Abstract
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.
Highlights
Fabry disease is a rare X-linked condition that results in storage of sphingolipids in multiple organs including the heart
left ventricular hypertrophy (LVH) was defined by mass index (LVMi) and a wall thickness ≥12 mm
An abnormal ECG was defined by a prolonged PR interval (>200 ms), prolonged QRS duration (>120 ms), the presence of T-wave inversions, and/or LVH by Sokolow criteria
Summary
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
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
