Role of myocardial inflammation in the progression of Fabry cardiomyopathy: a multicentre cardiac magnetic resonance study

Abstract Background Inflammation plays a central role in the pathophysiology of Fabry disease (FD) and can be assessed non-invasively using cardiac magnetic resonance (CMR) with T2 mapping. However, its significance in the progression of FD cardiomyopathy remains unclear. Objective To evaluate the relationship between CMR T2 values and the progression of tissue alterations, specifically late gadolinium enhancement (LGE), in patients with FD. Methods A total of 105 pairs of CMR examinations from FD patients were retrospectively analysed and categorised into four groups based on LGE status: A) absence of LGE on both examinations; B) absence of LGE on the first CMR, with new LGE appearance at follow-up; C) presence of LGE on the first CMR that remained unchanged at follow-up; D) presence of LGE on the first CMR that increased at follow-up. T2 was measured in the basal inferolateral segment and, for LGE-positive patients, in the LGE area, with results reported as Z-scores. To increase the sample size, the analysis was repeated including all available pairs of CMR for each patient (185 pairs in total). The association between T2 Z-score and worsening of LGE, as well as the correlation between T2 Z-score and LGE extension, was also tested. Results Among LGE-negative patients at baseline (Groups A and B), the T2 Z-score was significantly higher in Group B compared to Group A, both at the first (A: 0.00 [-0.67; 1] vs B: 1.67 [0.33; 2.00], p=0.003) and second scans (A: 0.33 [-0.33; 1.17] vs B: 3.00 [2.67; 5.00], p