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

Aikawa, Tadao; Takeda, Atsuhito; Oyama-Manabe, Noriko; Naya, Masanao; Yamazawa, Hirokuni; Koyanagawa, Kazuhiro; Ito, Yoichi M.; Anzai, Toshihisa

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

Aikawa, Tadao; Takeda, Atsuhito; Oyama-Manabe, Noriko et al.

Pediatric Cardiology, 2019, 40(2), 384-392

Permalink : https://hdl.handle.net/2115/76647

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アクセス権	open access
URI	https://hdl.handle.net/2115/76647
タイトル	Progressive left ventricular dysfunction and myocardial fibrosis in Duchenne and Becker muscular dystrophy : a longitudinal cardiovascular magnetic resonance study
著者	Aikawa, Tadao ; Takeda, Atsuhito ; Oyama-Manabe, Noriko ; Naya, Masanao ; Yamazawa, Hirokuni ; Koyanagawa, Kazuhiro ; Ito, Yoichi M. ; Anzai, Toshihisa
e-Rad_Researcher 20795059 著者名 Aikawa, Tadao
e-Rad_Researcher 00374413 著者名 Takeda, Atsuhito
e-Rad_Researcher 70463742 著者名 Oyama-Manabe, Noriko
e-Rad_Researcher 20455637 著者名 Naya, Masanao
著者名 Yamazawa, Hirokuni
著者名 Koyanagawa, Kazuhiro
e-Rad_Researcher 10334236 著者名 Ito, Yoichi M.
e-Rad_Researcher 60232089 著者名 Anzai, Toshihisa
言語	英語
キーワード	Duchenne muscular dystrophy
	Becker muscular dystrophy
	Angiotensin-converting enzyme inhibitor
	Cardiovascular magnetic resonance imaging
	Late gadolinium enhancement
	Mixed-effects model
発行日	2019-02
出版者	Springer
収録物名	Pediatric Cardiology
巻(号)	40(2)
ページ	384-392
抄録	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.
資源タイプ	学術雑誌論文
出版タイプ	Accepted Manuscript
権利情報	The final publication is available at link.springer.com
PISSN	0172-0643
関連情報 (isVersionOf)	出版社版DOI https://doi.org/10.1007/s00246-018-2046-x