Prenatal Ultrasound Observation of Persistent Left Superior Vena Cava with Intracardiac Malformation
-
摘要:
目的 探讨胎儿永存左上腔静脉(PLSVC)合并心内畸形类型及妊娠演变。 方法 基于云南大学附属医院影像采集及病例系统中90例永存左上腔静脉胎儿病例资料,回顾分析胎儿合并的心内、外畸形类型,妊娠结局。 结果 (1)76例(84.4%)胎儿在22~24周系统超声检查时诊断PLSVC,14例(15.6%)胎儿在晚孕期发现;(2)90例胎儿中,合并心内异常26例(28.9%),合并心外异常21例(23.3%),未合并异常50例(55.5%);(3)心内异常以主动脉弓缩窄或弓发育不良最常见(34.6%),其次为室间隔缺损(26.9%),心外异常以单脐动脉最常见(15.6%)。 结论 永存左上腔静脉与先天性心脏病关系密切;对于发现PLSVC的胎儿,孕期加强心功能观测;产前超声筛查在四腔及三血管切面基础上增加无名静脉切面,提高PLSVC检出率。 Abstract:Objective To investigate the type and pregnancy evolution of persistent fetal left superior vena cava(PLSVC) complicated with intracardiac malformations. Methods The data of 90 fetal cases with persistent left superior vena cava were collected via imaging and patients’ case system of the Affiliated Hospital of Yunnan University. The types of congenital and external malformations and pregnancy outcomes of the fetus were retrospectively analyzed. Result PLSVC was diagnosed in 76(84.4%) fetuses at 22-24 weeks of systemic ultrasonography, and 14(15.6%) fetuses were found in late pregnancy. Among the 90 fetuses, 26(28.9%) had combined intracardiac anomalies, 21(23.3%) had combined extracardiac anomalies, and 50(55.5%) had uncomplicated anomalies. The most common intracardiac anomaly was aortic arch narrowing or arch dysplasia(34.6%), followed by ventricular septal defect(26.9%), and the most common extracardiac anomaly was single umbilical artery. Conclusion The persistent left superior vena cava is closely related to congenital heart disease. For fetuses with PLSVC, observation of cardiac function should be strengthened during pregnancy. In prenatal ultrasound screening, the section of innominate vein should be added to the section of four cavities and three vessels to improve the detection rate of PLSVC. -
图 1 合并心内异常的产前超声图像
A:主动脉缩窄;B:室间隔缺损;C:左心发育不良;D:右侧锁骨下动脉迷走,右锁骨下动脉起源于降主动脉起始段,绕过气管后方朝向右肩走行;E:心内膜垫缺损,房间隔下部与室间隔上部可见连续中断;F、G:右位主动脉弓并右位动脉导管,主动脉弓及动脉导管位于气管右侧,主动脉弓分支顺序为左头臂干、右颈总动脉及右锁骨下动脉,呈“镜像”;H:永存动脉干,心底仅可见一组瓣膜,连接粗大动脉骑跨室间隔上;I:右室双出口,肺动脉和主动脉均起源于右心室;J:肺静脉异位引流,右上肺静脉流入右心房;K:肺动脉狭窄,肺动脉内径仅2.2 mm;LA左心房;LV左心室;RA右心房;RV右心室;DAO降主动脉;ARSA迷走右锁骨下动脉;T气管;DA动脉导管;ARCH主动脉弓;IVC下腔静脉;AO主动脉;LINA左头臂干;RCCA右颈总动脉;RSA右锁骨下动脉;RSPV右上肺静脉。
Figure 1. Prenatal ultrasound images of combined intracardiac abnormality
表 1 PLSVC合并心内畸形情况[n(%)]
Table 1. Combined intracardiac malformations in PLSVC [n (%)]
合并心内异常类型 例数 主动脉弓缩窄或主动脉发育不良 9 (34.6) 室间隔缺损 7(26.9) 左心发育不良 5(19.2) 右侧锁骨下动脉迷走 4(15.4) 心内膜垫缺失 2(7.7) 右位动脉弓 2(7.7) 右室双出口 2(7.7) 肺静脉异位引流 2(7.7) 永存动脉干 1(3.8) 肺动脉狭窄 1(3.8) 表 2 90例胎儿诊断孕周情况[n(%)]
Table 2. Diagnostic gestational weeks of 90 fetuses [n (%)]
项目 A组 B组 合计 ≤24周 36(90.0) 40(80.0) 76(84.4) >24周 4(10.0) 10(20.0) 14(15.6) 合计 40(100.0) 50(100.0) 90(100.0) 表 3 26例合并心内异常胎儿的产前超声及妊娠结局
Table 3. Prenatal utrasound and pregnancy outcome in 26 fetuses with combined intracardiac abnormality
