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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 of 1 fetus with hypoplastic left heart syndrome with pulmonary venous common cavity occlusion and its characteristics of autopsy. Methods The cases of complicated congenital heart malformations detected by echocardiography were analyzed. Pathological anatomy was performed after induced labor. Cardiac and vascular malformations were analyzed and summarized according to the method of heart segment analysis. Results Fetal hypoplasia syndrome was complicated congenital heart malformation. In the meantime, cases with pulmonary venous common cavity atresia were more rare. Maternal echocardiography could describe more significant anatomic and physiological features. Detailed postmortem cadaveric anatomy could be further clarified diagnosis. This example shows the absence of atrial septum, single atrium, left ventricular cavity wall thickness, mitral valve atresia and pulmonary vein common cavity atresia, and fetus and infants with pulmonary vein common cavity atresia different, histology is not associated with pulmonary lymphatic dilatation. Conclusion Fetal left ventricular dysplasia syndrome with pulmonary venous common cavity atresia rare congenital cardiovascular malformations, the diagnosis more difficult.