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Published on: August 8, 2022
Differentiation between Fabry disease and hypertrophic cardiomyopathy with cardiac T1 mapping
E Deborde1, B Dubourg2, S Bejar3
1Department of Radiology, University Hospital of Rouen, 76031 Rouen, France; Department of Radiology, University Hospital of Strasbourg, 67098 Strasbourg, France.
Insights
Non-contrast T1 mapping in cardiovascular magnetic resonance (CMR) shows significantly lower native T1 values in Fabry disease (FD) patients compared to hypertrophic cardiomyopathy (HCM) and healthy controls, aiding in diagnosis.
Area of Science:
- Cardiovascular Magnetic Resonance (CMR)
- Cardiac Imaging
- Myocardial Tissue Characterization
Background:
- Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) can present with left ventricular hypertrophy (LVH), complicating differential diagnosis.
- Non-contrast cardiovascular magnetic resonance (CMR) T1 mapping offers a method for myocardial tissue characterization.
Purpose of the Study:
- To assess the utility of non-contrast T1 mapping in differentiating FD from HCM and healthy subjects.
- To determine if T1 values can distinguish between these cardiac conditions.
Main Methods:
- Seventeen FD patients, 36 HCM patients, and 70 healthy controls underwent cardiac T1 mapping using a modified Look-Locker inversion (MOLLI®) sequence.
- Native T1 values were measured in septal and global mid-ventricular segments.
- Statistical analyses included Mann-Whitney tests and receiver operating characteristic (ROC) curve analysis.
Main Results:
- Septal and global native T1 values were significantly lower in FD patients compared to both HCM patients (P<0.001) and healthy controls (P<0.001).
- A septal native T1 cutoff of 940ms distinguished FD from HCM with 88% sensitivity and 92% specificity.
- T1 values were abnormal in 50% of FD patients without LVH.
Conclusions:
- Native T1 values are significantly reduced in patients with FD.
- Non-contrast T1 mapping is a valuable tool for differentiating FD from HCM and healthy individuals.
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.
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