Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease

Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A. Approximately 50% of patients with AFD may have cardiac involvement. Gadolinium-enhanced cardiac magnetic resonance (CMR) is useful for the diagnosis of cardiac involvement of AFD...

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Autores principales: Kasuya, Shusuke, Suzuki, Masayo, Inaoka, Tsutomu, Odashima, Masayuki, Nakatsuka, Tomoya, Ishikawa, Rumiko, Tokuyama, Wataru, Terada, Hitoshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Ubiquity Press 2016
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6100657/
https://www.ncbi.nlm.nih.gov/pubmed/30151436
http://dx.doi.org/10.5334/jbr-btr.906
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author Kasuya, Shusuke
Suzuki, Masayo
Inaoka, Tsutomu
Odashima, Masayuki
Nakatsuka, Tomoya
Ishikawa, Rumiko
Tokuyama, Wataru
Terada, Hitoshi
author_facet Kasuya, Shusuke
Suzuki, Masayo
Inaoka, Tsutomu
Odashima, Masayuki
Nakatsuka, Tomoya
Ishikawa, Rumiko
Tokuyama, Wataru
Terada, Hitoshi
author_sort Kasuya, Shusuke
collection PubMed
description Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A. Approximately 50% of patients with AFD may have cardiac involvement. Gadolinium-enhanced cardiac magnetic resonance (CMR) is useful for the diagnosis of cardiac involvement of AFD by recognizing typical late gadolinium enhancement (LGE) patterns. We report a 48-year-old man with cardiac involvement in classical AFD, showing atypical distribution of the LGE at the mid-lateral wall of left ventricle, predominantly apical segments without basal involvement on gadolinium-enhanced CMR.
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spelling pubmed-61006572018-08-27 Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease Kasuya, Shusuke Suzuki, Masayo Inaoka, Tsutomu Odashima, Masayuki Nakatsuka, Tomoya Ishikawa, Rumiko Tokuyama, Wataru Terada, Hitoshi J Belg Soc Radiol Case Report Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A. Approximately 50% of patients with AFD may have cardiac involvement. Gadolinium-enhanced cardiac magnetic resonance (CMR) is useful for the diagnosis of cardiac involvement of AFD by recognizing typical late gadolinium enhancement (LGE) patterns. We report a 48-year-old man with cardiac involvement in classical AFD, showing atypical distribution of the LGE at the mid-lateral wall of left ventricle, predominantly apical segments without basal involvement on gadolinium-enhanced CMR. Ubiquity Press 2016-01-29 /pmc/articles/PMC6100657/ /pubmed/30151436 http://dx.doi.org/10.5334/jbr-btr.906 Text en Copyright: © 2016 The Author(s) http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License (CC-BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See http://creativecommons.org/licenses/by/4.0/.
spellingShingle Case Report
Kasuya, Shusuke
Suzuki, Masayo
Inaoka, Tsutomu
Odashima, Masayuki
Nakatsuka, Tomoya
Ishikawa, Rumiko
Tokuyama, Wataru
Terada, Hitoshi
Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease
title Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease
title_full Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease
title_fullStr Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease
title_full_unstemmed Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease
title_short Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease
title_sort atypical distribution of late gadolinium enhancement of the left ventricle on cardiac magnetic resonance in classical anderson-fabry disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6100657/
https://www.ncbi.nlm.nih.gov/pubmed/30151436
http://dx.doi.org/10.5334/jbr-btr.906
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