Anderson Fabry disease (AFD) is a rare intracellular lipid disorder which can lead to myocardial fibrosis.
We propose that besides late gadolinium enhancement,
T1-mapping might allow an early detection of cardiac involvement.
Furthermore MRI findings may correlate with serum biomarkers indicating myocardial damage.
Methods and Materials
46 patients (20 LGE positive [group1],
26 LGE negative [group2]) with manifest AFD and 28 healthy subjects [group3] were examined.
T1-mapping was performed with a modified Look-Locker IR-sequence (MOLLI) at 3T after i.v.
We performed ROI-based analysis (Fig.
results were derived from 8 slices (6 ROIs/slice).
Absolute T1 values were compared in between those three groups.
Futhermore,T1-valueswere correlated with the biochemical markers NT-proBNP,
Troponin T and lyso-Gb3.
Final results show visible changes in the T1-maps wherever fibrosis in LGE imaging was observed (Fig.
2 + 3).
Absolute T1-values for several ROIs were significantly lower in group 1 compared to group 2 and 3 (inferolateral wall: p<0.01; others: p<0.05),
even in areas where no fibrosis was detected by LGE imaging (anterior and septal wall).
NT-proBNP and Troponin T levels were significantly higher in group 1 compared to group 2 (p<0,01).
Lyso-Gb3 serum levels were elevated in 100%[group 1]and 86%[group 2]of patients.
Our data suggest that T1-mapping is a sensitive tool to detect early replacement fibrosis in AFD,
and might be more sensitive than visual LGE analysis in the detection of regional myocardial involvement.
Elevated levels of Troponin T and NT-proBNP indicate myocardial involvement and correlate with findings in cardiac MRI.