胎儿冠状静脉窦扩张合并心内外畸形的超声表现

目的探讨胎儿冠状静脉窦扩张及其合并心内外畸形的产前超声诊断特点。方法回顾性分析我院经超声诊断的202例冠状静脉窦扩张胎儿,并与出生后随访或引产后病理解剖结果进行对照分析。结果 202例冠状静脉窦扩张胎儿中,合并永存左上腔静脉194例(96.0%),肺静脉异位引流4例,无顶冠状静脉窦1例,同时合并永存左上腔静脉及肺静脉异位引流1例,余2例为单纯冠状静脉窦扩张。其中合并其他畸形78例(38.6%),包括永存左上腔静脉合并其他心内畸形52例,合并心外畸形17例,合并心内外畸形7例;肺静脉异位引流合并其他心内畸形1例;无顶冠状静脉窦合并其他心内畸形1例。结论永存左上腔静脉是胎儿冠状静脉窦扩张的主要原因,其中室间隔缺损、肺动脉狭窄及法洛四联症是其最常见的并发心内畸形。产前超声在胎儿冠状静脉窦扩张及合并心内外畸形鉴别诊断中具有重要意义。     

时间-空间关联成像联合B-flow在胎儿永存左上腔静脉产前超声诊断中的应用价值

目的探讨时间-空间关联成像(STIC)联合B-flow在胎儿永存左上腔静脉产前超声诊断中的应用价值。方法对产前胎儿常规二维超声心动图可疑或明确心脏畸形的132例胎儿进行B-flow显像及STIC数据采集,观察有无左上腔静脉,并将产前超声诊断结果与产后新生儿超声心动图及引产后胎儿尸体解剖病理检查结果进行对比分析。结果 132例胎儿中120例在B-flow显像模式下STIC数据采集成功,采集成功率为90.9%(120/132);120例STIC数据采集成功的胎儿中,常规二维超声心动图诊断永存左上腔静脉15例(15/20)。B-flow-STIC诊断永存左上腔静脉20例。其中5例伴无名静脉缺如,4例合并心脏严重复杂畸形(2例合并完全性房室间隔缺损,1例合并三尖瓣发育不良,1例合并右心室双出口,1例合并右位主动脉弓,5例合并室间隔缺损),6例为单纯性永存左上腔静脉。B-flow-STIC修正常规二维超声心动图诊断5例;其中2例常规二维超声心动图诊断为房间隔缺损,1例常规二维超声心动图诊断为完全性房室间隔缺损,1例常规二维超声心动图诊断为心内型肺静脉异位引流,均经B-flow-STIC修正诊断为永存左上腔静脉;1例常规二维超声心动图漏诊,B-flow-STIC诊断为永存左上腔静脉引流至左心房。结论 STIC联合B-flow可清晰显示胎儿永存左上腔静脉及其空间位置关系,有利于提高永存左上腔静脉产前超声诊断准确性。     

左无名静脉在产前超声筛查胎儿永存左上腔静脉中的价值

目的 探讨胎儿左无名静脉对产前超声检测胎儿永存左上腔静脉的价值.方法 首先对胎儿进行系统的产前超声筛查,着重观察胎儿三血管气管切面,观察在肺动脉左侧有无异常的血管存在.随后再将探头向胎儿头侧稍稍侧动,观察有无左无名静脉存在,并测量其内径及观察血流方向.结果 100例正常胎儿均存在左无名静脉,内径为(2.56±0.48) mm,血流方向均由左流向右,汇入右上腔静脉.44例永存左上腔静脉时左无名静脉均缺如,而3例完全性心上型肺静脉异位引流病例均存在左无名静脉,内径为(2.77±0.35) mm,其血流方向与正常胎儿一样,由左向右流入右上腔静脉.结论 左无名静脉缺失对产前超声检测永存左上腔静脉具有重要的意义,能提高永存左上腔静脉的检出率,并为鉴别诊断永存左上腔静脉和心上型完全性肺静脉异位引流提供重要的价值.     

