中国循证儿科杂志 ›› 2023, Vol. 18 ›› Issue (4): 255-260.DOI: 10.3969/j.issn.1673-5501.2023.04.002

• 论著 • 上一篇    下一篇

杜氏肌营养不良男性患儿486份心电图特征病例系列报告

张琳玲1,3,王川1,3,谢佩环1,3,邵淑冉1,3,魏丽1,3,周晖2,3,蔡晓唐2,3,周开宇1,3   

  1. 四川大学华西第二医院成都,610041;1 儿童心血管科,2 儿童康复科,3 西部妇幼研究院心脏发育及心肌疾病基因治疗研究平台、出生缺陷与相关妇儿疾病教育部重点实验室


  • 收稿日期:2023-07-18 修回日期:2023-08-08 出版日期:2023-08-25 发布日期:2023-08-25
  • 通讯作者: 周开宇,蔡晓唐

Analysis of 486 ECG characteristics in boys with duchenne muscular dystrophy: A case series report

ZHANG Linling1,3, WANG Chuan1,3, XIE Peihuan1,3, SHAO Shuran1,3, WEI Li1,3, ZHOU Hui2,3, CAI Xiaotang2,3, ZHOU Kaiyu1,3   

  1. West China Second University Hospital, Sichuan University, Chengdu 610041,China, 1 Department of Pediatric Cardiology; 2 Department of Pediatric Rehabilitation; 3 Research Platform on Cardiac Development and Gene Therapy of Myocardial Disease in Western Maternal and Child Research Institute, Key Laboratory of Birth Defects and Related Diseases of Women and Children, Ministry of Education

  • Received:2023-07-18 Revised:2023-08-08 Online:2023-08-25 Published:2023-08-25
  • Contact: ZHOU Kaiyu, email: kaiyuzhou313@163.com; CAI Xiaotang, email: cxt1999@126.com

摘要: 背景:心血管并发症已成为杜氏肌营养不良(DMD)患儿死亡的首要原因,心电图正逐步成为监测DMD心肌受累的早期敏感指标。 目的:分析DMD患儿的心电图特征。 设计:病例系列报告。 方法:分析304例基因确诊的DMD患儿486份心电图特点和指标,并与112份按年龄匹配的正常儿童心电图参数进行对比分析。 主要结局指标:DMD患儿心电图异常发生率及其类型。 结果:DMD患儿心电图异常发生率为38.7%(188/486);心电图异常类型主要包括窦性心动过速、左室高电压/左室肥厚、右室肥厚、双室肥厚、STT改变、T波改变、病理性Q波、完全性右束支传导阻滞(CRBBB)、不完全性右束支传导阻滞(ICRBBB)、室内传导阻滞、房性早搏/室性早搏、短PR综合征,未见长QT综合征及其他类型传导阻滞;DMD患儿心电图异常发生率随着年龄增长呈显著上升趋势(P<0.05);与正常儿童相比, DMD患儿和左室射血分数(LVEF)正常的DMD患儿均心率显著增快、PR间期显著缩短、RV1和RV5振幅显著升高(P均<0.05)。 结论:心电图异常是DMD患儿的常见表现,随着年龄增长其发生率逐渐升高;心率增快、PR间期缩短以及RV1振幅增高是DMD患儿心电图异常的早期以及特征性改变。

关键词: 关键词:肌营养不良, 杜氏, 心电描记术, 儿童

Abstract: Background:Cardiovascular complications have become the leading cause of death in children with Duchenne muscular dystrophy(DMD). Electrocardiography(ECG) is emerging as an early sensitive indicator for monitoring myocardial involvement in this population. Objective:To analyze ECG characteristics in children with DMD. Design:Retrospective cohort study. Methods:The characteristics of 486 ECG in 304 children with genetically diagnosed DMD were retrospectively analyzed, and compared with 112 ECG indices of 112 normal children in each age group. Main outcome measures:The incidence and type of ECG abnormalities in children with DMD. Results:The overall incidence of abnormal ECG in children with DMD was 38.7% (188/486). ECG abnormalities mainly include sinus tachycardia, left ventricular hyper voltage/left ventricular hypertrophy, right ventricular hypertrophy, double ventricular hypertrophy, STT changes, T wave changes, pathological Q waves,incomplete right bundle branch block(ICRBBB), complete right bundle branch block(CRBBB), ventricular block, atrial premature beats/ventricular premature beats and short PR syndrome.No long QT syndrome and other types of conduction block were identified. The incidence of abnormal ECG in children with DMD regardless of LVEF increased significantly with age (P<0.05), and children with DMD had significantly faster heart rate, significantly shortened PR interval, and significantly increased amplitude of RV1 and RV5, compared to agematched healthy children (P<0.05). Conclusions:ECG abnormalities are common manifestations in children with DMD, and their incidence gradually increases with age, and increased heart rate, shortened PR interval, and increased RV1 amplitude are early characteristic changes in ECG abnormalities in children with DMD.

Key words: Keywords Muscular dystrophy, Duchenne, Electrocardiography, Pediatrics