Differentiation between Fabry disease and hypertrophic cardiomyopathy with cardiac T1 mapping - 26/01/20
, B. Dubourg a, c, d, S. Bejar a, A.-C. Brehin e, S. Normant a, P. Michelin a, J.-N. Dacher a, c, d
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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: 784–980ms) compared to those with HCM (995±48 [SD] ms; range: 935–1125ms) (P<0.001) and versus healthy controls (965±29 [SD] ms; range: 910–1028ms) (P<0.001). Global native T1 values were also significantly decreased in patients with FD (891±49 [SD] ms; range 794–970ms) compared to those with HCM (995±34 [SD] ms; range: 952–1086ms) (P<0.001) and versus healthy controls (966±27 [SD] ms; range: 920–1042ms) (P<0.001). A septal left ventricular native T1 cutoff value of 940ms could distinguish FD from HCM with 88% sensitivity (95% CI: 73–100%) and 92% specificity (95% CI: 83–100%). Positive likelihood ratio was 11, negative likelihood ratio was 0.12. Compared to controls, the same threshold could distinguish FD with 88% sensitivity (95% CI: 73–100%) and 86% specificity (95% CI: 78–94%). Positive likelihood ratio was 6.3, negative likelihood ratio was 0.14. T1 value was abnormal in 4 of 8 (50%) of FD patients who did not have LVH.
Conclusion |
Native T1 values are significantly lower in patients with FD by comparison with those with HCM and healthy volunteers.
Le texte complet de cet article est disponible en PDF.Keywords : Cardiac magnetic resonance imaging (MRI), Fabry disease, Left ventricular hypertrophy, Hypertrophic cardiomyopathy
Abbreviations : FD, LVH, ERT, CMR, LVM, LGE, LV, MRI, HCM, CI, SD
Plan
Vol 101 - N° 2
P. 59-67 - février 2020 Retour au numéro
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