Brunklaus A, Parish E, Muntoni F, Scuplak S, Tucker S K, Fenton M, Hughes M L, Manzur A Y
Dubowitz Neuromuscular Centre, UCL Institute of Child Health & Great Ormond Street Hospital, London WC1N 3JH, UK.
Department of Paediatric Anaesthesia, Great Ormond Street Hospital, London WC1N 3JH, UK.
Eur J Paediatr Neurol. 2015 Jul;19(4):395-401. doi: 10.1016/j.ejpn.2015.03.008. Epub 2015 Mar 24.
BACKGROUND/PURPOSE: Duchenne Mmuscular Ddystrophy (DMD) related cardiomyopathy is associated with significant perioperative mortality. Cardiac MRI (CMR) has not previously been systematically evaluated as pre-operative assessment tool for heart function in DMD. Our aim was to establish whether CMR versus echocardiography contributes to pre-operative DMD assessment.
Case records were retrospectively reviewed of 35 consecutive DMD boys who underwent evaluation for surgical procedures between 2010 and 2013.
Echocardiography revealed a median left ventricular (LV) shortening fraction (SF) of 29/% (range: 7-44). 37% of boys (13/35) had abnormal SF <25%, 66% (23/35) showed hypokinesia and 26% (9/35) had LV dilatation. CMR revealed a median left ventricular ejection fraction (LVEF) of 52% (range: 27-67%). 57% of boys (20/35) had abnormal LVEF <55%, 71% (25/35) had hypokinesia, and 82% late gadolinium enhancement. Extensive versus minimal late gadolinium enhancement was associated with reduced left ventricular ejection fraction (48% vs 58%; p = 0.003) suggesting more severe cardiomyopathy. Although echocardiography shortening fraction correlated with CMR ejection fraction (rs = 0.67; p < 0.001), three-quarter of echocardiography studies had suboptimal scanning windows and in 26% measurements significantly over- or underestimated left-ventricular function compared to CMR.
Our findings clearly demonstrate the added value of CMR versus echocardiography in assessing DMD-cardiomyopathy. Particularly when echocardiographic scanning windows are suboptimal, CMR should be considered to allow accurate pre-operative cardiac assessment.
背景/目的：杜氏肌营养不良（DMD）相关的心肌病与围手术期的高死亡率相关。此前，心脏磁共振成像（CMR）尚未被系统评估作为DMD患者心脏功能的术前评估工具。我们的目的是确定CMR与超声心动图相比，是否有助于DMD的术前评估。
回顾性分析了2010年至2013年间连续接受手术评估的35例DMD男孩的病例记录。
超声心动图显示左心室（LV）缩短分数（SF）中位数为29%（范围：7%-44%）。37%的男孩（13/35）SF异常<25%，66%（23/35）表现为运动减弱，26%（9/35）有左心室扩张。CMR显示左心室射血分数（LVEF）中位数为52%（范围：27%-67%）。57%的男孩（25/35）LVEF异常<55%，71%（25/35）有运动减弱，82%有延迟钆增强。广泛与轻微延迟钆增强与左心室射血分数降低相关（48%对58%；p = 0.003），提示心肌病更严重。尽管超声心动图缩短分数与CMR射血分数相关（rs = 0.67；p < 0.001），但四分之三的超声心动图检查扫描窗口不理想，26%的测量结果与CMR相比显著高估或低估了左心室功能。
我们的研究结果清楚地证明了CMR与超声心动图相比在评估DMD心肌病方面的附加价值。特别是当超声心动图扫描窗口不理想时，应考虑使用CMR进行准确的术前心脏评估。
