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Journal of Cardiovascular Magnetic Resonance
Published in: Journal of Cardiovascular Magnetic Resonance 1/2014

Open Access 01-12-2014 | Research

Reproducibility of native myocardial T1 mapping in the assessment of Fabry disease and its role in early detection of cardiac involvement by cardiovascular magnetic resonance

Authors: Silvia Pica, Daniel M Sado, Viviana Maestrini, Marianna Fontana, Steven K White, Thomas Treibel, Gabriella Captur, Sarah Anderson, Stefan K Piechnik, Matthew D Robson, Robin H Lachmann, Elaine Murphy, Atul Mehta, Derralyn Hughes, Peter Kellman, Perry M Elliott, Anna S Herrey, James C Moon

Published in: Journal of Cardiovascular Magnetic Resonance | Issue 1/2014

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Abstract

Background

Cardiovascular magnetic resonance (CMR) derived native myocardial T1 is decreased in patients with Fabry disease even before left ventricular hypertrophy (LVH) occurs and may be the first non-invasive measure of myocyte sphingolipid storage. The relationship of native T1 lowering prior to hypertrophy and other candidate early phenotype markers are unknown. Furthermore, the reproducibility of T1 mapping has never been assessed in Fabry disease.

Methods

Sixty-three patients, 34 (54%) female, mean age 48 ± 15 years with confirmed (genotyped) Fabry disease underwent CMR, ECG and echocardiographic assessment. LVH was absent in 25 (40%) patients. Native T1 mapping was performed with both Modified Look-Locker Inversion recovery (MOLLI) sequences and a shortened version (ShMOLLI) at 1.5 Tesla. Twenty-one patients underwent a second scan within 24 hours to assess inter-study reproducibility. Results were compared with 63 healthy age and gender-matched volunteers.

Results

Mean native T1 in Fabry disease (LVH positive), (LVH negative) and healthy volunteers was 853 ± 50 ms, 904 ± 46 ms and 968 ± 32 ms (for all p < 0.0001) by ShMOLLI sequences. Native T1 showed high inter-study, intra-observer and inter-observer agreement with intra-class correlation coefficients (ICC) of 0.99, 0.98, 0.97 (ShMOLLI) and 0.98, 0.98, 0.98 (MOLLI). In Fabry disease LVH negative individuals, low native T1 was associated with reduced echocardiographic-based global longitudinal speckle tracking strain (−18 ± 2% vs −22 ± 2%, p = 0.001) and early diastolic function impairment (E/E’ = 7 [6–8] vs 5 [5–6], p = 0.028).

Conclusion

Native T1 mapping in Fabry disease is a reproducible technique. T1 reduction prior to the onset of LVH is associated with early diastolic and systolic changes measured by echocardiography.
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Metadata
Title
Reproducibility of native myocardial T1 mapping in the assessment of Fabry disease and its role in early detection of cardiac involvement by cardiovascular magnetic resonance
Authors
Silvia Pica
Daniel M Sado
Viviana Maestrini
Marianna Fontana
Steven K White
Thomas Treibel
Gabriella Captur
Sarah Anderson
Stefan K Piechnik
Matthew D Robson
Robin H Lachmann
Elaine Murphy
Atul Mehta
Derralyn Hughes
Peter Kellman
Perry M Elliott
Anna S Herrey
James C Moon
Publication date
01-12-2014
Publisher
BioMed Central
Published in
Journal of Cardiovascular Magnetic Resonance / Issue 1/2014
Electronic ISSN: 1532-429X
DOI
https://doi.org/10.1186/s12968-014-0099-4

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