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。     

下腔静脉中断的产前超声诊断价值

目的 探讨下腔静脉中断的产前超声诊断价值.方法 回顾性分析17例产前超声诊断为下腔静脉中断的胎儿超声资料及产后随访资料.结果 16例经产后尸体解剖证实,1例经产后超声及CT检查证实为下腔静脉中断.17例胎儿产前超声均表现为腹围切面下腔静脉回声缺如,腹部矢状切面肝静脉直接回流人右心房;四腔心观显示“双管征”.17例下腔静脉中断的胎儿产后证实均为内脏异位综合征,其中14例为左侧异构综合征.结论 下腔静脉中断是内脏异位综合征及左侧异构综合征的有效产前超声标志,产前超声诊断胎儿下腔静脉中断有重要的临床价值.     

胎儿下腔静脉离断的超声诊断分析

[目的]探讨产前超声诊断胎儿下腔静脉离断的价值。[方法]对2007年3月至2012年12月在本院产前超声系统筛查201069例孕妇,诊断胎儿下腔静脉离断34例的声像图特征及漏诊原因进行分析。[结果]胎儿下腔静脉离断诊断率为0．17‰,34例中内脏异位综合征11例（其中左侧异构9例,右侧异构2例）,完全性内脏反位2例;合并心脏畸形18例（房室间隔缺损4例,法洛四联症3例,永存动脉干3例,左上腔静脉3例,右室双出口1例,房室间隔缺损＋肺静脉异位引流1例,法洛四联症＋左上腔静脉1例,永存动脉干＋肺静脉异位引流1例,右室双出口＋肺静脉异位引流1例）;漏诊3例,2例在孕23、24周筛查时漏诊,孕32周复查时检出;1例出生后诊断,随访至今5年余,患儿生长发育正常,无异常临床表现。[结论]上下腔静脉长轴切面、胸腹部斜冠状切面、四腔心切面及上腹部横切面是筛查胎儿下腔静脉离断的重要切面,对产前超声诊断胎儿下腔静脉离断具有重要的临床价值。     

产前超声诊断胎儿静脉导管汇入异常

<正>静脉导管（ductus venosus,DV）位于胎儿肝脏内,连接脐静脉与下腔静脉近心端,在胎儿血液循环中起重要作用。DV汇入异常是罕见胎儿期血液循环异常。本研究观察产前超声诊断胎儿DV汇入异常的价值。1资料与方法1.1一般资料回顾性分析2019年9月—2022年8月16胎于保定市妇幼保健院经产前超声诊断的单胎DV汇入异常胎儿,孕妇年龄22～40岁、平均（29.7±4.7）岁,孕周22～34周、平均孕周（26.2±3.1）周。检查前孕妇或家属签署知情同意书。     

产前超声心动图与解剖铸型观察胎儿完全性肺静脉异位引流

目的 采用产前超声心动图与解剖铸型观察胎儿完全性肺静脉异位引流（TAPVC）。方法 纳入9胎经产前超声心动图诊断为TAPVC的胎儿,对引产后3胎标本进行解剖、6胎标本制作胎儿心脏铸型,观察胎儿静脉回流特点及合并心内外畸形。结果 9胎TAPVC中,66.67%（6/9）为心上型,33.33%（3/9）为心下型。胎儿超声四腔心切面见左心房缩小,未见静脉与左心房相连;三血管气管切面见左心房后方赘余血管;6胎心上型TAPVC中,4胎肺静脉汇入右上腔静脉、2胎肺静脉汇入左上腔静脉;3胎心下型TAPVC肺静脉均汇入门静脉。9胎均合并心房异构综合征,8胎（8/9,88.89%）合并单心室,7胎（7/9,77.78%）合并肺动脉狭窄,合并单心房及完全型心内膜垫缺损各4胎（4/9,44.44%）;5胎（5/9,55.56%）合并永存左上腔静脉,合并双下腔静脉及左头臂静脉主动脉弓下走行各1胎（1/9,11.11%）;1胎（1/9,11.11%）合并膈疝。结论 TAPVC胎儿多合并心内外畸形及体静脉异常。产前超声具有重要诊断价值,解剖铸型有助于认识TAPVC胎儿血管走行及静脉引流途径。     

