中文摘要：

目的 探讨伴有先天性心脏病（congenital heart disease,CHD）的癫痫患儿的临床特征,为临床诊疗提供理论依据。方法 回顾性分析78例伴有CHD的癫痫患儿的临床资料并总结其临床特征。结果 78例伴有CHD的癫痫患儿中,男44例（56.4%）,女34例（43.6%）,年龄范围为1个月~15岁。左向右分流CHD有41例（52.6%）,右向左分流CHD有32例（41.0%）,无分流的CHD有5例（6.4%）;其中室间隔缺损有24例,动脉导管未闭及法洛四联症各9例,完全性大动脉转位7例,单心室及房间隔缺损各5例,右心室双出口4例,其余为其他先天性心脏畸形。不同CHD分型患儿术前的头颅核磁共振成像（magnetic resonance imaging,MRI）比较,差异无统计学意义（似然比χ2=2.13,P〉0.05）;不同CHD分型患儿术后头颅MRI比较,差异有统计学意义（似然比χ2=6.2,P=0.04）。右向左分流CHD术后头颅MRI改变常见为多脑叶的缺血梗死软化灶伴脑萎缩,而左向右分流CHD术后头颅MRI改变多为局灶性小缺血梗死灶。脑电图表现为背景节律以中-低波幅的慢活动为主,多见于额、颞叶及中央区阵发尖慢波、棘慢波、多棘慢波的痫样放电。结论 癫痫是先天性心脏病常见的神经系统合并症,早期可通过头颅MRI及脑电图检查评估脑损伤,早期进行干预治疗,降低癫痫发生率,减少神经系统的损伤。

英文摘要：

Objectives To analyze the clinical features of epilepsy in children with congenital heart disease（CHD）,and to provide theoretical basis for its clinical diagnosis and treatment. Methods We retrospectively analyzed the clinical data of 78 children with epilepsy and CHD, and summarized their clinical characteristics. Results Of the 78 children with epilepsy and CHD, 44（56.4%） were male and 34（43.6%） were female, aged from 1 month to 15 years old. Cases of left-to-right shunt CHD, right-to-left shunt CHD and no shunt CHD were 41（52.6%）, 32（41.0%） and5（6.4%） respectively, including 24 cases with ventricular septal defect（VSD）, 9 with patent ductus arteriosus（PDA）, 9 with tetralogy of Fallot（TOF）, 7 with transposition of the great arteries（TGA）, 5 with single ventricle defect（SVD）, 5 with atrial septal defect（ASD）, 4 with double outlet right ventricle（DORV）. The difference in preoperative brain magnetic resonance imaging（MRI） among different types of CHD was not significant（χ2=2.13,P〉0.05）; but the difference in postoperative brain MRI among different types of CHD was significant（χ2=6.2, P =0.04）.The common change of postoperative brain MRI in right-to-left shunt CHD patients was multiple cerebral ischemic infarction and encephalatrophy, while the change of postoperative brain MRI in left-to-right shunt CHD patients was small focal ischemic infarction. The background of electroencephalogram（EEG） showed a slow activity with mediumlow amplitude, discharging in frontal, temporal and central lobes, including sharp-slow wave, spike-slow wave and multiple spike-slow wave. Conclusions Epilepsy is a common nerve complication in CHD heart disease. We should assess brain injury through MRI and EEG in order to provide intervention treatment timely and reduce the incidence of epilepsy and nerve damage.