Value of Left Ventricular Feature Tracking Strain Analysis for Detection of Early Cardiac Involvement in Fabry Disease (FD)

J Clin Med 2021 Aug 22;10(16):3734. doi: 10.3390/jcm10163734.

PMID: 34442030

Fritz Christian Roller, Alexander Brose, Martin Richter

Highlights: According to the findings of this study, strain abnormalities occur early beside native T1 reductions in cardiac FD involvement.

Abstract

Purpose: The detection of cardiac involvement in Fabry disease (FD) is critical for effective treatment. Native T1 mapping, in particular, showed a lot of promise for detecting early cardiac symptoms. In FD patients, echocardiographic tests revealed strain anomalies, but data on MRI feature tracking strain analysis (FT-SA) is scarce. As a result, the goal of our research was to compare the potential of FT-SA to native T1 and the FD biomarker Globotriaosylsphingosine (LysoGb3).

Methods: A total of 28 FD patients (18 female; 47.8 years ± 17.4 SD) and 28 control participants (18 female; 46.6 years ± 18.2 SD) underwent 1.5 Tesla cardiac MRI. The left ventricular FT-SA and global native T1 timings were measured. The results were compared to Lyso-Gb3 serum concentrations.

Results: In FD patients, native T1 times, global longitudinal (GLS), and global radial strain (GRS) were all considerably lower (p < 0.0064, p = 0.0009, and p = 0.0184, respectively). Furthermore, Lyso-Gb3 positive FD patients had significantly decreased native T1 timings and GLS (p < 0.005 and p = 0.03). GLS, native T1 times showed significant moderate correlations to LysoGb3 (p = 0.002 and p < 0.001). Furthermore, when LysoGb3 is high, GLS and native T1 times decrease, and this decrease increases with the presence of left ventricular hypertrophy (LVH) or late gadolinium enhancement (LGE).

Conclusions: Native T1 times and strain values differ dramatically between FD patients and controls, with promising correlations to the FD biomarker LysoGb3. We conclude that, in addition to native T1 decreases, strain anomalies occur early in cardiac FD involvement. To confirm our findings, large-scale experiments are required.

Keywords: Fabry, Cardiac MRI, Feature tracking strain, Native T1 mapping, rare diseases