基层医院产前胎儿心脏超声筛查漏诊情况分析

目的探讨深圳地区基层医院产前胎儿心脏超声筛查常见漏诊疾病及原因。方法对我院在基层医院初诊中漏诊胎儿心脏畸形的会诊病例103例以四腔心、左右心室流出道和三血管气管切面为基本筛查切面进行产前胎儿心脏超声筛查,分析深圳地区基层医院胎儿心脏畸形的常见漏诊情况。结果本组四腔心切面遗漏左位上腔静脉33例,完全性肺静脉异位回流11例;左右心室流出道切面漏诊12例法洛四联征,6例永存动脉干,5例右室双出口,5例肺动脉瓣闭锁;三血管气管切面漏诊迷走锁骨下动脉20例,主动脉弓离断7例,主动脉弓缩窄4例。结论各个筛查标准切面细节观察不足是深圳地区基层医院漏诊胎儿心脏畸形的主要原因。     

胎儿心脏超声筛查常见漏误诊分析及解决方案

正随着产前超声医学的发展,许多胎儿心脏畸形能在孕期筛查出来。以往,仅筛查四腔心切面,理论上能筛查出约50%以上的严重心脏畸形,目前增加流出道切面及三血管气管切面后可以筛查出约90%的心脏畸形[1]。但仍有一部分心脏畸形如肺静脉异位引流、肺动脉分支异常、主动脉弓缩窄或离断、房室间隔缺损、室间隔缺损、不典型法洛四联症、完全型大动脉转位、半月瓣狭窄、横纹肌瘤等结构性畸形易发生漏诊及误诊。     

超声心动图在筛查胎儿心脏畸形中的应用体会

目的探讨产前超声心动图筛查胎儿心脏畸形的必要性。方法 以胎儿四腔心、左室流出道、右室流出道、三血管-气管、主动脉弓、动脉导管弓这六个切面为标准切面,观察胎儿心脏结构、形态。结果 1863例胎儿中发现61例心脏畸形:室间隔缺损21例,法洛氏四联症10例,左心发育不良5例,永存左上腔静脉4例,右室双出口4例,心内膜垫缺损3例,右心发育不良3例,单心腔2例,三尖瓣下移畸形2例,主动脉弓离断2例,肺动脉狭窄2例,永存动脉干2例,右位主动脉弓伴血管环1例。漏诊室间隔缺损3例,肺动脉瓣轻度狭窄1例;诊断符合率99.8%(1859/1863);敏感性为93.8%,特异性为100.0%。结论以上述6个切面为标准切面进行胎儿心脏超声筛查,可发现绝大多数的心脏畸形,特别是复杂畸形无1例漏诊,应逐渐将胎儿超声心动图检查纳入产前检查常规工作中并建立标准化扫查切面。     

胎儿冠状静脉窦扩张合并心内外畸形的的超声诊断特点

【】 目的：探讨胎儿冠状静脉窦扩张及其合并心内外畸形的产前超声诊断特点。 方法：回顾性分析2011年7月~2015年4月于广州市妇女儿童医疗中心诊断胎儿冠状静脉窦扩张病例,并与出生后随访或引产后病理解剖结果进行对照分析。 结果：共有202例胎儿冠状静脉窦扩张,其中合并永存左上腔静脉194例(194/202, 96%),合并肺静脉异位引流4例,合并无顶冠状静脉窦1例,同时合并永存左上腔静脉及肺静脉异位引流1例,余2例为单纯冠状静脉窦扩张。其中合并其他畸形78例(78/202, 38.6%)。其中永存左上腔静脉合并其他心内畸形52例,合并心外畸形17例,合并心内外畸形7例;肺静脉异位引流合并其他心内畸形1例;无顶冠状静脉窦合并其他心内畸形1例。室间隔缺损、肺动脉狭窄、法洛氏四联症是最常见的并发心内畸形,单脐动脉是最常见的并发心外异常。 结论：永存左上腔静脉是胎儿冠状静脉窦扩张的主要原因,其中室间隔缺损、肺动脉狭窄、法洛氏四联症是最常见的并发心内畸形。产前超声对胎儿冠状静脉窦扩张及合并心内外畸形进行鉴别诊断具有重要诊断意义。     

