产前超声心动图诊断胎儿左心室发育不良综合征

Prenatal echocardiography in diagnosis of fetal hypoplastic left heart syndrome

目的探讨胎儿超声心动图诊断左心室发育不良综合征(HLHS)的临床价值。方法对18胎HLHS胎儿的临床资料进行回顾性研究,分析其超声心动图特征。结果 18胎HLHS胎儿的超声心动图结果:①心尖四腔心切面:左心室小、心室发育不良。8胎二尖瓣及主动脉瓣闭锁,且左心室重度发育不良;5胎二尖瓣闭锁合并主动脉瓣狭窄及主动脉缩窄;3胎二尖瓣狭窄伴主动脉瓣狭窄及主动脉弓缩窄;2胎二尖瓣狭窄伴主动脉瓣闭锁及主动脉弓缩窄。②三血管切面:主动脉内径明显小于肺动脉,主动脉与肺动脉内径比值为0.25～0.40,平均0.38±0.18,主动脉横弓与动脉导管、肺动脉血流方向相反。③胸骨旁四腔心切面:6胎房间隔增厚,未显示房间隔穿隔血流。12胎胎儿卵圆孔径偏小、显示房间隔左向右分流。结论胎儿超声心动图可早期诊断胎儿HLHS,结合染色体检查对围生期的监测和临床处理有重要意义。

Objective To observe the clinical value of fetal echocardiography in the diagnosis of fetal hypoplastic left heart syndrome （HLHS）. Methods Echocardiographic features and clinical data of 18 fetuses prenatally diagnosed with HLHS were analyzed retrospectively. Results The findings of 18 fetal echocardiogram were as followed： ①Eight fetuses were found with aortic and mitral valve atresia and severe left ventricular dysplasia,5 with mitral valve atresia and various degrees of aortic stenosis as well as aortic arch coarctation,3 with thickened mitral valve,aortic stenosis and aortic arch coarctation,while 2 with thickened mitral valve,aortic atresia atreasia and aortic arch coarctation. ②The diameter of aorta was smaller than that of pulmonary artery. The ratio of AO/PA was 0.25—0.40 （0.38±0.18）. There was reverse flow from ductus artery to the aortic arch and ascend aortic. ③Intact atrial septum and thickeness appeared in 6 fetuses. No blood shunt was detected in intact atrial septum. Other 12 fetuses presented few left to right shunts. Conclusion Fetal echocardiography may serve as a reliable methods for early diagnosis of fetal HLHS. It is important for the detection during the parental period and the guidance of the clinical treatment.