ECG, LVH and T1 changes in Fabry disease - implications for screening and understanding of the disease model
نویسندگان
چکیده
Background Fabry disease is a rare X-linked condition that results in storage of sphingolipids in multiple organs including the heart. The cardiac phenotype consists mainly of conduction abnormalities, left ventricular hypertrophy (LVH) and disease progression (fibrosis, arrhythmias and heart failure). CMR LGE classically shows basal inferolateral LGE. Native T1 mapping has recently shown a low T1 likely to represent myocycte storage of fat, even when no LVH is present. We sought to understand the relationship of storage to LVH and ECG abnormalities.
منابع مشابه
Reproducibility of native myocardial T1 mapping in the assessment of Fabry disease and its role in early detection of cardiac involvement by cardiovascular magnetic resonance
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