Differentiation between Fabry disease and hypertrophic cardiomyopathy with cardiac T1 mapping

Abstract Purpose To evaluate the potential of non-contrast myocardial T1 mapping on cardiovascular magnetic resonance examination (CMR) in differentiating patients with Fabry disease (FD) from those with hypertrophic cardiomyopathy (HCM) and healthy control subjects. Materials and methods Seventeen patients with FD (8 men, 9 women; mean age, 48  ± 18 [SD] years; [range: 19–73 years]; 53% with left ventricular hypertrophy [LVH]) were matched with 36 patients with hypertrophic cardiomyopathy (HCM) (22 men, 14 women; mean age, 57 ± 16 [SD] years; [range: 22–85 years]) and 70 healthy control subjects (34 men, 36 women; mean age, 38 ± 15 [SD] years; [range: 18–65 years]). Cardiac T1 mapping was performed using the modified Look-Locker inversion (MOLLI®) sequence on a 1.5-T magnet. T1 values were calculated, on midventricular section, for septal left ventricular segments (S8–S9) and all mid-ventricular ones (global T1 values; S7–S12). Statistical analysis included unpaired Mann-Whitney test, receiver operating characteristic curve and likelihood ratios. Results Septal native T1 values were significantly decreased in patients with FD (889 ± 61 [SD] ms; range: 784–980 ms) compared to those with HCM (995 ± 48 [SD] ms; range: 935–1125 ms) (P  Conclusion Native T1 values are significantly lower in patients with FD by comparison with those with HCM and healthy volunteers.