胎儿左心室发育不良综合征并肺静脉共同腔闭锁尸检1例

Fetal left ventricular dysplasia syndrome and pulmonary venous common cavity atresia

目的探讨1例左心发育不良综合征并肺静脉共同腔闭锁胎儿的临床资料、超声分析及尸检解剖学特点。方法分析超声心动检查发现的复杂胎儿先天性心脏畸形病例,引产后进行尸体病理解剖,心、血管畸形按心脏节段分析方法进行分析和总结。结果胎儿左心发育不良综合征为复杂的先天性心脏畸形,同时合并肺静脉共同腔闭锁的病例更罕见,母体超声心动图可描述比较显著的解剖及生理特征,引产后详细的尸体解剖可进一步明确诊断。本例可见房间隔缺如、单心房、左心室腔小壁厚,二尖瓣闭锁及肺静脉共同腔闭锁,而且胎儿与婴幼儿的肺静脉共同腔闭锁不同,组织学不伴有肺淋巴管扩张。结论胎儿左心发育不良综合征合并肺静脉共同腔闭锁为少见的先天性心血管畸形,诊断更加困难。

Objective To investigate the clinical data, ultrasound analysis and pathologic manifestations of hypoplastie left heart syndrome and atresia of common pulmonary vein （ ACPV ） of fetus. Methods Fetal congenital cardiovascular malformations found by echocardiography were studied and pathological examination of cardiovascular malformation by heart segment analysis method were carried out. Results Fetal left ventricular dysplasia syndrome was complex congenital cardiac malformations, and merging with combined pulmonary venous occlusion was rare. The ultrasonic eehocardiography could significantly describe anatomical and physiological characteristics, and detailed autopsy could be further clarified. Absence of atrial septum, left ventrieular chamber, mitral valve attsia and atresia of common pulmonary vein were found in this cases. It was different from infant that the fetus was not associated with pulmonary lymphangiectasis. Conclusion Fetal left ventricular dysplasia syndrome with ACPV is an extremely rare congenital heart disease. It is even more difficult to make such a diagnosis.