摘要
儿童扩张型心肌病(dilated cardiomyopathy,DCM)主要临床表现为心力衰竭、心律失常、血栓形成、血栓栓塞及猝死。DCM预后差,病死率高,诊断后5年、10年内无移植生存率分别为54%~72%、46%~62%。诊断时左室射血分数较高、病因为心肌炎提示预后较好,诊断时年龄偏大、有心肌病家族史、心律失常、严重二尖瓣反流、左室舒张和收缩末期内径越大、心胸比例>0.65提示预后可能较差。儿童DCM治疗以药物为主,以心室逆重构及改善长期预后为目标,主要药物为血管紧张素转化酶抑制剂、β受体阻滞剂、醛固酮受体拮抗剂,部分患者药物治疗后心脏功能和大小可以恢复正常;而伊伐布雷定、心肌代谢药物、丙种球蛋白也被报道可改善心功能。
Pediatric dilated cardiomyopathy(DCM)is a myocardial disorder characterized by a dilated left ventricular chamber and systolic dysfunction that often manifested as heart failure,arrhythmia,thrombosis,thromboembolism and sudden death.Survival free from transplantation was 54%-72%5 years after diagnosis,and 46%-62%at 10 years.Several factors indicating better prognosis are higher left ventricular ejection fraction,and the presence of myocarditis.Other predictors such as older age at diagnosis,a family history of cardiomyopathy,arrhythmias,severe mitral regurgitation,lager left ventricular end-diastolic dimension and end-systolic dimension,and cardiothoracic ratio greater than 65%suggest that the prognosis may be poor.The cornerstone of pediatric DCM therapy is drug therapy including angiotensin-converting enzyme inhibitors,β-blockers and aldosterone antagonists.Ivabradine,myocardial metabolic drugs and intravenous immunoglobulins may be associated with better improvement of systolic left ventricular function.
作者
潘宇(综述)
刘晓燕(审校)
Pan Yu;Liu Xiaoyan(Department of Cardiology,Children′s Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Developmental Disorder,National Clinical Research Center for Child Health and Disorders,China International Science and Technology Cooperation Base of Child Development and Critical Disorders,Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China)
出处
《国际儿科学杂志》
2020年第4期227-230,共4页
International Journal of Pediatrics
关键词
扩张型心肌病
儿童
药物治疗
预后
风险预测
Dilated cardiomyopathy
Children
Drug therapy
Outcomes
Predictors
作者简介
通信作者:刘晓燕,Email:lxylxy65@163.com。
