胎儿右心发育不良综合征超声影像学及病理学研究

Characteristics of echocardiographic imaging and pathology in fetuses with hypoplastic right heart syndrome

目的探讨右心发育不良综合征在胎儿时期的超声影像学及病理学特征。方法选取胎儿右心发育不良综合征引产标本进行病理形态学检查；取右室游离壁心肌、肺动脉及肺动脉圆锥材料进行病理组织学检测；回顾性分析总结产前超声心动图影像学资料。结果孕24～38周右心发育不良综合征胎儿心脏标本10例，其右室横径、右室上下径及肺动脉内径测值均较对照组偏小且差异有统计学意义（P〈0．05），三尖瓣反流面积和反流速度均显著大于对照组，差异有统计学意义（P〈O．05）。组织切片染色后光镜下检测结果显示，右室心肌纤维化发生率100％。产前超声心动图诊断符合率为l％。结论产前超声心动图对诊断胎儿右心发育不良综合征具有较高的准确率，右心发育不良综合扯胎儿心脏除形态学明显异常外，右室心肌内部、肺动脉和肺动脉圆锥亦存在显著的组织学结构异常。

Objective To detect the echocardiographic and pathologic characteristics of hypoplastic right heart syndrome （HRHS） during fetal period. Methods The pathomorphology of fetal heart specimens with HRHS was detected, and the sections of the right ventricle, pulmonary artery and pulmonary conus were studied by light microscopy. Prenatal echocardiographic images were analysed retrospectively. Results Ten formalin-fixed necropsy heart specimens with HRHS were selected. Among the specimens, transverse diameter and vertical diameter of right ventricular and pulmonary artery measured values was smaller than those of control group, the difference was statistically significant（ P d0.05）, tricuspid regurgitation area and regurgitation velocity was significantly greater than that in control group and the difference was statistically significant（ P d0.05）. Histological change of right ventricular myocardial fibrosis was present in 100%. The coincidence of prenatal echocardiographic diagnosis was 100%. Conclusions There is higher accuracy of prenatal echocardiography in diagnosing fetal HRHS. Marked histological abnormalities in right ventricular myocardium,pulmonary artery and pulmonary conus with HRHS exist excepting morphologic abnormalities.