Focal reduction in cardiac ¹²³i-metaiodobenzylguanidine uptake in patients with anderson-fabry disease

Background: It remains to be elucidated whether cardiac sympathetic nervous activity is impaired in patients with Anderson-Fabry disease (AFD). Methods and Results: We performed ¹²³I-meta-iodobenzylguanidine (MIBG) scintigraphy and gadolinium-enhanced cardiovascular magnetic resonance (CMR) in 5 AFD patients. MIBG uptake in the inferolateral wall, where wall thinning and delayed enhancement were noted on CMR, was significantly lower compared with the anteroseptal wall. The localized reduction in MIBG uptake was also noted in 2 patients with no obvious abnormal findings on CMR. Conclusions: Cardiac sympathetic nervous activity is impaired in AFD before development of structural myocardial abnormalities.