Fig. 4

Asymmetric septal hypertrophy in Anderson-Fabry Disease (AFD). Short-axis (a-c) and 4-chamber (d) cine steady state free precession and short-axis (e-g) and 4-chamber (h) late gadolinium enhancement images in a patient with Anderson-Fabry Disease on enzyme replacement therapy. The 4 chamber view revealed a reverse septal curvature subtype of asymmetric septal hypertrophy (d). There is a non-ischemic pattern of scar with high intensity hinge point scar (more so in the anteroseptum than the inferoseptum - dashed arrows on images e-f. There is further intermediate intensity patchy midwall and subendocardial scar not typical of ischemic heart disease (preserved muscle bulk and patchy sparing of subendocardium and trabeculae) distributed with an apical predominance (white arrowheads on images G-H). Scar was distributed with an increasing percentage from base to apex (Basal LV scar 5 %; Mid LV scar 22 %; Apical LV scar 47 %)