Key result
Native T1 mapping effectively differentiated Fabry disease from hypertrophic cardiomyopathy, yielding a sensitivity of 93.3% and specificity of 80.0% for septal T1 values.
Why the study?
There was no report for the systematic evaluation of native T1 values in Fabry disease in Japan.
Does native T1 mapping using 3-T CMR effectively differentiate Fabry disease from hypertrophic cardiomyopathy in Japanese patients?
Observational (n=45)
Does native T1 mapping using 3-T CMR effectively differentiate Fabry disease from hypertrophic cardiomyopathy in Japanese patients?
Effect estimate: Sensitivity 93.3% and specificity 80.0% (septal T1)
Native T1 mapping using 3-T CMR can effectively differentiate Fabry disease from hypertrophic cardiomyopathy and reflects myocardial glycosphingolipid accumulation.
Native T1 mapping may aid Fabry detection among Japanese LVH patients; leaves open validation in larger prospective cohorts.
T1 mapping in cardiac magnetic resonance imaging enables us to distinguish various myocardial diseases showing left ventricular hypertrophy. Fabry disease is a lysosomal storage disorder causing the accumulation of glycosphingolipids into various organs, including the heart, which can be detected by native T1 values in T1 mapping. However, there is no report for the systematic evaluation of native T1 values in Fabry disease in Japan. We analyzed native T1 values of 30 Fabry disease patients (14 males and 16 females) obtained by 3-T cardiac magnetic resonance imaging. Averaged T1 values were significantly lower in male patients (septal T1: 1149.5 ± 63.3 ms; total T1: 1145.1 ± 59.5 ms) than in female patients (septal T1: 1210.5 ± 45.5 ms; total T1: 1198.8 ± 51.8 ms) (p < 0.01). We compared the native T1 values of Fabry disease patients with those obtained from 15 hypertrophic cardiomyopathy patients (9 males and 6 females). Native T1 values effectively differentiate Fabry disease from hypertrophic cardiomyopathy (septal T1: sensitivity 93.3% and specificity 80.0%; total T1: sensitivity 86.7% and specificity 73.3%). In addition, native T1 values had a significant negative correlation with the left ventricular mass index in male patients at the pre-hypertrophic stage (p < 0.05). In male and female patients without late-gadolinium enhancement, native T1 values also had a significant negative correlation with the left ventricular mass index (p < 0.05). These results suggest that native T1 values can be used to discriminate Fabry disease from hypertrophic cardiomyopathy and can reflect the accumulation of glycosphingolipids in cardiomyocytes.
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Anan et al. (2022) conducted an observational in Fabry disease and hypertrophic cardiomyopathy (n=45). Native T1 mapping vs. Hypertrophic cardiomyopathy was evaluated on Differentiation of Fabry disease from hypertrophic cardiomyopathy (Sensitivity 93.3% and specificity 80.0% (septal T1)). Native T1 mapping effectively differentiated Fabry disease from hypertrophic cardiomyopathy, yielding a sensitivity of 93.3% and specificity of 80.0% for septal T1 values.
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