病例 孕妇年龄(岁) 孕周(周) 心内异常 心外异常 结局 1 30 23+6 主动脉弓缩窄,中位心 左侧鼻骨缺失,右侧鼻骨短,单脐动脉 新生儿存活 2 23 23+6 主动脉弓缩窄,动脉导管迂曲扩张 单脐动脉 新生儿死亡 3 37 21+3 主动脉弓缩窄,主肺比失调,左心小,右位主动脉弓伴右位动脉导管 右肾缺如,单脐动脉 引产 4 41 22+2 主动脉弓缩窄,左心稍小,室间隔缺损 ,右室双出口 引产 5 28 23+4 主肺比失调(主动脉内径偏细),左右心比例失调(左心偏小),右室壁稍厚,心包少量积液 胎儿宫内死亡 6 30 21+3 主动脉峡部偏细,室间隔小缺损 新生儿存活 7 22 22+3 主动脉弓缩窄 唇腭裂 引产 8 26 21+5 主肺比失调(主动脉内径偏细) 新生儿存活 9 30 21+3 主动脉弓发育不良,左心小,卵圆孔开放受限 引产 10 40 23+4 室间隔缺损,永存动脉干畸形 引产 11 30 21+3 右室双出口,室间隔缺损 小下颌,颅脑结构异常(小脑蚓部发育不良 中帆腔扩张),右侧鼻骨缺失 引产 12 37 22+2 室间隔缺损 新生儿存活 13 28 23+4 室间隔缺损,主动脉骑跨 新生儿存活 14 16 24+0 室间隔缺损 右侧侧脑室增宽 新生儿存活 15 28 23+4 左心稍小,右侧锁骨下动脉迷走 新生儿存活 16 27 22+3 右侧锁骨下动脉迷走 单脐动脉 新生儿存活 17 45 20+3 右侧锁骨下动脉迷走 单脐动脉,FL小于M-2SD 新生儿存活 18 27 22+3 右侧锁骨下动脉迷走 盆腔异位肾,双肾测值小 引产 19 31 30+3 右房内隔膜回声(考虑部分型肺静脉异位引流) 新生儿存活 20 28 23+4 胎儿右房隔膜样回声(考虑肺静脉异位引流) 新生儿存活 21 42 23+6 心内膜垫缺损、大动脉转位、动脉导管畸形,肺动脉狭窄 小下颌 引产 22 20 22+5 心内膜垫缺损 引产 23 43 23+3 左室强光点、三尖瓣轻-中度返流 新生儿存活 24 31 23+1 右位主动脉弓 新生儿存活 25 27 22+0 冠状静脉窦显著扩张,左室强光点 新生儿存活 26 25 22+0 肺主动脉比例失调(肺动脉内径稍宽) 新生儿存活 -
[1] 李胜利, 罗国阳 主编. 胎儿畸形产前超声诊断学[M]. 北京: 科学出版社, 2017: 333-334. [2] Chaoui R,Heling K-S,Karl K. Ultrasound of the fetal veins part 2: Veins at the cardiac level[J]. Ultraschall in der Medizin (Stuttgart,Germany,1980),2014,35(4):302-321. [3] Galindo A,Gutiérrez-Larraya F,Escribano D,et al. Clinical significance of persistent left superior vena cava diagnosed in fetal life.[J]. Ultrasound in Obstetrics & Gynecology,2007,30(2):152-161. [4] Gustapane S,Leombroni M,Khalil A,et al. Systematic review and meta-analysis of persistent left superior vena cava on prenatal ultrasound: associated anomalies,diagnostic accuracy and postnatal outcome.[J]. Ultrasound in Obstetrics & Gynecology,2016,48(6):701-708. [5] Yonehara K,Terada K,Morine M. Prenatal Diagnosis of Persistent Left Superior Vena Cava Raises Suspicion for Coarctation of Aorta[J]. Cureus,2022,14(10):e30220. [6] Agnoletti G,Annecchino F,Preda L,et al. Persistence of the left superior caval vein: can it potentiate obstructive lesions of the left ventricle?[J]. Cardiology in the Young,1999,9(3):285-290. doi: 10.1017/S1047951100004959 [7] Kele A,Ylmaz O,Gülah Dadeviren,et al. Persistent left superior vena cava: Why is prenatal diagnosis important?[J]. Fetal and Pediatric Pathology,2022,41(4):592-602. doi: 10.1080/15513815.2021.1933662 [8] Cao Q,Zhen L,Pan M,et al. Prenatal persistent left superior vena cava in low population: Not a benign vascular anomaly[J]. Taiwan residents Journal of Obstetrics & Gynecology,2022,61(3):459-463. [9] Rein A J,Nir A,Nadjari M. The coronary sinus in the fetus.[J]. Ultrasound Obstet Gynecol,2000,15(6):468-472. doi: 10.1046/j.1469-0705.2000.00142.x [10] Ma Mingming,Tan Yan,Chen Ran,et al. Comparison of coronary sinus diameter Z-scores in normal fetuses and fetuses with persistent left superior vena cava (PLSVC).[J]. The International Journal of Cardiovascular Imaging,2018,34(2):152-161. [11] Yokoyama Utako,Ichikawa Yasuhiro,Minamisawa Susumu,et al. Pathology and molecular mechanisms of coarctation of the aorta and its association with the ductus arteriosus.[J]. The Journal of Physiological Sciences,2017,67(2):259-270. doi: 10.1007/s12576-016-0512-x [12] Xu Ran,Zhou Dan,Liu Yushan,et al. Impaired Elastic Properties of the Ascending Aorta in Fetuses With Coarctation of the Aorta.[J]. Journal of the American Heart Association,2023,12(2):e028015. doi: 10.1161/JAHA.122.028015 [13] 朱昱霏,罗红,温激翔,孔丽娜,张琼月,杨琳. 冠状静脉及左无名静脉联合三血管气管切面在孕中期胎儿永存左上腔静脉诊断中的价值[J]. 实用妇产科杂志,2021,37(09):716-718. [14] Tuo Giulia,Paladini Dario,Marasini Lucia,et al. Fetal aortic coarctation: A combination of third-trimester echocardiographic parameters to improve the prediction of postnatal outcome[J]. Frontiers in Pediatrics,2022,10:866994. doi: 10.3389/fped.2022.866994