Key result
Expanding LGE linked to ~6 ms higher regional T2 in Anderson-Fabry disease.
Why the study?
Myocardial inflammation is frequent in Anderson-Fabry disease patients, but its role across disease stages and association with cardiac damage progression remains unclear.
Does myocardial inflammation assessed by CMR T2 mapping associate with LGE scar expansion in patients with Anderson-Fabry disease?
Observational (n=41)
No
Does myocardial inflammation assessed by CMR T2 mapping associate with LGE scar expansion in patients with Anderson-Fabry disease?
Absolute Event Rate: 62% vs 56%
p-value: p=0.03
In patients with Anderson-Fabry disease, elevated myocardial T2 mapping values are associated with the progression of late gadolinium enhancement, suggesting that inflammation plays a role in scar expansion.
T2 elevation in LGE+ regions may indicate inflammation in AFD; hypothesis-generating for staging and requires prospective validation before clinical use.
BACKGROUND: Anderson-Fabry Disease (AFD) is a lysosomal storage disorder characterized by glycosphingolipid accumulation, leading to multi-organ failure. Cardiac involvement, marked by left ventricular (LV) hypertrophy and fibrosis, is a major cause of mortality. Myocardial inflammation is a frequent finding in these patients, but its role across disease stages remains unclear. This pilot study investigates the association between myocardial inflammation, assessed via cardiac magnetic resonance (CMR) T2 mapping (T2m), and cardiac damage progression, measured by late gadolinium enhancement (LGE) expansion. METHODS: This retrospective, single-center study, includes 131 paired CMR scans (1.5T, Philips Ingenia) collected between June 2018 and December 2023 from 41 AFD patients. Cine imaging, T2m, and LGE sequences were acquired in all. Scans were categorised based on quantitative LGE changes into three groups: LGE- (no LGE), LGE= (stable LGE compared to the previous scan), and LGE+ (new/increased LGE compared to the previous scan). T2m was measured globally, in the basal slice (B T2m), infero-lateral basal segment (ILB T2m), and in LGE-matching regions. RESULTS: The sample consisted of 61% women (average of 3±1 scans/patient); at baseline, mean age was 44±18 years, the LV mass was 58±24g/m2. The average time between CMR scans was 14±4 months. In LGE-matching regions, T2m was significantly higher in LGE+ scans compared to LGE= (62ms vs 56ms, p=0.03), and ILB T2m was higher in LGE+ than LGE- (56ms vs 50ms, p=0.007). No other significant differences were observed. CONCLUSION: In AFD patients, we demonstrated an association between myocardial inflammation and LGE expansion, with T2m potentially serving as an early marker of disease activity. Region-specific T2m measurement is particularly useful in this disease Further research is needed to understand whether T2m changes precedes structural myocardial damage and assess T2m's prognostic value in AFD.
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Torlasco et al. (2026) conducted an observational in Anderson-Fabry disease (n=41). New or increased late gadolinium enhancement (LGE+) vs. Stable LGE (LGE=) or no LGE (LGE-) was evaluated on Myocardial inflammation (T2 mapping in LGE-matching regions) (p=0.03). In Anderson-Fabry disease, T2 mapping in LGE-matching regions was higher in scans with new or increased LGE compared to stable LGE (62ms vs 56ms, P=0.03).
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