Noninvasive mapping of the electrophysiological substrate in cardiac amyloidosis and its relationship to structural abnormalities

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Title: Noninvasive mapping of the electrophysiological substrate in cardiac amyloidosis and its relationship to structural abnormalities
Authors: Orini, M
Graham, AJ
Martinez‐Naharro, A
Andrews, CM
De Marvao, A
Statton, B
Cook, SA
O'Regan, DP
Hawkins, PN
Rudy, Y
Fontana, M
Lambiase, PD
Item Type: Journal Article
Abstract: Background The relationship between structural pathology and electrophysiological substrate in cardiac amyloidosis is unclear. Differences between light‐chain (AL) and transthyretin (ATTR) cardiac amyloidosis may have prognostic implications. Methods and Results ECG imaging and cardiac magnetic resonance studies were conducted in 21 cardiac amyloidosis patients (11 AL and 10 ATTR). Healthy volunteers were included as controls. With respect to ATTR, AL patients had lower amyloid volume (51.0/37.7 versus 73.7/16.4 mL, P=0.04), lower myocardial cell volume (42.6/19.1 versus 58.5/17.2 mL, P=0.021), and higher T1 (1172/64 versus 1109/80 ms, P=0.022) and T2 (53.4/2.9 versus 50.0/3.1 ms, P=0.003). ECG imaging revealed differences between cardiac amyloidosis and control patients in virtually all conduction‐repolarization parameters. With respect to ATTR, AL patients had lower epicardial signal amplitude (1.07/0.46 versus 1.83/1.26 mV, P=0.026), greater epicardial signal fractionation (P=0.019), and slightly higher dispersion of repolarization (187.6/65 versus 158.3/40 ms, P=0.062). No significant difference between AL and ATTR patients was found using the standard 12‐lead ECG. T1 correlated with epicardial signal amplitude (cc=−0.78), and extracellular volume with epicardial signal fractionation (cc=0.48) and repolarization time (cc=0.43). Univariate models based on single features from both cardiac magnetic resonance and ECG imaging classified AL and ATTR patients with an accuracy of 70% to 80%. Conclusions In this exploratory study cardiac amyloidosis was associated with ventricular conduction and repolarization abnormalities, which were more pronounced in AL than in ATTR. Combined ECG imaging–cardiac magnetic resonance analysis supports the hypothesis that additional mechanisms beyond infiltration may contribute to myocardial damage in AL amyloidosis. Further studies are needed to assess the clinical impact of this approach.
Issue Date: 17-Sep-2019
Date of Acceptance: 30-Jul-2019
ISSN: 2047-9980
Publisher: American Heart Association
Journal / Book Title: Journal of the American Heart Association
Volume: 8
Issue: 18
Copyright Statement: © 2019 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley. This is an open access article under the terms of the Creative Commons Attribution License (, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Sponsor/Funder: Imperial College Healthcare NHS Trust- BRC Funding
Imperial College Healthcare NHS Trust- BRC Funding
Funder's Grant Number: RDC04
Keywords: 1102 Cardiorespiratory Medicine and Haematology
Publication Status: Published
Open Access location:
Article Number: e012097
Online Publication Date: 2019-09-08
Appears in Collections:Clinical Sciences
Imaging Sciences

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