产前超声心动图诊断胎儿左心发育不良综合征

The diagnosis of prenatal echocardiography in fetal left ventricular dysplasia syndrome

目的探讨产前超声心动图诊断胎儿左心发育不良综合征(HLHS)的技术要点。方法对产前超声检出的25例HLHS胎儿的临床及超声资料进行回顾性分析,重点探讨合并房间隔异常的HLHS胎儿超声声像图改变及血流动力学变化特点,并与尸检和出生后超声检查结果进行对比分析。结果25例HLHS胎儿中7例为二尖瓣及主动脉瓣闭锁,左心室重度发育不良;16例为二尖瓣闭锁合并不同程度主动脉瓣狭窄及主动脉缩窄,其中2例主动脉瓣狭窄伴主动脉缩窄随孕周进展其发育不良的左心室心内膜回声增强及左心室收缩力明显降低;另2例二尖瓣增厚和瓣膜开启运动受限伴主动脉瓣狭窄及主动脉弓缩窄。25例中7例伴房间隔完整,超声示房间隔增厚及卵园孔不开放,多普勒超声示房水平无分流信号,另外18例有房水平左向右少量分流;25例均见动脉导管逆向血流信号。结论产前超声心动图可早期诊断胎儿HLHS,除检出导致左心室发育不良系列左心系统病变外,房间隔完整或卵园孔径小及房水平的左向右分流也是评价HLHS预后及出生后手术治疗的重要指征。

Objective To investigate the diagnosis technology in fetal left ventricular dysplasia syndrome （HLHS） by using fetal echocardiograms （Echo）. Methods Twenty-five cases of fetal left ventricular dysplasia syndrome （HLHS） diagnosed by prenatal Echo findings were analysed and the echo diagnosis technology, characteristics of＇ changes in hemodynamics were assessed. Results Seven of 25 cases with serious HLHS had aortic and mitral valve atresia, serious left ventricular dysplasia. Sixteen cases were mitral valve atresia with varied degrees of aOrtic stenosis and aortic arch coarctation including two cases of thickened mitral valve and limited movement for aortic stenosis and aortic arch coarctation. Seven cases of 25 cases had intact atrial septum. The ultrasound showed closed thickening of the foramen ovale. The Doppler ultrasound showed intact atrial septum without blood streaming. The left-to-right shunt was observed at the level of the rest in the 18 cases. The refluence into the transverse srch and ascending aorta was shown from the ductus arteriosus. Conclusions The fetal echocardiographic studies should be performed every 16 week until delivery for monitoring intrauterine growth retardation, ventricular dysfunction, growth rate of the ventricle and its associated structures, and vacular regurgitation The flow pattern across the foramen oval should also be assessed. The intact atrial septnm or small foramen oval is important indications for surgery after birth and prognosis.