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

Journal of the Belgian Society of Radiology
Shusuke KasuyaHitoshi Terada

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.

References

Jun 8, 2006·Journal of Cardiovascular Magnetic Resonance : Official Journal of the Society for Cardiovascular Magnetic Resonance·James C MoonDudley J Pennell
Feb 22, 2007·AJR. American Journal of Roentgenology·Massimo ImbriacoMarco Salvatore
Feb 24, 2009·AJR. American Journal of Roentgenology·Francesco De CobelliAlessandro Del Maschio
Sep 30, 2009·Circulation·Christopher A GangeMartin S Maron
Aug 12, 2014·Journal of the Neurological Sciences·Rima El-AbassiJohn D England

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