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. 2018 Aug 22;7(17):e009052. doi: 10.1161/JAHA.118.009052

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© 2018 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

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Group 1 patient. A 22‐year‐old woman with prehypertrophic Fabry disease cardiomyopathy (maximal wall thickness [MWT]: 8 mm) showing normal myocardium in a T2‐weighted short τ inversion recovery image (A) and a late gadolinium–enhanced image (C). Analysis of T1 mapping (B) showed a reduced septal T1 value (923±30 ms, n.v. 960–990 ms), reflecting initial tissue lipid accumulation. D, At histology (hematoxylin and eosin, ×200), cardiomyocytes are intermittently enlarged because of cytoplasm vacuoles that, at electron microscopy, consist of myelin bodies (insert) and are focally (arrows) surrounded by CD3⁺ infiltrates (E) with cell necrosis. F, Correlation between MWT and CD3 count (P=ns) in all group 1 patients (n=13).