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

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Kasuya, Shusuke ; Suzuki, Masayo ; Inaoka, Tsutomu ; Odashima, Masayuki ; Nakatsuka, Tomoya ; Ishikawa, Rumiko ; Tokuyama, Wataru ; Terada, Hitoshi (2016)
  • Publisher: Ubiquity Press
  • Journal: Journal of the Belgian Society of Radiology, volume 100, issue 1 (issn: 2514-8281, eissn: 2514-8281)
  • Related identifiers: doi: 10.5334/jbr-btr.906, pmc: PMC6100657
  • Subject: Anderson-Fabry disease (AFD); cardiac magnetic resonance (CMR); late gadolinium enhancement (LGE); hypertrophic cardiomyopathy | Anderson-Fabry disease (AFD) | Case Report | hypertrophic cardiomyopathy | cardiac magnetic resonance (CMR) | R895-920 | late gadolinium enhancement (LGE) | Medical physics. Medical radiology. Nuclear medicine
    mesheuropmc: cardiovascular system | cardiovascular diseases

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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