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目的探讨儿童心肌致密化不全(NVM)的临床表现及超声心动图诊断的临床意义。方法对2002-08—2005-11深圳市儿童医院超声心动图9238例次中确诊45例儿童NVM的超声心动图资料进行回顾分析。结果超声心动图确诊NVM45例,患病率为0.48%(45/9238),男29例,女16例,男女比为1.8∶1,年龄3日龄至8岁,平均年龄2.21岁。孤立型31例,非孤立型14例。孤立型临床表现:心前区收缩期Ⅱ~Ⅲ/Ⅵ级杂音6例,X线平片见心影增大3例,室性早搏1例,心衰3例,无临床表现28例。非孤立型临床表现:癫癎2例,紫绀4例,心衰9例,非孤立型14例中合并有先天性心脏病9例,心内膜弹力纤维增生症3例,心脏错构瘤2例。超声心动图示左室型36例(87%),右室型3例(6.7%),双室型6例(13%);随访死于心衰5例(5/45,病死率11%)。结论婴幼儿及儿童心肌致密化不全并非罕见,在先天性心脏病中发生率较高,且易导致小婴儿心衰,临床表现与NVM的大小范围有关,孤立型患儿早期多无临床表现,超声心动图特征性声像图对早期诊断、评价心脏功能、判断预后有重要临床意义。
Objective To investigate the clinical manifestations of children with myocardial degeneration (NVM) and the clinical significance of echocardiography. Methods The echocardiographic data of 45 children with NVM diagnosed in 9,238 cases of echocardiography of Shenzhen Children’s Hospital from August 2002 to August 2005 were retrospectively analyzed. Results Echocardiography confirmed 45 cases of NVM, the prevalence rate was 0.48% (45/9238), 29 males and 16 females, male to female ratio of 1.8: 1, ranging in age from 3 days to 8 years with a mean age of 2.21 years. Isolated type in 31 cases, non-isolated type in 14 cases. Isolated clinical manifestations: precordial systolic Ⅱ ~ Ⅲ / Ⅵ level murmur in 6 cases, X-ray see the heart shadow increased in 3 cases, 1 case of ventricular premature beats, heart failure in 3 cases, no clinical manifestations in 28 cases. Non-isolated clinical manifestations: 2 cases of epilepsy, cyanosis in 4 cases, 9 cases of heart failure, non-isolated type 14 cases of congenital heart disease in 9 cases, 3 cases of endocardial fibroelastosis, cardiac hamartoma 2 example. Echocardiography showed left ventricular in 36 cases (87%), right ventricular in 3 cases (6.7%) and double ventricular in 6 cases (13%). Five patients died of heart failure at follow-up (5/45, ). Conclusion Infants and children with myocardial densification is not uncommon, the incidence of congenital heart disease is high, and easily lead to small infants with heart failure, clinical manifestations and the size of the NVM range, isolated children with no clinical manifestations early, Echocardiographic features of the echographic image of early diagnosis, evaluation of cardiac function, prognosis of important clinical significance.