Key result
Elevated extracellular volume fraction was observed in 5 of 6 male patients with muscular dystrophy and all 5 female putative carriers, indicating early diffuse myocardial fibrosis.
Why the study?
Does CMR with T1-mapping and ECV quantification detect diffuse myocardial fibrosis in patients and putative carriers of muscular dystrophy?
Cross-Sectional (n=11)
No
Does CMR with T1-mapping and ECV quantification detect diffuse myocardial fibrosis in patients and putative carriers of muscular dystrophy?
Myocardial T1-mapping and ECV quantification can detect early diffuse myocardial fibrosis in both patients with muscular dystrophy and asymptomatic female carriers, potentially preceding overt LV dysfunction or LGE.
Answers are grounded in this paper and cite their sources.
May support early CMR ECV screening in muscular dystrophy; leaves open prospective validation before practice change.
-mapping and extracellular volume fraction (ECV) quantification was prospectively performed using cardiovascular MR (CMR) imaging in 6 male patients with muscular dystrophy and 5 female putative carriers of Duchenne or Becker muscular dystrophy. Five patients and all putative carriers had an elevated ECV (>29.5% for men and >35.2% for women), suggesting that ECV has a potential to detect diffuse fibrotic changes in patients and putative carriers of muscular dystrophy.
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Koyanagawa et al. (2020) conducted a cross-sectional in Muscular dystrophy and putative carriers (n=11). Myocardial T1-mapping and extracellular volume fraction (ECV) quantification vs. Historical healthy reference values was evaluated on Elevated extracellular volume fraction (ECV). Elevated extracellular volume fraction was observed in 5 of 6 male patients with muscular dystrophy and all 5 female putative carriers, indicating early diffuse myocardial fibrosis.
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