Cui Yiqin, Feng Yijie, Xia Yu, Fu Xingpeng, Gong Ming, Qian Jingjing, Yu Jin, Ye Jingjing, Gao Feng, Cheng Hongqiang, Mao Shanshan
Department of Neurology, National Clinical Research Center for Child Health, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Department of Ultrasound, National Clinical Research Center for Child Health, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Front Cell Neurosci. 2022 Aug 18;16:953620. doi: 10.3389/fncel.2022.953620. eCollection 2022.
Patients with spinal muscular atrophy (SMA) may suffer from multisystem injury, including an impaired cardiovascular system. However, M-mode echocardiography, the current dominant echocardiographic modality, is limited in the detection of myocardial injury. We considered the use of left ventricular strain imaging in detecting myocardial injury and explored the serum lipid profile related to cardiovascular disease in later-onset SMA children.
A case-control study involving 80 patients with later-onset SMA and 80 age-, gender-, and body surface area-matched control children was conducted in a single tertiary pediatric hospital in China. Data on the left ventricular strain measured using two-dimensional speckle tracking echocardiography, left ventricular function parameters assessed by M-mode echocardiography, and serum lipid profile of these two groups were retrospectively collected for differential analysis.
The mean age of the 80 SMA patients were (6.87 ± 2.87) years, of which 46 were type 2 and 34 were type 3 patients. The global longitudinal strain (GLS) of the SMA group (-18.7 ± 2.9%, < 0.001) was lower than that of the control group; the time to peak longitudinal strain (TTPLS) of the SMA group (22.9 ± 13.6 ms, < 0.001) was higher than that of the control group, while left ventricular ejection fraction (LVEF) and left ventricular fractional shortening (LVFS), measured by the Teichholz method of M-mode echocardiography, showed no significant differences between the two groups. In addition, independent indicators for cardiovascular risk, including total cholesterol (TC)/HDL, low-density lipoprotein (LDL)/HDL, and Apo B/Apo A1 levels, were higher in SMA children than in the control group.
Compared with healthy controls, later-onset SMA children presented with reduced GLS and prolonged TTPLS while the LVEF and LVFS values were within normal range. In particular, whether a reduced GLS or prolonged TTPLS in later-onset SMA compared to the control group can predict the risk of future cardiomyopathy remains to be investigated.
脊髓性肌萎缩症（SMA）患者可能会遭受多系统损伤，包括心血管系统受损。然而，目前占主导地位的超声心动图检查方式——M型超声心动图，在检测心肌损伤方面存在局限性。我们考虑使用左心室应变成像来检测心肌损伤，并探讨晚发型SMA儿童中与心血管疾病相关的血脂谱情况。
在中国一家三级儿童医院进行了一项病例对照研究，纳入80例晚发型SMA患者以及80例年龄、性别和体表面积相匹配的对照儿童。回顾性收集两组患者使用二维斑点追踪超声心动图测量的左心室应变数据、M型超声心动图评估的左心室功能参数以及血脂谱数据，进行差异分析。
80例SMA患者的平均年龄为（6.87±2.87）岁，其中2型患者46例，3型患者34例。SMA组的整体纵向应变（GLS）（-18.7±2.9%，<0.001）低于对照组；SMA组的纵向应变达峰时间（TTPLS）（22.9±13.6毫秒，<0.001）高于对照组，而采用M型超声心动图的Teichholz法测量的左心室射血分数（LVEF）和左心室缩短分数（LVFS）在两组间无显著差异。此外，SMA儿童中包括总胆固醇（TC）/高密度脂蛋白（HDL）、低密度脂蛋白（LDL）/HDL以及载脂蛋白B/载脂蛋白A1水平等心血管风险独立指标高于对照组。
与健康对照组相比，晚发型SMA儿童表现为GLS降低和TTPLS延长，而LVEF和LVFS值在正常范围内。特别是，与对照组相比，晚发型SMA中GLS降低或TTPLS延长是否能预测未来心肌病的风险仍有待研究。