胎儿永存左上腔静脉的产前超声诊断

目的 探讨胎儿永存左上腔静脉的声像图特征及产前超声诊断胎儿永存左上腔静脉的价值.方法 对6例产前超声诊断为永存左上腔静脉胎儿的超声图像特征进行回顾性分析,并结合文献探讨产前超声诊断胎儿永存左上腔静脉的价值.结果 6例中1例为双胎之一,5例为单胎.1例为单纯性永存左上腔静脉,5例还合并其他心脏异常.1例合并染色体异常.结论 胎儿心脏超声三血管切面可准确诊断胎儿永存左上腔静脉.     

超声诊断胎儿永存左上腔静脉的探讨

目的：探讨胎儿永存左上腔静脉的超声检查方法及声像图特征。方法：对超声诊断的10例胎儿永存左上腔静脉进行回顾分析。结果：超声诊断的10例胎儿永存左上腔静脉均为Ⅰ型,其中2例合并室间隔缺损,1例合并永存动脉干及室间隔缺损。结论：3VT切面及连续追踪扫查法是诊断胎儿永存左上腔静脉较好的切面及方法。     

产前超声诊断胎儿永存左上腔静脉

目的探讨胎儿永存左上腔静脉的声像图特征及产前超声诊断胎儿永存左上腔静脉的价值。方法对8例产前超声诊断为永存左上腔静脉胎儿的超声图像特征进行回顾性分析,并结合文献分析总结胎儿永存左上腔静脉各切面声像图特点。结果 8例中1例合并完全性心内膜垫缺损,1例合并Dandy-Walker畸形,2例合并多系统畸形,其余4例为单纯性永存左上腔静脉。结论产前超声诊断胎儿永存左上腔静脉具有很好的可操作性和较高的临床价值。     

胎儿左、右侧异构综合征产前超声特征的对比研究

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-室传导阻滞及主动脉发育不良为主要合并声像图表现.

Abstract：

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 atrioventricular 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 cava as well as right aortic arch.The left isomerism cases usually have interruption of inferior vena cave,heart block and hypoplasia of aorta.     

Prenatal diagnosis of total anomalous pulmonary venous connection to the portal vein associated with right atrial isomerism.

We report the prenatal diagnosis of total anomalous pulmonary venous connection to the portal system in a 20-week fetus with right atrial isomerism. The apex of the fetal heart pointed to the left, the fetal stomach was on the right, there was a common atrioventricular valve, the left ventricle was small and the abdominal aorta and inferior vena cava were on the left side; all these features were suggestive of right atrial isomerism. An anomalous vein was connected to the portal vein which ascended above the diaphragm and ended in a confluence of pulmonary veins, posterior to the common atrium. Color Doppler imaging helped confirm the diagnosis of total anomalous pulmonary venous connection. The prenatal findings were confirmed on autopsy.     

产前超声诊断胎儿异构综合征 12 例分析

目的探讨异构综合征的产前超声表现。方法分析12例异构综合征胎儿的超声资料（部分胎儿引产后经尸检证实）。结果12例均存在共同的内脏异构及复杂的心脏畸形。8例右侧异构综合征主要超声表现为右房同侧异构,下腔静脉与主动脉同侧并行,内脏异构,肺静脉异位引流,房室共同通道,右位主动脉弓,功能性单心室;4例左侧异构综合征主要超声表现为：均存在左房同侧异构,内脏异构,下腔静脉离断,奇静脉异常连接,其中2例主动脉发育不良,1例功能性单心室,1例未表现出心脏其他畸形。结论产前超声可准确诊断异构综合征及其中的心脏复杂畸形。     

