婴幼儿先天性心脏病并心内膜弹力纤维增生症的临床特点

Clinical Character of 21 Infants with Congenital Heart Disease Unite Endocardial Fibroelastosis

目的研究婴幼儿先天性心脏病(CHD)并心内膜弹力纤维增生症(EFE)的临床及实验室特点,诊断和治疗。方法对本院收治婴幼儿CHD并EFE患儿的临床及实验室检查资料进行回顾性分析、总结。结果3166例CHD中21例(0.66%)并EFE,其中动脉导管未闭(PDA)5例,房间隔缺损(ASD)6例,室间隔缺损(VSD)6例,PDA+VSD 1例,ASD+VSD 2例.法洛四联症1例。临床有不同程度心力衰竭,多伴肺炎,心脏增大较明显,起病绝大多数较急,病情多较重.预后较差。结论CHD是引起EFE的原因之一,但不除外其他原因尤其是病毒感染导致EFF;疾病预后可能与心脏扩大程度、心力衰竭程度、并发症严重程度及治疗及时与否有关。

Objective To report and recognize the clinical and laboratory character, dignosis and treatment of congenital heart di-sease(CHD) unite endocardial fibroelastosis( EFE). Methods Take retrospective analysis and summary to the data of manifestation and laboratory examination of 21 case infant with CHD unite EFE. Results Five patent ductus arteriosus(PDA). 6 atrial septal defect (ASD), 6 ventricular septal defect(VSU),1 PDA + VSD.2 ASD + VSD,and 1 Fallot tetralogy among 21 patients with CHD unite EFE, they had a different degree heart failure, pneumonia,apparent cardiac enlargement; the majority of them had a urgent episode, severe state of an illness, and bad prognoses. Conclusions CHD is an etiological factor of EFE, there probably are other cause,above all viral infection;prognoses of disease probably correlate degree of the cardiac enlargement,heart failure, complication and time of thera-peusis;concrete treatment and long- term prognoses wait for more study