Atypical distribution of late gadolinium enhancement of the left ventricle on cardiac magnetic resonance in classical Anderson-Fabry disease

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Abstract

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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Kasuya, S., Suzuki, M., Inaoka, T., Odashima, M., Nakatsuka, T., Ishikawa, R., … Terada, H. (2016). Atypical distribution of late gadolinium enhancement of the left ventricle on cardiac magnetic resonance in classical Anderson-Fabry disease. JBR-BTR, 100(1). https://doi.org/10.5334/jbr-btr.906

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