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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Detalles Bibliográficos
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
Descripción
Sumario: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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