Anderson Fabry disease (AFD) is a rare lipid disorder which leads to LV-hypertrophy and fibrosis in end-stage disease. Besides late gadolinium enhancement (LGE), T1-mapping was suggested to be a promising diagnostic tool in AFD allowing an early detection of cardiac involvement [1].

23 patients with manifest AFD (10 LGE positive [group 1]; 13 LGE negative [group 2] 3 Tesla Scanner (MAGNETOM TIM Trio, Siemens AG Sector HealthCare, Erlangen, Germany) spatial resolution: 2.2 x 1.4 x 8.0 mm3 ; temporal resolution: 14 slices / 120 s Modified Look-Locker Inversion Recovery (MOLLI) sequence [2,3], VB17 WIP prep, Siemens AG, Sector HealthCare, Germany) free breathing, ECG-triggering acquisition 20-25 minutes after body-weight adapted i.v. application of gadobenate dimeglumine acquisition of 8 images per slice, each at a different moment in time...

Results were derived from SAX as well as septal an inferolateral wall. The T1-maps showed visible changeswherever fibrosis in the LGE imaging was observed. Absolute T1 values for all 3 regions were significantly lower in group 1 compared to group 2 (inferolateral wall: p<0.01; SAX and septal wall: p<0.05), even in areas where no fibrosis was detected by LGE imaging (septal wall). Compared to recently published reference values [5], higher overallabsolute T1 values were detected in both of our groups (491±48 ms vs. 558±77 ms).

Our preliminary data ina yet small patient sample suggest that T1-mapping is a sensitive tool to detect early replacement fibrosis in AFD, and might be more sensitive than the standard visual LGE analysis in the detection of regionalmyocardial involvement.

[1] Daniel M. Sado et al. (2013) Circ Cardiovasc Imaging 6:392-398 [2] Messroghli et al. (2004) Magn Reson Med 52:141–146 [3] Messroghli et al. (2007) J Magn Reson Imag 26:1081–1086 [4] Chefd’hotel et al. (2002) Proc ISBI 753-756 [5] Knobelsdorff-Brenkenhoff et al. (2013) J Cardiovasc Magn Reson Med 18;15(1):53