apical segments of the left ventricle (LV) myocardium.

Main Results:

  • Global longitudinal strain was significantly reduced across all myocardial layers (epicardial, mid-myocardial, endocardial) in AL amyloidosis patients compared to controls (p < 0.001).
  • Segmental strain analysis revealed significant reductions in basal and mid-LV segments in the AL amyloidosis group.
  • Basal segmental strain (cut-off -16%) demonstrated high sensitivity (96%) and specificity (70%) in differentiating AL amyloidosis from hypertension (AUC 0.93).

Conclusions:

  • 2D-STE strain analysis reveals myocardial involvement in all layers in AL amyloidosis.
  • Reduced basal segmental longitudinal strain is a sensitive marker for early-stage cardiac involvement in AL amyloidosis.
  • 2D-STE strain imaging is valuable for early detection and characterization of cardiac dysfunction in AL amyloidosis.