Article
The myocardial phenotype of Fabry disease pre-hypertrophy and pre-detectable storage.
European heart journal. Cardiovascular Imaging - 22 Jun 2021
Augusto João B, Johner Nicolas, Shah Dipen, Nordin Sabrina, Knott Kristopher D, Rosmini Stefania, Lau Clement, Alfarih Mashael, Hughes Rebecca, Seraphim Andreas, Vijapurapu Ravi, Bhuva Anish, Lin Linda, Ojrzyńska Natalia, Geberhiwot Tarekegn, Captur Gabriella, Ramaswami Uma, Steeds Richard P, Kozor Rebecca, Hughes Derralynn, Moon James C, Namdar Mehdi
Abstract excerpt
AIMS: 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. METHODS AND RESULTS: FD patients and age-, sex-,...
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