摘要
左心发育不良综合征是二尖瓣和(或)主动脉瓣狭窄或闭锁, 导致左心室、升主动脉和主动脉弓发育不良为特征的先天性心脏病, 如不及时治疗, 患儿多在出生后几周内死亡。先天性完全性房室传导阻滞是一种罕见的先天性心脏传导异常疾病, 如合并严重的心脏畸形, 死亡率极高。本例孕妇, 26岁, 孕3产1, 自然受孕, 单绒毛膜双羊膜囊双胞胎, 孕22+5周时通过胎儿超声心动图检查首次发现双胎之一患有左心发育不良综合征合并先天性完全性房室传导阻滞, 这种情况极为罕见, 现有的研究缺乏如何管理妊娠及确定分娩时间的临床指南。本例产前超声通过脑胎盘血流比值(CPR)联合胎儿心血管整体评分(CVPS)监测及综合评估胎儿心功能, 进行妊娠安全管理并择期行剖宫产术, 另一健康胎儿未受到不良影响, 本例为保证围生期健康胎儿及母亲安全提供了有力证据, 也证实两项指标联合评估胎儿心功能及妊娠结局更准确及全面, 此方法简便、准确、无创, 可以成为临床评估胎儿心功能的一个有效手段。
Hypoplastic left heart syndrome is a congenital heart disease characterized by mitral and(or)aortic valve stenosis or atresia,leading to the hypoplasia of the left ventricle,ascending aorta and aortic arch.If not treated in time,most children die within a few weeks after birth.Congenital complete atrioventricular block is a rare congenital cardiac conduction disorder.When combined with severe cardiac malformations,the mortality rate is extremely high.In this case,a 26-year-old pregnant woman with a history of 3 pregnancies and 1 delivery,had a natural conception with monochorionic diamniotic twins.At 22+5 weeks of gestation,it was first found that one of the twins had hypoplastic left heart syndrome with congenital complete atrioventricular block through fetal echocardiography detection.This situation was extremely rare,and there was a lack of clinical guidelines on how to manage the pregnancy and determine the time of delivery time.In this case,prenatal ultrasound was used to monitor and comprehensively evaluate fetal cardiac function through the cerebroplacental ratio(CPR)combined with the cardiovascular overall performance score(CVPS).Pregnancy safety management was implemented,and a cesarean section was performed at an elective time.Another healthy fetus did not suffer any adverse effects.This case provides strong evidence for ensuring the safety of healthy fetus and mother in perinatal period.It is also confirmed that CPR and CVPS combined evaluation of fetal cardiac function and pregnancy outcome will be more accurate and comprehensive.This method is simple,accurate,non-invasive,and can be used as an effective method for clinical evaluation of fetal cardiac function.
作者
付小田
赵胜
成晨
张莉
伍玉晗
黄文理
刘福
Fu Xiaotian;Zhao Sheng;Cheng Chen;Zhang Li;Wu Yuhan;Huang Wenli;Liu Fu(Department of Ultrasound Diagnosis,Maternal and Child Health Hospital of Hubei Province,Wuhan 430070,China)
出处
《中华超声影像学杂志》
北大核心
2025年第7期633-636,共4页
Chinese Journal of Ultrasonography
基金
国家自然科学基金(82302085)
湖北省卫生健康委员会重点项目(WJ2023Z007)。
