Shanat Baig

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Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by mutations in a-galactosidase A. Sphingolipid accumulates in organs including the heart, causing left ventricular hypertrophy (LVH) and myocardial fibrosis. Cardiac involvement is the leading cause of death in FD. Cardiovascular magnetic resonance (CMR) provides insights with late(More)
Aims Left ventricular papillary muscles (LVPM) can appear disproportionately hypertrophied, particularly in Fabry disease (FD) where storage appears detectable by cardiovascular magnetic resonance (CMR) T1 mapping. The aim of the study was to measure LVPM mass in heart diseases with left ventricular hypertrophy (LVH) and to gain insight into the mechanisms(More)
Fabry disease (FD) is an X-linked lysosomal storage disorder caused by deficiency of α-galactosidase A enzyme. Cardiovascular (CV) disease is a common cause of mortality in FD, in particular as a result of heart failure and arrhythmia, with a significant proportion of events categorized as sudden. There are no clear models for risk prediction in FD. This(More)
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