HUSCAP logo Hokkaido Univ. logo

Hokkaido University Collection of Scholarly and Academic Papers >
Hokkaido University Hospital >
Peer-reviewed Journal Articles, etc >

Progressive left ventricular dysfunction and myocardial fibrosis in Duchenne and Becker muscular dystrophy : a longitudinal cardiovascular magnetic resonance study

Files in This Item:
HUSCAP_PEDC-D-18-00579.pdf355.71 kBPDFView/Open
Please use this identifier to cite or link to this item:

Title: Progressive left ventricular dysfunction and myocardial fibrosis in Duchenne and Becker muscular dystrophy : a longitudinal cardiovascular magnetic resonance study
Authors: Aikawa, Tadao Browse this author →KAKEN DB
Takeda, Atsuhito Browse this author →KAKEN DB
Oyama-Manabe, Noriko Browse this author →KAKEN DB
Naya, Masanao Browse this author →KAKEN DB
Yamazawa, Hirokuni Browse this author
Koyanagawa, Kazuhiro Browse this author
Ito, Yoichi M. Browse this author →KAKEN DB
Anzai, Toshihisa Browse this author →KAKEN DB
Keywords: Duchenne muscular dystrophy
Becker muscular dystrophy
Angiotensin-converting enzyme inhibitor
Cardiovascular magnetic resonance imaging
Late gadolinium enhancement
Mixed-effects model
Issue Date: Feb-2019
Publisher: Springer
Journal Title: Pediatric Cardiology
Volume: 40
Issue: 2
Start Page: 384
End Page: 392
Publisher DOI: 10.1007/s00246-018-2046-x
Abstract: This study examined the progression of left ventricular dysfunction and myocardial fibrosis in patients with Duchenne muscular dystrophy (DMD) or Becker muscular dystrophy (BMD) to evaluate the effects of angiotensin-converting enzyme inhibitor (ACEI). Ninety-eight cardiovascular magnetic resonance (CMR) studies in 34 consecutive patients with DMD (n=21) or BMD (n=13) were retrospectively reviewed. Left ventricular ejection fraction (LVEF) and the extent of myocardial late gadolinium enhancement (LGE) were semiautomatically quantified. During the study period, 5 patients had already been treated with ACEI at the first CMR; 5 were started on ACEI at LVEF≥55% and 10 at LVEF<55%. All patients had hyperenhanced myocardium on LGE images at the first CMR (median extent, 3.3%; interquartile range, 0.1%-14.3%). A mixed-effects model for longitudinal data of each patient, adjusted for age, type of muscular dystrophy, steroid use, and ACEI use, showed that higher age (β=-1.1%/year; 95% confidence interval [CI], -1.8% to -0.4%; p=0.005) and no use of ACEI (β=-3.1%; 95% CI; -5.4% to -0.8%; p=0.009) were significantly associated with a lower LVEF. When ACEI use was stratified by time of initiation (LVEF≥55% vs. <55%), only ACEI initiation at LVEF<55% had a beneficial effect on LVEF at each imaging examination (β=3.7%; 95% CI, 0.9% to 6.4%; p=0.010). ACEI use or the time of initiation of ACEI did not significantly affect age-related increase in LGE. Conclusion: ACEI attenuated the age-related decline in LVEF only in patients with DMD or BMD and reduced LVEF, suggesting that further investigation on prophylactic use of cardioprotective therapy in these patients is warranted.
Rights: The final publication is available at
Type: article (author version)
Appears in Collections:北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 相川 忠夫

Export metadata:

OAI-PMH ( junii2 , jpcoar )

MathJax is now OFF:


 - Hokkaido University