Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease
- Publisher: Ubiquity Press
Journal of the Belgian Society of Radiology,
(issn: 2514-8281, eissn: 2514-8281)
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.