胎儿左、右侧异构综合征产前超声特征的对比研究

Comparison of the prenatal ultrasonic features between left and right isomerism

目的对照病理解剖结果，比较胎儿左侧与右侧异构综合征声像图特征的差异。方法选择产前超声诊断并经病理解剖证实的胎儿异构综合征病例22例，分析比较左、右侧异构综合征的畸形特征及超声图像的差异。结果22例胎儿异构综合征中右侧异构18例，左侧异构4例。所有病例均有复杂的心血管畸形及内脏异构。18例右侧异构综合征的主要超声、病理表现为：右房同形异构（17例，94％），内脏异构（17例，94％），肺静脉异位引流（16例，89％），房室共同通道（15例，83％），下腔静脉与主动脉同侧并行（12例，67%），持续性左上腔静脉（11例，61％），右位主动脉弓（10例，56％），功能性单心室（10例，56％）。4例左侧异构综合征的超声、病理表现为：全部有左房同形异构、内脏异构及下腔静脉离断，3例并房～室传导阻滞、主动脉发育不良及房室共同通道，2例功能性单心室。结论内脏异构、房室共同通道、功能性单心室为胎儿左、右侧异构综合征的共同特征，右侧异构综合征多合并肺静脉异位引流、右位主动脉弓、下腔静脉与主动脉同侧并行，左侧异构综合征则以下腔静脉离断、房一室传导阻滞及主动脉发育不良为主要合并声像图表现。

Objective To compare the prenatal ultrasonic characteristics between left and right isomerism as referring to autopsy outcomes. Methods Between November 2007 and July 2010,fetuses with isomerism that were confirmed by autopsy, were identified from 1200 prenatal ultrasonic scans. Their abnormal spectrums and ultrasonic features were analyzed and comparied between left and right isomerism. Results A total of 18 fetuses with right isomerism and 4 fetuses with left isomerism were detected and confirmed. The major findings of the right isomerism were;viscerocardiac heterotaxy （17,94 % ）, anomalous pulmonary venous connection （ 16,89 % ）, complete atrioventricular septal defect （ 15,83 % ） ,juxtaposition of the descending aorta and inferior vena cava （ 12,67 % ）, left persistent superior vena cava （ 11,61% ）, right aortic arch （10, 56%） and univentricle （10, 56%）. As for the left isomerism, all had viscerocardiac heterotaxy and interruption of inferior vena cave, three of them had heart block, complete atrioventrieular septal defect and hypoplasia of aorta,two of them had univentricle. Conclusions Viscerocardiac heterotaxy, complete atrioventricular septal defect, univentricle are the most common anomalies detected. Right isomerism cases usually exist with anomalous pulmonary venous connection,juxtaposition of the descending aorta and inferior vena eava as well as right aortic arch. The left isomerism cases usually have interruption of inferior vena cave,heart block and hypoplasia of aorta.