产前超声诊断胎儿孤立完全性肺静脉异位连接

目的探讨本中心32例胎儿孤立完全性肺静脉异位连接(TAPVC)产前超声的主要特征。方法选择我院经产前超声心动图检出孤立TAPVC病例32例,经出生后检查或尸检证实诊断,回顾性分析超声检查的图像和结果。结果本研究胎儿共诊断TAPVC 75例,孤立TAPVC 35例。单发病例诊断时孕周为22~38周,平均(27.5±4.5)周,年龄为21~38岁,平均(28.0±4.0)岁,其中引产23例,12例出生。出生后证实诊断的9例,假阳性3例。出生的9例TAPVC病例8例在新生儿期手术治疗,平均手术年龄8.6d,平均随访40.6个月(18~75个月)。引产病例由尸检证实诊断。总结可作为超声诊断特征的直接征象和间接征象:包括肺静脉共同腔、肺静脉回流完整途径(是否存在梗阻)、右心优势、左房后壁-降主动脉的距离、引流入血管(腔静脉和冠状窦)扩张等。结论胎儿孤立TAPVC产前超声具有特征性表现。产前系统检查可对TAPVC的分型及梗阻做出准确的判断,为孕妇制定最佳的生产计划和手术方案。

Prenatal Ultrasound Diagnosis of Isolated Total Anomalous Pulmonary Venous Connection

Objective To explore the echocardiographic features of fetal TAPVC in order to assess accuracy of prenatal diagnosis.Methods 35 fetuses with suspected isolated TAPVC were observed between at Guangdong Cardiovascular Institute.Fetal echocardiographic images,confirmed by pathology or postnatal assessment,were reviewed.We systematically analyzed echocardiographic features.Results 35 fetuses were diagnosed with suspected isolated TAPVC at a mean gestational age of(27.5±4.5)weeks(range from 22 to 38 weeks).23 cases were terminated and their diagnosis was confirmed by autopsy.12 cases were born and 9 of them were diagnosed with TAPVC by postnatal assessment,whereas the rest were considered as false-positive cases.The direct and indirect signs of ultrasonography were summarized:presence of a pulmonary venous confluence behind the left atrium or a vertical vein,the complete pulmonary venous return pathway(obstructive or not),ventricular disproportion,the left atrium-descending aorta distance and Doppler flow patterns.Conclusions Fetal echocardiography permits prenatal diagnosis of isolated TAPVC.Leading sonographic features were very useful for the estimate of subtype and obstruction site.