Does native T1 mapping by CMR accurately grade histologically proven myocardial glycosphingolipid accumulation in patients with Fabry disease?
Native T1 mapping by CMR is a reliable, noninvasive surrogate biomarker that strongly correlates with histological glycosphingolipid accumulation in Fabry disease cardiomyopathy.
Native T1 values are a valid biomarker of glycosphingolipid accumulation in myocardial tissue and correlate with accumulation degree. Midventricular interventricular septum is the preferred site for nT1 measurement, showing the best agreement with glycosphingolipid accumulation at endomyocardial biopsy. The use of native T1 in the setting of suspected cardiac Fabry disease will help to stratify disease severity, without the intrinsic risk of endomyocardial biopsy.
Galea et al. (Wed,) studied this question.