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Scientific article
Open access
English

The myocardial phenotype of Fabry disease pre-hypertrophy and pre-detectable storage

Publication date2020
Abstract

Cardiac involvement in Fabry disease (FD) occurs prior to left ventricular hypertrophy (LVH) and is characterized by low myocardial native T1 with sphingolipid storage reflected by cardiovascular magnetic resonance (CMR) and electrocardiogram (ECG) changes. We hypothesize that a pre-storage myocardial phenotype might occur even earlier, prior to T1 lowering.

Keywords
  • Fabry disease
  • Cardiovascular magnetic resonance
  • Electrocardiogram
  • Global longitudinal strain
  • Microvascular dysfunction
Citation (ISO format)
AUGUSTO, João B et al. The myocardial phenotype of Fabry disease pre-hypertrophy and pre-detectable storage. In: European Heart Journal Cardiovascular Imaging, 2020. doi: 10.1093/ehjci/jeaa101
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Identifiers
ISSN of the journal2047-2404
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Technical informations

Creation10/12/2020 11:01:00 AM
First validation10/12/2020 11:01:00 AM
Update time03/16/2023 12:01:10 AM
Status update03/16/2023 12:01:08 AM
Last indexation05/06/2024 6:38:30 AM
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