RT Journal Article SR Electronic T1 Global longitudinal strain, myocardial storage and hypertrophy in Fabry disease JF Heart JO Heart FD BMJ Publishing Group Ltd and British Cardiovascular Society SP 470 OP 476 DO 10.1136/heartjnl-2018-313699 VO 105 IS 6 A1 Vijapurapu, Ravi A1 Nordin, Sabrina A1 Baig, Shanat A1 Liu, Boyang A1 Rosmini, Stefania A1 Augusto, Joao A1 Tchan, Michel A1 Hughes, Derralynn A A1 Geberhiwot, Tarekegn A1 Moon, James C A1 Steeds, Richard Paul A1 Kozor, Rebecca YR 2019 UL http://heart.bmj.com/content/105/6/470.abstract AB Introduction Detecting early cardiac involvement in Fabry disease (FD) is important because therapy may alter disease progression. Cardiovascular magnetic resonance (CMR) can detect T1 lowering, representing myocardial sphingolipid storage. In many diseases, early mechanical dysfunction may be detected by abnormal global longitudinal strain (GLS). We explored the relationship of early mechanical dysfunction and sphingolipid deposition in FD.Methods An observational study of 221 FD and 77 healthy volunteers (HVs) who underwent CMR (LV volumes, mass, native T1, GLS, late gadolinium enhancement), ECG and blood biomarkers, as part of the prospective multicentre Fabry400 study.Results All FD had normal LV ejection fraction (EF 73%±8%). Mean indexed LV mass (LVMi) was 89±39 g/m2 in FD and 55.6±10 g/m2 in HV. 102 (46%) FD participants had left ventricular hypertrophy (LVH). There was a negative correlation between GLS and native T1 in FD patients (r=−0.515, p<0.001). In FD patients without LVH (early disease), as native T1 reduced there was impairment in GLS (r=−0.285, p<0.002). In the total FD cohort, ECG abnormalities were associated with a significant impairment in GLS compared with those without ECG abnormalities (abnormal: −16.7±3.5 vs normal: −20.2±2.4, p<0.001).Conclusions GLS in FD correlates with an increase in LVMi, storage and the presence of ECG abnormalities. In LVH-negative FD (early disease), impairment in GLS is associated with a reduction in native T1, suggesting that mechanical dysfunction occurs before evidence of sphingolipid deposition (low T1).Trial registration number NCT03199001; Results.