PMID- 30564867
OWN - NLM
STAT- MEDLINE
DCOM- 20190412
LR  - 20200225
IS  - 1432-1971 (Electronic)
IS  - 0172-0643 (Linking)
VI  - 40
IP  - 2
DP  - 2019 Feb
TI  - Progressive left ventricular dysfunction and myocardial fibrosis in Duchenne and 
      Becker muscular dystrophy: a longitudinal cardiovascular magnetic resonance 
      study.
PG  - 384-392
LID - 10.1007/s00246-018-2046-x [doi]
AB  - 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, five patients had already been treated with ACEI at the first CMR; 
      five 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 (beta = - 1.1%/year; 95% confidence interval [CI], - 1.8% 
      to - 0.4%; p = 0.005) and no use of ACEI (beta = - 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 (beta = 3.7%; 
      95% CI, 0.9-6.4%; p = 0.010). ACEI use or the time of initiation of ACEI did not 
      significantly affect age-related increase in LGE. 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.
FAU - Aikawa, Tadao
AU  - Aikawa T
AD  - Department of Cardiovascular Medicine, Hokkaido University Hospital, Kita-14, 
      Nishi-5, Kita-ku, Sapporo, 060-8648, Japan.
FAU - Takeda, Atsuhito
AU  - Takeda A
AD  - Department of Pediatrics, Hokkaido University Hospital, Kita-14, Nishi-5, 
      Kita-ku, Sapporo, 060-8648, Japan.
FAU - Oyama-Manabe, Noriko
AU  - Oyama-Manabe N
AD  - Department of Diagnostic and Interventional Radiology, Hokkaido University 
      Hospital, Kita-14, Nishi-5, Kita-ku, Sapporo, 060-8648, Japan. 
      norikooyama@med.hokudai.ac.jp.
FAU - Naya, Masanao
AU  - Naya M
AD  - Department of Cardiovascular Medicine, Hokkaido University Hospital, Kita-14, 
      Nishi-5, Kita-ku, Sapporo, 060-8648, Japan.
FAU - Yamazawa, Hirokuni
AU  - Yamazawa H
AD  - Department of Pediatrics, Hokkaido University Hospital, Kita-14, Nishi-5, 
      Kita-ku, Sapporo, 060-8648, Japan.
FAU - Koyanagawa, Kazuhiro
AU  - Koyanagawa K
AD  - Department of Cardiovascular Medicine, Hokkaido University Hospital, Kita-14, 
      Nishi-5, Kita-ku, Sapporo, 060-8648, Japan.
FAU - Ito, Yoichi M
AU  - Ito YM
AD  - Department of Biostatistics, Faculty of Medicine, Graduate School of Medicine, 
      Hokkaido University, Kita-15, Nishi-7, Kita-ku, Sapporo, 060-8638, Japan.
FAU - Anzai, Toshihisa
AU  - Anzai T
AD  - Department of Cardiovascular Medicine, Hokkaido University Hospital, Kita-14, 
      Nishi-5, Kita-ku, Sapporo, 060-8648, Japan.
LA  - eng
PT  - Journal Article
DEP - 20181218
PL  - United States
TA  - Pediatr Cardiol
JT  - Pediatric cardiology
JID - 8003849
RN  - 0 (Angiotensin-Converting Enzyme Inhibitors)
SB  - IM
MH  - Adolescent
MH  - Angiotensin-Converting Enzyme Inhibitors/*therapeutic use
MH  - Child
MH  - Disease Progression
MH  - Female
MH  - Fibrosis/etiology
MH  - Humans
MH  - Longitudinal Studies
MH  - Magnetic Resonance Imaging, Cine/*methods
MH  - Male
MH  - Muscular Dystrophy, Duchenne/*complications
MH  - Myocardium/*pathology
MH  - Retrospective Studies
MH  - Ventricular Dysfunction, Left/diagnostic imaging/drug therapy/*etiology
MH  - Ventricular Function, Left/drug effects
MH  - Young Adult
OTO - NOTNLM
OT  - Angiotensin-converting enzyme inhibitor
OT  - Becker muscular dystrophy
OT  - Cardiovascular magnetic resonance imaging
OT  - Duchenne muscular dystrophy
OT  - Late gadolinium enhancement
OT  - Mixed-effects model
EDAT- 2018/12/20 06:00
MHDA- 2019/04/13 06:00
CRDT- 2018/12/20 06:00
PHST- 2018/09/18 00:00 [received]
PHST- 2018/12/09 00:00 [accepted]
PHST- 2018/12/20 06:00 [pubmed]
PHST- 2019/04/13 06:00 [medline]
PHST- 2018/12/20 06:00 [entrez]
AID - 10.1007/s00246-018-2046-x [pii]
AID - 10.1007/s00246-018-2046-x [doi]
PST - ppublish
SO  - Pediatr Cardiol. 2019 Feb;40(2):384-392. doi: 10.1007/s00246-018-2046-x. Epub 
      2018 Dec 18.