磁共振成像在心房异构诊断中的应用

目的：评价磁共振成像在心房异构（心脾综合征）中的应用价值。方法：MRI应用于88例心脾综合征检查中，平均年龄3.69岁（1月~15岁），扫描序列包括真稳态进动快速成像序列（FIESTA），双反转恢复序列（double IR），CE-MRA。所有患儿均行心脏超声心动图检查及心血管造影，72例心血管畸形经手术证实。结果：右房异构67例，左房异构21例。右房异构中均为复杂先天性心脏病，支气管均为对称的右支气管形态，双上腔静脉45例（64.3％），肝静脉分支单独连接心房17例，肺静脉异位引流23例，食道裂孔疝2例。左房异构均为对称左主支气管形态，下腔静脉中断经奇静脉延续16例（78.2％）。心外大血管异常诊断符合率100％。结论：MRI既可检出心内及心外大血管的各种复杂畸形，同时又可显示对复杂先天性心脏病病诊断有重要帮助的脾脏及气管的位置及形态，是心脾综合征的理想影像学诊断方法。     

复杂先心病合并体静脉畸形的二维及彩色多普勒超声诊断

目的：探讨复杂先天性心脏病合并常见体静脉畸形的超声特征，以便做出正确的诊断。方法：与结果：本文报道经二维和彩色多普勒诊断并经造影或手术证实69例复杂先天性心脏病合并体静脉畸形引流，其中部分患者为多发静脉畸形；69例中合并静脉畸形77例次，包括永存左上腔静脉(LSVC）47例次；左上腔静脉入左房15例次；合并奇静脉异常连接的下腔静脉(IVC)肾上段中断10例次和严重狭窄1例；左无名静脉走行异常4例次。结论：1．心房异构时，体静脉畸形发生率显菩高于心房正位．且异构的性质与体静脉畸形引流类型密切相关；2．为了正确诊断体静脉畸形引流，应进行多切面系统检查，尤其注意对剑下、胸骨上窝和胸骨旁高位切面的观察；3．剑下短轴切面见腹主动脉/下腔静脉与脊柱的对称关系消失，则提示下腔静脉异常；左胸骨旁高位与胸骨上窝切面多能直接显示左上腔静脉，同时左无名静脉非常细小或缺如。如果冠状静脉窦无扩张，则高度怀疑左上腔静脉入左房。左上肢静脉声学造影对左上腔静脉畸形的识别有重要意义。     

A case of prenatally detected left isomerism and hemiazygos continuation of inferior vena cava

Heterotaxy syndromes are defined as the disorders that involve abnormal arrangement of viscera. We present a case of prenatally diagnosed left isomerism in a 30‐year‐old primigravida woman referred to our hospital for complex cardiac abnormality. Sonographic findings included heart block, unbalanced atrioventricular septal defect, interruption of the inferior vena cava with hemiazygos continuation, double superior vena cava, a right‐sided stomach, and biliary atresia. The hemiazygos vein drained into the right atrium by the persistent left superior vena cava. This type of inferior vena cava interruption and continuation is rarely reported in prenatally detected cases of left isomerism. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 45 :430–433, 2017     

Prenatal diagnosis of cardiosplenic syndromes: a 10-year experience.

OBJECTIVE: To assess the accuracy of fetal echocardiography in the prenatal diagnosis of cardiosplenic syndromes and the spectrum of associated anomalies. METHODS: This was a retrospective survey of fetuses in our databases over a period of 10 years with postnatally confirmed prenatal diagnosis of cardiosplenic syndromes. RESULTS: In 32 of 35 fetuses the prenatal diagnosis of cardiosplenic syndromes was confirmed postpartum. Twenty-two fetuses had left isomerism. Their main prenatal ultrasound features were interrupted inferior vena cava (n = 21), complete atrioventricular septal defect (n = 15), viscerocardiac heterotaxy (n = 15), persistent bradyarrhythmia (n = 12) and fetal hydrops or nuchal edema (n = 12). Twelve pregnancies were terminated, two fetuses were stillborn and eight infants survived. Ten fetuses had right isomerism. Their main sonographic features were juxtaposition of the descending aorta and inferior vena cava (n = 7), complete atrioventricular septal defect (n = 7), left persistent superior vena cava (n = 6) and viscerocardiac heterotaxy (n = 6). In this group there was one stillbirth, five infant deaths and four survivors. The overall survival rate and spectrum of other cardiac malformations were similar between the two groups. Prenatal diagnosis of other visceral features of cardiosplenic syndromes was inconsistent. CONCLUSION: Cardiosplenic syndromes can be diagnosed with high accuracy by prenatal sonography. A diagnosis of left isomerism should be strongly suggested in the presence of a combination of at least two of the following: (1) complete atrioventricular septal defect or other structural heart disease; (2) interruption of inferior vena cava with azygos continuation; (3) early fetal heart block; (4) viscerocardiac heterotaxy. Right isomerism should be suspected in the presence of a combination of at least two of the following: (1) structural heart disease, namely complete atrioventricular septal defect; (2) juxtaposition of inferior vena cava and descending aorta; (3) viscerocardiac heterotaxy.     

