摘要
儿童肥厚型心肌病(HCM)是由编码心肌肌节蛋白的基因突变所致的最常见的遗传性心肌病之一,主要特征为心室肥厚、心腔无扩大,存在心源性猝死的潜在风险。通常依据患者的一般情况、临床症状、影像学检查结果、家族史、基因检测等进行评估,以此为判断心源性猝死风险提供依据,进而明确肥厚型心肌病的心源性猝死危险因素。本文依据近几年国内外相关文献以及北京儿童医院心脏内科的工作经验积累,将这些危险因素分为主要危险因素及次要危险因素。全面了解这些危险因素,能够指导临床对可能发生猝死的高危患儿进行早期预警,有助于更准确地评估儿童肥厚型心肌病的预后并实施针对性干预。本文对儿童肥厚型心肌病预后的相关危险因素研究进展予以综述。
Hypertrophic cardiomyopathy(HCM)in children is one of the most common hereditary cardiomyopathies caused by gene mutations encoding cardiac carcomeric proteins.It is mainly characterized by ventricular hypertrophy and non-enlarged cardiac chambers,with a potential risk of sudden cardiac death.Usually,the assessment is based on the general condition,clinical symptoms,imaging examination results,family history,and genetic testing of the patients,thereby providing a basis for judging the risk of sudden cardiac death,and then identifying the risk factors for sudden cardiac death in hypertrophic cardiomyopathy.Based on the relevant domestic and foreign literature in recent years and the accumulated work experience in the department of cardiology of Beijing Children's Hospital,these risk factors are divided into major and minor risk factors.A comprehensive understanding of these risk factors can guide the clinical early warning of high-risk children with possible sudden death,which is helpful for more accurate assessment of the prognosis of hypertrophic cardiomyopathy in children and the implementation of targeted intervention.This article reviewed the research progress of related risk factors for the prognosis of hypertrophic cardiomyopathy in children.
作者
田芝瑜
甄珍
高路
袁越
Tian Zhiyu;Zhen Zhen;Gao Lu;Yuan Yue(Department of Cardiology,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China)
出处
《中国小儿急救医学》
CAS
2024年第11期861-866,共6页
Chinese Pediatric Emergency Medicine
关键词
肥厚型心肌病
预后
心源性猝死
危险因素
Hypertrophic cardiomyopathy
Prognosis
Sudden cardiac death
Risk factors
