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Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease
Author(s) -
Shusuke Kasuya,
Masayo Suzuki,
Tsutomu Inaoka,
Masayuki Odashima,
Tomoya Nakatsuka,
Rumiko Ishikawa,
Wataru Tokuyama,
Hitoshi Terada
Publication year - 2016
Publication title -
journal of the belgian society of radiology
Language(s) - English
Resource type - Journals
ISSN - 1780-2393
DOI - 10.5334/jbr-btr.906
Subject(s) - gadolinium , ventricle , fabry disease , magnetic resonance imaging , medicine , cardiac magnetic resonance , basal (medicine) , cardiology , radiology , disease , chemistry , organic chemistry , insulin
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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