Anderson, Lisa

Banypersad, Sanjay M

Dungu, Jason

Gibbs, Simon D

Gillmore, Julian D

Hawkins, Philip N

Lachmann, Helen J

Pinney, Jennifer H

Venner, Christopher P

Wechalekar, Ashutosh

Whelan, Carol J

English

PubMed

Jason Dungu

Carol J Whelan

Simon D Gibbs

Jennifer H Pinney

Sanjay M Banypersad

Christopher P Venner

Helen J Lachmann

Ashutosh Wechalekar

Julian D Gillmore

Philip N Hawkins

Lisa Anderson

Crossref

Journal of Cardiovascular Magnetic Resonance

Patterns of late gadolinium enhancement in 94 patients with AL or transthyretin cardiac amyloidosis

Dungu Jason

Whelan Carol J

Gibbs Simon D

Pinney Jennifer H

Banypersad Sanjay M

Venner Christopher P

Lachmann Helen J

Wechalekar Ashutosh

Gillmore Julian D

Hawkins Philip N

Anderson Lisa

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ORAL PRESENTATION Open Access
Patterns of late gadolinium enhancement in 94
patients with AL or transthyretin cardiac
amyloidosis
Jason Dungu1,2*, Carol J Whelan1, Simon D Gibbs1, Jennifer H Pinney1, Sanjay M Banypersad1,
Christopher P Venner1, Helen J Lachmann1, Ashutosh Wechalekar1, Julian D Gillmore1, Philip N Hawkins1,
Lisa Anderson2
From 15th Annual SCMR Scientific Sessions
Orlando, FL, USA. 2-5 February 2012
Background
Cardiac MRI (CMR) is increasingly used to further
investigate patients in whom amyloidosis is suspected
on echocardiography. Late gadolinium enhancement
(LGE) reflects expansion of the interstitium, and cir-
cumferential subendocardial LGE has been reported to
be a typical finding in AL amyloidosis; by contrast, a
more diffuse transmural LGE pattern has been asso-
ciated with ATTR (transthyretin amyloidosis).
Methods
We studied patients who had been referred to the UK
National Amyloidosis Centre (NAC) between June 2007
and June 2011 with a possible diagnosis of cardiac amy-
loidosis based on the findings of CMR performed at
their local centres. We analysed the CMR images and
LGE patterns in the left ventricle at the base, mid wall
and apex. The LGE pattern at each level was described
as circumferential if it involved all segments at either
the base, mid or apical levels, and transmural if it
involved the subendocardium through to the epicardial
layer.
Results
Studies were performed in 32 UK centres, and we were
able to obtain 103 studies for analysis. The mean age of
the patients was 69.2 ± 11.4 years with a male predomi-
nance (72.7%). Amyloidosis was confirmed at the NAC
in 99 patients and excluded in 4 patients. The amyloid
type was AL in 39 patients (39.4%), ATTR in 55 patients
(55.5%) and rarer forms of non-AL, non-TTR amyloid in
5 patients (5.1%). We report the CMR findings in 94
patients with AL or ATTR. Interventricular wall thick-
ness was greater in ATTR compared to AL (16.7 mm ±
2.5 vs 14.2 mm ± 2.1, p <0.01). LGE was evident in all
patients with ATTR amyloid and 36 patients (92.3%)
with AL (p=0.07). Right ventricular (RV) LGE was also
present in all patients with TTR amyloid and 26 patients
(66.7%) with AL (p <0.01). A gradient with greater LGE
at the base than the apex was more often present in
ATTR (62.5% vs 35.9% in AL - p <0.01). Circumferential
subendocardial LGE was present in only 8 patients
(20.5%) with AL and only 2 patients (3.6%) with ATTR
(p <0.01). Circumferential transmural LGE was present
in 6 patients with ATTR (10.7%) and none with AL
amyloid (p<0.01). Fifty-two patients with ATTR (92.9%)
demonstrated transmural LGE in at least one segment
compared to 12 patients (30.8%) with AL amyloid (p
<0.01).
Conclusions
This study of CMR performed in multiple regional cen-
tres confirms its high diagnostic value in cardiac amyloi-
dosis. Whilst greater wall thickness and a transmural
pattern of LGE was more suggestive of ATTR, only the
absence of RV LGE in one-third of AL patients reliably
distinguished the two types of amyloid.
Funding
Funding for this study was obtained from the British
Heart Foundation Clinical Research Training Fellowship
no. FS/09/063/28026 to J.D.1UK National Amyloidosis Centre, London, UK
Full list of author information is available at the end of the article
Dungu et al. Journal of Cardiovascular Magnetic Resonance 2012, 14(Suppl 1):O87
http://www.jcmr-online.com/content/14/S1/O87
© 2012 Dungu et al; licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons
Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited.
Author details
1UK National Amyloidosis Centre, London, UK. 2St George’s University of
London, London, UK.
Published: 1 February 2012
doi:10.1186/1532-429X-14-S1-O87
Cite this article as: Dungu et al.: Patterns of late gadolinium
enhancement in 94 patients with AL or transthyretin cardiac
amyloidosis. Journal of Cardiovascular Magnetic Resonance 2012 14(Suppl
1):O87.
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Table 1 Comparison of the cardiac MRI features in patients with AL and TTR amyloidosis.
AL (n=39) ATTR (n=55) P value
Age (years) 64.3 ± 9.6 74.1 ± 8.6 <0.01
Survival (months) 18 48 <0.01
NT pro-BNP (pMol/L) 644 (84-7323) 497 (43-2791) 0.177
Interventricular septum (mm) 14.2 ± 2.1 16.9 ± 2.2 <0.01
Late gadolinium enhancement (LGE) 36 (92.3%) 55 (100%) 0.07
RV LGE 26 (66.7%) 55 (100%) <0.01
Circumferential subendocardial LGE 8 (20.5%) 2 (3.6%) 0.01
Circumferential transmural LGE 0 (0%) 6 (10.9%) <0.01
Base-apex gradient 14 (35.9%) 35 (63.6%) 0.01
Any segment transmural LGE 12 (30.8%) 52 (94.5%) <0.01
Figure 1 A late gadolinium enhancement image in a patient with
ATTR demonstrating biventricular thickening, transmural
enhancement at the base, a base-apex gradient and right
ventricular involvement.
Dungu et al. Journal of Cardiovascular Magnetic Resonance 2012, 14(Suppl 1):O87
http://www.jcmr-online.com/content/14/S1/O87
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Patterns of late gadolinium enhancement in 94 patients with AL or transthyretin cardiac amyloidosis

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