左心发育不良综合征的鉴别诊断与预后分析

发布时间：2018-10-14 12:38

【摘要】：目的通过对比分析胎儿左心偏小病例,探讨胎儿左心发育不良综合征(HLHS)的超声特点,进行准确诊断与鉴别诊断,为进行合理预后咨询提供依据。方法对产前超声心动图检出的280例左心偏小的超声图像进行回顾性分析,与尸检或出生后超声结果对比分析其预后特点,并对其病因进行鉴别。结果 280例左心偏小病例中,HLHS 26例,主动脉缩窄95例,卵圆孔血流受限30例,肺静脉畸形引流6例,右室双出口51例,永存左上腔静脉32例,生理性右心优势13例,非对称性房室间隔缺损27例。HLHS与其他左心偏小疾病的主要鉴别点为,二尖瓣及主动脉瓣狭窄或闭锁;左心可小也可球形扩张,但左心功能减低,心尖由右室构成。结论产前超声心动图早期诊断HLHS,并与其他引起左心偏小的疾病进行鉴别,对其预后判断及胎儿围产期管理意义重大。
[Abstract]:Objective to investigate the ultrasonographic characteristics of fetal left ventricular dysplasia syndrome (HLHS) by comparing and analyzing the fetal left ventricular small cases, and to make accurate diagnosis and differential diagnosis, so as to provide the basis for reasonable prognosis consultation. Methods the echocardiographic findings of 280 cases with small left heart were analyzed retrospectively. The prognostic features were compared with those of postnatal or postmortem echocardiography, and the etiology was distinguished. Results there were 26 cases of HLHS, 95 cases of coarctation of aorta, 30 cases of blood flow limitation of foramen ovale, 6 cases of anomalous drainage of pulmonary vein, 51 cases of double outlet of right ventricle, 32 cases of persistent left superior vena cava and 13 cases of physiologic right ventricular dominance. There were 27 cases of asymmetric atrioventricular septal defect. The main differentiating points between HLHS and other diseases were mitral valve and aortic valve stenosis or atresia, left heart could be small or spherical dilatation, but left ventricular function was decreased, and the apex of heart was composed of right ventricle. Conclusion early diagnosis of HLHS, by prenatal echocardiography and differential diagnosis with other diseases which cause small left heart are of great significance for prognosis judgment and fetal perinatal management.
【作者单位】：首都医科大学附属北京安贞医院超声科胎儿心脏病母胎医学实验室;
【基金】：胎儿心脏病母胎医学北京市重点实验室(No.BZ0308) 教育部重点实验室(No.PXW2014_014226_000012) 国家科技支撑计划(No.2014BAI08B07)
【分类号】：R714.5;R540.45

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