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Article

Myocardial Storage, Inflammation, and Cardiac Phenotype in Fabry Disease After One Year of Enzyme Replacement Therapy.

Circulation. Cardiovascular imaging - 1 Dec 2019

Nordin Sabrina, Kozor Rebecca, Vijapurapu Ravi, Augusto João B, Knott Kristopher D, Captur Gabriella, Treibel Thomas A, Ramaswami Uma, Tchan Michel, Geberhiwot Tarekegn, Steeds Richard P, Hughes Derralynn A, Moon James C

Abstract excerpt

BACKGROUND: Cardiac response to enzyme replacement therapy (ERT) in Fabry disease is typically assessed by measuring left ventricular mass index using echocardiography or cardiovascular magnetic resonance, but neither quantifies myocardial biology. Low native T1 in Fabry disease represents sphingolipid accumulation; late gadolinium enhancement with high T2 and troponin elevation reflects inflammation. We...

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Identifiers

PMID: 31826677
DOI: 10.1161/CIRCIMAGING.119.009430
PMCID: PMC6924943

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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