复杂先心病病变类型以及与心脏位置相关性分析

【目的】分析复杂性先天性心脏病(复杂先心病)变类型与心脏位置之间的相关性。【方法】收集1986年9月至2000年2月期间81例复杂先心病资料，就病变类型与心脏位置相关性作一分析。【结果】在正常或镜面右位心中，复杂先心病发病率与性别无关，但在心脏位置异常情况下男女之间有显著差别（4∶1），本组就诊年龄平均在4岁，肺动脉瓣闭锁及左心室发育不全综合症常构成新生儿急诊。主要病变为大动脉错位38％、单心室16％、动脉共干13％、三尖瓣闭锁10%。正常或镜面右位心中大动脉转位71％系完全型，而心脏异位中69％系矫正型，此类畸形常伴畸形为室间隔缺损及肺动脉瓣狭窄。左旋心、孤立左位心常伴有最复杂心内畸形、畸型数达2～8(平均4.89±1.76)。本组病例中镜面右位心伴复杂心内畸形并非少见。还发现2例完全型体静脉异位引流。【结论】复杂先心病病变类型与心脏位置有一定联系。左旋心、孤立左位心常系最复杂心内畸形。

The Analysis of Pathologic Change in Complex-CHD Correlated with the Positional Anomalies of the Heart

【Objective】 To study the pathologic change of complex CHD and therelationship between complex CHD with the positional anomalies of the heart. 【Method】 From Sep., 1986 to 2000 Feb., 81 cases of complex CHD in our hospital were accumulated. The pathologic change correlated with the various positional anomalies was analysed. 【Results】 In the incidence of complex CHD, there were no sex different distributions in normal or mirror image dextrocardia, but with significant differences in positional anomalies of the heart, male: female was 4∶1. The average age came to clinic was 4 years old, PA and HLHS usually as a emergency in neonate. The incidence of major pathologic change in complex CHD was: TGA 38%, SV 16%, common trunk 13%, TA 10%. 71% of complete TGA in normal or mirror image dextrocardia and 69% of correlated TGA happened in positional anomalies of the heart. The combined lesions in TGA was VSD and PS. Levoversion and isolated levocardia always accompanied with the most complex lesions, the number of defects from 2 to 8 (4.89±1.76, average). In our group, complex CHD was not uncommon in mirror dextrocardia. Two scarcely cases of TASVD found in this group.