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Amyloid
The Journal of Protein Folding Disorders
Volume 15, 2008 - Issue 3
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Original Article

Late enhancement in cardiac amyloidosis: correlation of MRI enhancement pattern with histopathological findings

, , , , , , , , , , , , & show all
Pages 196-204 | Published online: 06 Jul 2009
 

Abstract

Late enhancement (LE) in cardiac magnetic resonance imaging (MRI) is a characteristic finding in patients with cardiac amyloidosis (CA) but the histomorphological explanation has not been clarified yet. Five patients with CA were evaluated by MRI prior to heart transplantation. This consisted of morphological, volumetric, and functional data, including LE analysis. For LE analysis, left ventricular (LV) short-axis sections from basal, midventricular, and apical positions were divided into 12 segments resulting in a 36-segment model. Each segment was differentiated by subendocardial, midmural, and subepicardial localization. Histological amyloid and collagenous fiber deposition was correlated with LE in corresponding MRI slides. LE was visualized in 103/180 (57.2%) predominantly subendocardial segments. Histological analysis of amyloid deposition was (peri-)vascular (n = 5), diffuse interstitial (n = 3) and/or nodular (n = 4). Extent of fibrosis was moderate to severe. Cytoplasmatic vacuolization and decline of myofibrils was seen in all patients. Fibrosis was significantly associated with LE in subendocardial and midmural localizations (p<0.05), whereas the extent of amyloid deposition was not associated with LE findings in any region. LE seems to be associated with fibrosis due to ischemia of cardiomyocytes by small vessel amyloid deposition rather than with amyloid deposition in CA, suggesting that amyloid deposition might be present prior to LE detection.

Abbreviations
AL=

light-chain amyloidosis

ATTR=

transthyretin amyloidosis

MRI=

magnetic resonance imaging

CA=

cardiac amyloidosis

LE=

late enhancement

NT-proBNP=

N-terminal pro-natriuretic peptide

cTnT=

cardiac troponin T

SSFP=

steady-state free precession

trueFISP=

true fast imaging with steady-state free precession;

TE=

time to echo

TR=

time to repeat

TI=

inversion time

FLASH=

fast low angle shot

TIRM=

inversion recovery-turbo spin echo sequence

Gd-DTPA=

gadolinium diethylenetriamine penta-acetic acid

H&E=

hematoxylin-eosin

SEM=

standard error of mean

LV=

left ventricular

EDV=

end-diastolic volume

ESV=

end-systolic volume

SV=

stroke volume

EF=

ejection fraction

CI=

cardiac index

MI=

myocardial mass index

NYHA=

New York Heart Association functional heart failure class

Abbreviations
AL=

light-chain amyloidosis

ATTR=

transthyretin amyloidosis

MRI=

magnetic resonance imaging

CA=

cardiac amyloidosis

LE=

late enhancement

NT-proBNP=

N-terminal pro-natriuretic peptide

cTnT=

cardiac troponin T

SSFP=

steady-state free precession

trueFISP=

true fast imaging with steady-state free precession;

TE=

time to echo

TR=

time to repeat

TI=

inversion time

FLASH=

fast low angle shot

TIRM=

inversion recovery-turbo spin echo sequence

Gd-DTPA=

gadolinium diethylenetriamine penta-acetic acid

H&E=

hematoxylin-eosin

SEM=

standard error of mean

LV=

left ventricular

EDV=

end-diastolic volume

ESV=

end-systolic volume

SV=

stroke volume

EF=

ejection fraction

CI=

cardiac index

MI=

myocardial mass index

NYHA=

New York Heart Association functional heart failure class

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