| 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 link.springer.com |
| Type: | article (author version) |
| URI: | http://hdl.handle.net/2115/76647 |
| 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)
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