The syndrome of left isomerism: sonographic findings and outcome in prenatally diagnosed cases.

OBJECTIVE: The purpose of this study was to evaluate the accuracy of the prenatal diagnosis of left isomerism and to assess possible diagnostic and prognostic markers. METHODS: We conducted a retrospective review of all previously unpublished cases of left isomerism diagnosed in the prenatal and postnatal periods in 2 tertiary referral centers in Germany over 15 years. RESULTS: Among 34 fetuses, 31 had a correct prenatal diagnosis of left isomerism; 31 had an interruption of the inferior vena cava with azygos continuation; 22 had different types of viscerocardiac heterotaxy; 13 had heart block; and 28 had cardiac defects, with a high prevalence of atrioventricular septal defects (n = 24), right outflow tract obstruction (n = 11), double-outlet right ventricles (n = 6), and anomalous pulmonary venous return (n = 6). Among the 34 cases, 9 underwent termination of pregnancy; 2 fetuses died in utero; 5 children died in the neonatal period; and 4 children died in infancy. Only the presence of heart block and hydrops was significantly correlated with nonsurvival (P < .05). Fourteen children survived, with a mean follow-up +/- SD of 2.9 +/- 2.6 years. Three survivors underwent single-ventricle palliation, and 1 had successful biventricular repair. Three children were awaiting cardiac repair. The remaining 7 children had minor or no associated cardiac defects and were doing well. CONCLUSIONS: Prenatal diagnosis of left isomerism is feasible, with high accuracy. Important diagnostic pointers are viscerocardiac heterotaxy, complex cardiac malformations, heart block, and interruption of the inferior vena cava. The mortality in fetuses and neonates is high in the presence of heart block and hydrops, whereas the cardiac defects influence the long-term outcome.     

交叉肺动脉患儿临床及CT特点(附17例报道)

目的 探讨交叉肺动脉患儿的临床及CT特点。方法 回顾性分析17例交叉肺动脉患儿的临床及CT检查资料,记录合并心血管畸形、气道畸形、肺炎及其他临床合并症情况。结果 心胸CT三维重建图像可清晰显示交叉肺动脉及其合并心血管畸形、气道畸形、肺炎。其中合并房间隔缺损9例,室间隔缺损9例,主动脉右弓右降9例,动脉导管未闭5例,迷走左或右锁骨下动脉4例,主动脉缩窄3例,法洛四联症3例,主肺动脉间隔缺损2例,部分型肺静脉异位引流2例,左肺动脉狭窄1例,主动脉瓣狭窄1例,主动脉瓣二叶畸形1例,永存动脉干1例,右心室双出口1例,主动脉离断1例,肺动脉闭锁1例,冠状动脉起源异常1例,主动脉左弓右降1例,永存左上腔静脉1例,心肌致密化不全1例。10例合并肺炎,其中2例有支气管狭窄。其他临床合并症包括18-三体综合征、顽固性低钙血症、癫痫各1例。结论 交叉肺动脉是一种罕见的先天性心脏病,常伴其他心脏、大血管发育异常,且多合并肺炎。CT三维重建图像可直观、清晰显示交叉肺动脉及其合并心血管解剖异常,同时显示肺内、气道异常及其与大血管的立体关系。