Abstract
Introduction: Cardiac magnetic resonance(CMR), especially T1 mapping, has an important role in the diagnosis of Fabry disease, but the differential value in myocardial diseases with hypertrophic phenotype needs further study. More importantly, there has been no systematic study of the difference between different CMR techniques indistinguishing Fabry cardiomyopathy from hypertrophic cardiomyopathy (HCM). Hypothesis: Myomapping technology can be a parameter in distinguishing Fabry cardiomyopathy from HCM. Methods: We continuously included patients with clinically diagnosed hypertrophic cardiomyopathy, completed systematic magnetic resonance imaging, and used genetic testing as the diagnostic criteria. We reported the prevalence rate in the population of HCM, and deeply conducted comparative studies on the differences in magnetic resonance characteristics between Fabry disease and HCM. We also analyzed the diagnostic value of magnetic resonance imaging in distinguishing Fabry cardiomyopathy from HCM. Results: We continuously included patients diagnosed with hypertrophic cardiomyopathy at West China Hospital from 2012 to 2022 and completed systematic magnetic resonance imaging examinations. We reported the prevalence of Fabry cardiomyopathy in the population using genetic testing as the diagnostic criteria, and deeply conducted comparative studies on the differences in CMR features between Fabry disease and HCM. We also analyzed the diagnostic value of CMR in distinguishing Fabry cardiomyopathy from HCM. Conclusions: We found a prevalence of 13 (2.6%) cases of Fabry disease in the hypertrophic cardiomyopathy cohort (494 cases). Our further analysis found that there were no significant differences in the degree of hypertrophy, overall cardiac function and size of Fabry patients compared with patients with HCM, as well as strain analysis. NativeT1 was significantly reduced in Fabry disease, and segmental analysis found that the basal and middle ventricular septum and anterior wall were the most obvious. ECV differed significantly between the two groups. There was no difference in T2 between the two groups. ROC analysis showed that T1 had the most discriminating value
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