Poster presentationpublished_or_final_versio

Calleja, A

Crean, A

Deva, DP

Gruner, C

Iwanochko, RM

Li, Q

Ng, MY

Thavendiranathan, P

Journal of Cardiovascular Magnetic Resonance

PubMed

Qin Li

Ming-Yen Ng

Anna Calleja

Djeven P Deva

Andrew Crean

Christiane Gruner

Robert M Iwanochko

Paaladinesh Thavendiranathan

Springer - Publisher Connector

POSTER PRESENTATION Open Access
Relationship between MRI derived right
ventricular mass and left ventricular involvement
in patients with anderson-fabry disease
Qin Li3,1, Ming-Yen Ng1,2*, Anna Calleja3, Djeven P Deva1,4, Andrew Crean1,3, Christiane Gruner3,
Robert M Iwanochko3, Paaladinesh Thavendiranathan3,1
From 18th Annual SCMR Scientific Sessions
Nice, France. 4-7 February 2015
Background
Anderson-Fabry’s Disease is an X-linked lysosomal storage
disorder in which there is an accumulation of globotriao-
sylceramide in both atria and ventricles as well as valvular
tissue. We sought to investigate if there was a correlation
on cardiac MRI between the degree of left ventricular (LV)
involvement (ie. LV mass or late gadolinium enhance-
ment) and right ventricular mass.
Methods
The study was approved by the research ethics board.
Patients with Anderson-Fabry’s disease were identified
through the Metabolic Genetic Disease Clinic between 1st
January 2000 and 31st December 2013. We included only
patients with genotyping and leucocyte alpha galactosidase
A activity test confirming a diagnosis of Anderson-Fabry’s
disease. Clinical data obtained included age and body mass
index. Cardiac MRI was analysed using CMR 42 (Circle
Cardiovascular Imaging) to measure LV and right ventri-
cular (RV) volumes, ejection fraction and mass using short
axis steady state free precession (SSFP) cine images (see
figure 1). Presence of late gadolinium enhancement (LGE)
and degree of LGE was calculated using a threshold of 6
SD above a remote region. Right ventricular hypertrophy
(RVH) was defined as >28g/m2 for males and >27g/m2 for
women. This threshold was based on a paper by Hudsmith
et al (2005 Journal of Cardiovascular Magnetic
Resonance).
1Radiology, University of Toronto, Toronto, ON, Canada
Full list of author information is available at the end of the article
Figure 1 Assessment of RV volume, function, and mass using short axis SSFP cine images (CMR42). The RV septal band was included in the
assessment of the RV mass.
Li et al. Journal of Cardiovascular Magnetic
Resonance 2015, 17(Suppl 1):P275
http://www.jcmr-online.com/content/17/S1/P275
© 2015 Ng 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/4.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://
creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Comparisons between RV mass, LV mass and LGE were
performed using Pearson’s correlation and unpaired stu-
dent t-test.
Results
43 patients (22 males) with a mean age of 45 ± 14yrs were
included. There was good correlation between LV and
RVEF (r=0.59, p<0.01), and LV and RV mass index
(r=0.67, p<0.01). Total LV scar was higher in patients with
right ventricular hypertrophy (RVH) (12.48g ± 24g) than
in those without RVH (1.44g ± 2.29g) (p=0.0047). There
was also a statistically significant difference between the
LV mass of patients with increased RV mass (117.03g/m2
+/- 69g/m2) compared to patients with normal RV mass
(71g/m2 +/- 28.7g/m2) (see table 1).
Conclusions
Patients with Anderson-Fabry’s disease with increased LV
mass and LGE had a positive correlation with increased
RV mass. RV mass changes appear to occur in more
advanced myocardial involvement with Anderson-Fabry’s
disease.
Funding
None.
Authors’ details
1Radiology, University of Toronto, Toronto, ON, Canada. 2Radiology, The
University of Hong Kong, Hong Kong, Hong Kong. 3Cardiology, University of
Toronto, Toronto, ON, Canada. 4Radiology, St. Michael’s Hospital, Toronto,
ON, Canada.
Published: 3 February 2015
doi:10.1186/1532-429X-17-S1-P275
Cite this article as: Li et al.: Relationship between MRI derived right
ventricular mass and left ventricular involvement in patients with
anderson-fabry disease. Journal of Cardiovascular Magnetic Resonance
2015 17(Suppl 1):P275.
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Table 1 Comparison of LV mass and LGE quantification
in Anderson-Fabry’s patients with increased and normal
RV mass with p-values from unpaired t-test.
Increased RV mass Normal RV mass p-value
LV mass (g/m2) 117.03 ±69 71 ±28.7 0.0146
LGE 6SD (g) 12.48 ±24 1.44 ±2.29 0.0047
Li et al. Journal of Cardiovascular Magnetic
Resonance 2015, 17(Suppl 1):P275
http://www.jcmr-online.com/content/17/S1/P275
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