Predictive Value of Myocardial Delayed Enhancement in Duchenne Muscular Dystrophy

被引:0
作者
Shaji C. Menon
Susan P. Etheridge
Kirk N. Liesemer
Richard V. Williams
Tyler Bardsley
Mason C. Heywood
Michael D. Puchalski
机构
[1] University of Utah,Division of Pediatric Cardiology
[2] University of Utah,Department of Pediatrics
来源
Pediatric Cardiology | 2014年 / 35卷
关键词
Duchenne muscular dystrophy; Myocardial delayed enhancement; Arrhythmias; Ventricular dysfunction;
D O I
暂无
中图分类号
学科分类号
摘要
In other cardiomyopathies, cardiac magnetic resonance imaging (CMR)-derived myocardial delayed enhancement (MDE), a marker of myocardial fibrosis, is a risk factor for sudden cardiac death (SCD). In Duchenne muscular dystrophy (DMD), the prognostic value of MDE for ventricular arrhythmias and death is unknown. This study aimed to evaluate associations between MDE and electrocardiographic (ECG) changes, ventricular remodeling, risk of arrhythmias, and death in DMD. This retrospective study included all subjects with DMD who had undergone a CMR between January 2006 and December 2011 and had available ECG and 24-h Holter records from the same period. Left ventricular (LV) MDE was semiquantitatively graded from 0 to 4. Comparisons of demographic and clinical characteristics between MDE and no-MDE groups were made. Cox regression analysis was performed to assess factors associated with death. This study investigated 32 boys with a median age of 13.8 years (range, 7.2–17.4 years) and found MDE present in 25 (78 %) of the boys. Compared with the no-MDE subjects, the MDE subjects were older (15.7 ± 3.3 vs 12.1 ± 4.8 years) and had a wider QT dispersion (QTd: 74 ± 30 vs 55 ± 33 ms), a higher incidence of ventricular tachycardia (40 vs 0 %), a lower LV ejection fraction (46 ± 12 vs 56 ± 9 %), a larger LV end-diastolic volume (124 ± 58 vs 68 ± 14 ml/m2), and a larger end-systolic volume (57 ± 29 vs 28 ± 10 ml/m2) (p < 0.05 for all). During the study period, six of the subjects (19 %) died. The factors associated with mortality were increased age, advanced grade of MDE, higher LV end-systolic volume, lower LV ejection fraction, use of beta-blockers, and ventricular tachycardia. Myocardial fibrosis detected by CMR is an independent predictor of adverse cardiac remodeling, ventricular arrhythmias, and death in DMD. Cardiac MRI using MDE can be applied as a screening tool to detect patients at risk for ventricular arrhythmias, more advanced disease, adverse LV remodeling, and death.
引用
收藏
页码:1279 / 1285
页数:6
相关论文
共 50 条
  • [31] Cardiac atrial pathology in Duchenne muscular dystrophy
    Greiner, Eleanor
    Breaux, Andrea
    Kasten, Jennifer
    Seo, Jangdong
    Ollberding, Nicholas J.
    Spar, David
    Ryan, Thomas D.
    Lang, Sean M.
    Tian, Cuixia
    Sawnani, Hemant
    Villa, Chet R.
    MUSCLE & NERVE, 2024, 69 (05) : 572 - 579
  • [32] Cardiac medication management in Duchenne muscular dystrophy
    Wittekind, Samuel G.
    Villa, Chet R.
    PEDIATRIC PULMONOLOGY, 2021, 56 (04) : 747 - 752
  • [33] Increased susceptibility of the left lateral free wall to myocardial delayed enhancement in Duchenne Muscular Dystrophy: progressive systolic dysfunction demonstrable by CMR regional strain analysis
    Narayan Kissoon
    Kan N Hor
    Janaka P Wansapura
    Wojciech Mazur
    Robert J Fleck
    Micheal D Puchalski
    D Woodrow Benson
    William M Gottliebson
    Journal of Cardiovascular Magnetic Resonance, 11 (Suppl 1)
  • [34] Left ventricular deformation and myocardial fibrosis in pediatric patients with Duchenne muscular dystrophy
    Kerstens, Thijs P.
    van Everdingen, Wouter M.
    Habets, Jesse
    van Dijk, Arie P. J.
    Helbing, Willem A.
    Thijssen, Dick H. J.
    Cate, Floris E. A. Udink ten
    INTERNATIONAL JOURNAL OF CARDIOLOGY, 2023, 388
  • [35] Prevalence and distribution of regional scar in dysfunctional myocardial segments in Duchenne muscular dystrophy
    Kenneth C Bilchick
    Michael Salerno
    David Plitt
    Yoav Dori
    Thomas O Crawford
    Daniel Drachman
    W Reid Thompson
    Journal of Cardiovascular Magnetic Resonance, 13
  • [36] Duchenne Muscular Dystrophy Fatigue Trajectories
    Wei, Yi Sally
    Hnaini, Mona
    ElAloul, Basmah
    Zapata, Eugenio
    Campbell, Craig
    NEUROPEDIATRICS, 2024, 55 (01) : 42 - 48
  • [37] Update on the Treatment of Duchenne Muscular Dystrophy
    Rodino-Klapac, Louise R.
    Mendell, Jerry R.
    Sahenk, Zarife
    CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS, 2013, 13 (03)
  • [38] Targeting Fibrosis in Duchenne Muscular Dystrophy
    Zhou, Lan
    Lu, Haiyan
    JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 2010, 69 (08) : 771 - 776
  • [39] Scoliosis in Duchenne muscular dystrophy (DMD)
    Hsu, John D.
    Quinlivan, Ros
    NEUROMUSCULAR DISORDERS, 2013, 23 (08) : 611 - 617
  • [40] Gene therapy for Duchenne muscular dystrophy
    Suzuki, Naoki
    Miyagoe-Suzuki, Yuko
    Takeda, Shin'ichi
    FUTURE NEUROLOGY, 2007, 2 (01) : 87 - 96