摘要
心肌致密化不全(NVM)是一种少见的先天性遗传性心肌病,以心腔内异常粗大的肌小梁和小梁间深陷的隐窝为特征。其临床表现多样且无特异性,以心功能不全、心律失常、血栓形成为主要特征。超声心动图和心脏磁共振成像在NVM诊断中具有重要意义。治疗上主要是对症治疗,内科治疗无效者可行心脏移植。本病预后差异大,病死率高,但无症状者预后相对较好。
Non-compaction of ventricular myocardium(NVM),a rare congenital and inherited cardiomyopathy,is characterized by prominent trabeculations and deep intertrabecular recesses in communication with the ventricular cavity.NVM has diverse clinical presentations without specificity,of which the major characteristics are cardiac insufficiency,arrhythmias and thrombosis.Echocardiography and cardiac magnetic resonance imaging are necessary technologies to diagnose NVM.Symptomatic therapy is the only choice for most patients.Furthermore,if medical treatment fails,the heart transplantation could be performed.As a whole,NVM has a high mortality and entirely different prognosis,however,the prognosis of asymptomatic patients is relatively good.
作者
宋开艳
聂抒
韩燕燕
Song Kaiyan;Nie Shu;Han Yanyan(Department of Pediatric Cardiovascular Medicine,the First Hospital of Jinlin University,Changchun 130021,China;Department of Pediatric Cardiovascular Medicine,Qilu Children′s Hospital of Shandong University,Jinan 250022,China)
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2020年第1期70-73,共4页
Chinese Journal of Applied Clinical Pediatrics
关键词
心肌致密化不全
心功能不全
心律失常
血栓形成
Non-compaction of ventricular myocardium
Cardiac insufficiency
Arrhythmia
Thrombosis
