Myocardial T1 dispersion in fabry disease: comprehensive evaluation of a novel measurement of cardiovascular disease severity

Abstract

Abstract Background The cardiovascular manifestations of Fabry disease are common and represent the leading cause of death. Myocardial T1 dispersion has recently emerged as a robust predictor of adverse cardiac outcome in Fabry disease but the relationship between T1 dispersion and other cardiovascular manifestations has not been studied. Objective To evaluate the relationship between myocardial T1 dispersion and other cardiovascular manifestations of Fabry disease, and identify its determinants. Methods Prospective longitudinal cohort study including 181 participants with Fabry disease and 30 controls undergoing clinical cardiovascular magnetic resonance. T1 dispersion was assessed in relation to other structural and functional cardiovascular biomarkers and examined in Pre-hypertrophic, Hypertrophic and Fibrotic phenotypic sub-groups. Determinates of T1 dispersion were investigated using multivariable linear regression. Results T1 dispersion was similar in the Control, Pre-hypertrophic and Hypertrophic sub-groups, but was significantly elevated in the Fibrotic sub-group. Independent determinates of T1 dispersion were age, body surface area-indexed left ventricular mass, indexed left ventricular end diastolic volume, global longitudinal strain, native myocardial T1, non-ischaemic late gadolinium enhancement, and extracellular volume fraction (R2 0.414). T1 dispersion was consistently higher in females than males. Conclusions Myocardial T1 dispersion appears sensitive to both the T1 shortening effect of globotriaosylceramide accumulation and T1 lengthening effect of fibrosis, and accordingly increases as Fabry disease progresses. In conjunction with recent data showing it to be a robust predictor of adverse cardiac outcome, myocardial T1 dispersion is advocated as a key cardiovascular biomarker in Fabry disease.Figure 1Figure 2