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

Journal of Cardiovascular Magnetic Resonance

Research

Atul Mehta¹  Derralyn Hughes¹  Peter Kellman²  Marianna Fontana³  Sarah Anderson³  Silvia Pica⁴  Viviana Maestrini⁵  Thomas Treibel⁶  Gabriella Captur⁶  Perry M Elliott⁶  James C Moon⁶  Steven K White⁶  Anna S Herrey⁶  Daniel M Sado⁶  Stefan K Piechnik⁷  Matthew D Robson⁷  Elaine Murphy⁸  Robin H Lachmann⁸ 

[1] Lysosomal Storage Disorders Unit, Royal Free Hospital and University College London, London, UK;National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA;The Heart Hospital, 16-18 Westmoreland Street, W1G 8PH, London, UK;The Heart Hospital, 16-18 Westmoreland Street, W1G 8PH, London, UK;Department of Cardiology, IRCCS Policlinico San Matteo Hospital, Pavia, Italy;The Heart Hospital, 16-18 Westmoreland Street, W1G 8PH, London, UK;Department of Cardiovascular, Respiratory, Nephrologic, Anaesthesiologic and Geriatric Science, La Sapienza, University of Rome, Rome, Italy;The Heart Hospital, 16-18 Westmoreland Street, W1G 8PH, London, UK;Institute of Cardiovascular Science, University College London, WC1E 6BT, London, UK;The Heart Hospital, 16-18 Westmoreland Street, W1G 8PH, London, UK;Oxford Centre for Clinical Magnetic Resonance Research, Department of Cardiovascular Medicine, University of Oxford, OX3 9DU, Oxford, United Kingdom;The National Hospital for Neurology and Neurosurgery, Queens Square, London, UK;

关键词: Cardiovascular magnetic resonance;    T1 mapping;    Speckle-tracking strain;    Diastolic function;    Fabry disease;   

DOI  :  10.1186/s12968-014-0099-4

 received in 2014-06-30, accepted in 2014-11-17,  发布年份 2014

来源: Springer

【 摘 要 】

BackgroundCardiovascular 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.MethodsSixty-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.ResultsMean 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).ConclusionNative 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.

【 授权许可】

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© Pica et al.; licensee BioMed Central Ltd. 2014. This article is published under license to 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 credited. 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.