Fig. 7

Demonstration of scar at fibrous-muscular junctions in Anderson-Fabry disease (AFD). Late gadolinium enhancement images from 2 AFD patients in our cohort. On the 4-chamber stack and cardiac short-axis late gadolinium enhancement images from a patient on ERT (a-d), high intensity midwall scar is seen to extend from the aortic annulus into the basal anteroseptal segment (white arrowheads with black border on image A) and spiral downwards into the basal inferoseptal segment (b-c). Cardiac short-axis image (d) and more inferior slices from the 4-chamber stack (b-c) demonstrate high intensity scar in the posteromedial papillary muscle group at the junction between the heads and the chordae tendinae (black arrowheads with white border). On the 4-chamber orientation stack images (e-f) from another patient on enzyme replacement therapy (ERT), there is a focus of high intensity midwall scar extending from the mitral annulus into the basal inferoseptal segment (white arrowheads with black border) in this. The 4-chamber orientation stack images (e-f) demonstrate high intensity midwall scar extending from the mitral annulus into the basal inferoseptal segment (white arrowheads with black border). 3-chamber stack images from the same patient (g-h) demonstrate high intensity midwall scar extending from the mitral annulus into the basal inferolateral segment (white arrowheads with black border)