PMID- 24412186
OWN - NLM
STAT- MEDLINE
DCOM- 20150618
LR  - 20220409
IS  - 1876-7591 (Electronic)
IS  - 1876-7591 (Linking)
VI  - 7
IP  - 2
DP  - 2014 Feb
TI  - CMR-based differentiation of AL and ATTR cardiac amyloidosis.
PG  - 133-42
LID - S1936-878X(13)00819-X [pii]
LID - 10.1016/j.jcmg.2013.08.015 [doi]
AB  - OBJECTIVES: This study was devised to describe the different cardiac magnetic 
      resonance (CMR) appearances in light chain amyloid (AL) and transthyretin-related 
      amyloidosis (ATTR). BACKGROUND: CMR is increasingly used to investigate patients 
      with suspected amyloidosis. Global subendocardial late gadolinium enhancement 
      (LGE) has been reported as typical of AL amyloidosis, whereas different patterns 
      have been noted in ATTR amyloidosis. METHODS: We performed de novo analyses on 
      original DICOM magnetic resonance imaging in 46 patients with cardiac AL 
      amyloidosis and 51 patients with ATTR type who had been referred to a specialist 
      amyloidosis center between 2007 and 2012 after CMR. Histological examination was 
      performed in all cases, with immunohistochemistry, to confirm systemic 
      amyloidosis. RESULTS: Patients' median age was 68 +/- 10 years, and 74% were male. 
      Left ventricular mass was markedly increased in ATTR amyloidosis (228 g [202 to 
      267 g]) compared with AL type (167 g [137 to 191 g]) (p < 0.001). LGE was 
      detected in all but 1 cardiac amyloidosis patient (AL type) and was substantially 
      more extensive in ATTR compared with AL amyloidosis. Ninety percent of ATTR 
      patients demonstrated transmural LGE compared with 37% of AL patients (p < 
      0.001). Right ventricular LGE was apparent in all ATTR patients but in only 33 AL 
      patients (72%) (p < 0.001). Despite these findings, survival was significantly 
      better in cardiac ATTR amyloidosis compared with AL type. We derived an LGE 
      scoring system (Query Amyloid Late Enhancement) that independently differentiated 
      ATTR from AL amyloidosis and, when incorporated into a logistic regression model 
      with age and wall thickness, detected ATTR type with 87% sensitivity and 96% 
      specificity. CONCLUSIONS: Transmural patterns of LGE distinguished ATTR from AL 
      cardiac amyloidosis with high accuracy in this real-world analysis of CMR. 
      Precise diagnosis of cardiac amyloidosis is crucial given the role of 
      chemotherapy in AL type and with novel therapies for ATTR type currently in 
      development.
CI  - Copyright (c) 2014 American College of Cardiology Foundation. Published by Elsevier 
      Inc. All rights reserved.
FAU - Dungu, Jason N
AU  - Dungu JN
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom; Cardiovascular Sciences, St. George's University of London, 
      London, United Kingdom. Electronic address: jdungu@sgul.ac.uk.
FAU - Valencia, Oswaldo
AU  - Valencia O
AD  - Cardiovascular Sciences, St. George's University of London, London, United 
      Kingdom.
FAU - Pinney, Jennifer H
AU  - Pinney JH
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Gibbs, Simon D J
AU  - Gibbs SD
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Rowczenio, Dorota
AU  - Rowczenio D
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Gilbertson, Janet A
AU  - Gilbertson JA
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Lachmann, Helen J
AU  - Lachmann HJ
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Wechalekar, Ashutosh
AU  - Wechalekar A
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Gillmore, Julian D
AU  - Gillmore JD
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Whelan, Carol J
AU  - Whelan CJ
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Hawkins, Philip N
AU  - Hawkins PN
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, 
      United Kingdom.
FAU - Anderson, Lisa J
AU  - Anderson LJ
AD  - Cardiovascular Sciences, St. George's University of London, London, United 
      Kingdom.
LA  - eng
GR  - FS/09/063/28026/British Heart Foundation/United Kingdom
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20140108
PL  - United States
TA  - JACC Cardiovasc Imaging
JT  - JACC. Cardiovascular imaging
JID - 101467978
RN  - AU0V1LM3JT (Gadolinium)
RN  - Amyloidosis, Hereditary, Transthyretin-Related
SB  - IM
CIN - Nat Rev Cardiol. 2014 Mar;11(3):125. PMID: 24469675
CIN - JACC Cardiovasc Imaging. 2014 Feb;7(2):166-8. PMID: 24524743
CIN - JACC Cardiovasc Imaging. 2014 Feb;7(2):210-1. PMID: 24524750
CIN - JACC Cardiovasc Imaging. 2014 Oct;7(10):1067-8. PMID: 25323171
CIN - JACC Cardiovasc Imaging. 2014 Oct;7(10):1068. PMID: 25323172
MH  - Aged
MH  - Amyloid Neuropathies, Familial
MH  - Amyloidosis/*diagnosis
MH  - Diagnosis, Differential
MH  - Female
MH  - Gadolinium
MH  - Heart Diseases/*diagnosis
MH  - Humans
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Middle Aged
MH  - Prognosis
MH  - Sensitivity and Specificity
OTO - NOTNLM
OT  - amyloid
OT  - cardiomyopathy
OT  - magnetic resonance imaging
EDAT- 2014/01/15 06:00
MHDA- 2015/06/19 06:00
CRDT- 2014/01/14 06:00
PHST- 2013/07/25 00:00 [received]
PHST- 2013/08/23 00:00 [accepted]
PHST- 2014/01/14 06:00 [entrez]
PHST- 2014/01/15 06:00 [pubmed]
PHST- 2015/06/19 06:00 [medline]
AID - S1936-878X(13)00819-X [pii]
AID - 10.1016/j.jcmg.2013.08.015 [doi]
PST - ppublish
SO  - JACC Cardiovasc Imaging. 2014 Feb;7(2):133-42. doi: 10.1016/j.jcmg.2013.08.015. 
      Epub 2014 Jan 8.