左侧异构的产前超声诊断价值

目的:探讨产前超声诊断胎儿左侧异构的价值.方法:回顾性分析16例产后确诊的左侧异构胎儿的病例资料,对其超声特点进行分析总结.结果:产前超声发现下腔静脉中断14例(14/16),心脏畸形15例(15/16),心脏传导阻滞5例(5/16),胎儿胸、腹腔脏器位置异常13例(13/16).结论:产前超声诊断胎儿左侧异构具有很高的价值.产前超声发现胎儿心脏畸形及胸、腹腔脏器位置异常合并下腔静脉中断和/或心脏传导阻滞,提示左侧异构.     

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.     

胎儿三血管气管平面在胎儿心室发育不良诊断中的价值

目的探讨胎儿三血管气管平面(3V T切面)在胎儿心室发育不良诊断中的价值.方法回顾性分析2003年4月-2005年1月在我院产前诊断门诊接受产前胎儿心脏超声筛查孕妇1212例,年龄20～44岁,孕周为16～38周.取胎儿心脏检查的常规6个标准切面,然后在四腔心切面的基础上缓慢平行向胎儿头侧移动探头即可显示胎儿上纵隔横切面,即三血管气管平面,再叠加彩色多普勒血流显像,观察各房室及大动脉血流分布和血流方向.结果 1212例胎儿超声心动图均获得满意的3V T切面,共检出胎儿心脏结构异常47例,其中心室发育不良11例(8例左心发育不良综合征和3例右心发育不良),年龄23～42岁,平均(29.8±5.7)岁,孕周20～39周,平均(26.7±5.6)周.10例心室发育不良均已引产并有5例尸检,尸检结论与超声检查完全一致,1例胎死宫内.结论胎儿3V T切面是一个简单、可靠的胎儿心脏大血管的筛查方法,能提高我们对涉及大动脉异常胎儿先心病的理解,是诊断胎儿心室发育不良的又一个重要切面.     

B超诊断胎儿心脏异位畸形

Ｂ超诊断胎儿心脏异位畸形郑州黄河中心医院Ｂ超室陈鸽平患者２６岁，妊娠３９周，孕１产０。产前行Ｂ超常规检查。超声所见：胎儿胎位、胎头脊柱、四肢、胎盘及羊水均未见异常。内脏声象图发现，其心脏位于肝胆声象图上方胸腔内，肝胆声象的左侧方可见胃泡回声。心脏四腔...     

下腔静脉离断产前超声诊断

目的 探讨胎儿下腔静脉离断产前超声声像图特征和有效诊断切面,以提高其产前超声诊断准确率.方法 回顾性分析我院2006年1月至2010年5月诊断的10例下腔静脉离断胎儿(其中7例经引产后尸体解剖证实)产前超声声像图,与正常超声声像图对比,总结其产前超声声像图特征及有效切面.结果 10例下腔静脉离断胎儿,9例合并严重心内结构畸形,7例合并心外结构畸形(其中5例合并内脏反位).本组10例下腔静脉离断胎儿产前超声声像图均表现为四腔心切面异常(奇静脉扩张)、上腹部横切面异常(正常下腔静脉不能显示)、胸腹部斜冠状切面异常[离断的下腔静脉异位连接于奇(半奇)静脉并穿过膈肌连于腔静脉]及右心房纵切面异常(肝上段下腔静脉入右心房,肾上段肝段间的下腔静脉缺失并离断).结论 下腔静脉离断常合并心内、外结构畸形.下腔静脉离断胎儿在四腔心切面、上腹部横切面、胸腹部斜冠状切面及右心房纵切面上均有特征性超声声像图表现.识别下腔静脉离断特征性超声声像图表现可明显提高其产前超声检出率和诊断准确率.     

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.     

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.     

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.     

双下腔静脉并右肾重复肾胎儿产前超声诊断学特征并文献复习

目的探讨双下腔静脉并右肾重复肾胎儿产前超声诊断学特征。 方法回顾性分析2019年8月5日湖北省妇幼保健院超声科诊断的1例双下腔静脉并右肾重复肾胎儿的产前超声资料，总结双下腔静脉的超声声像图特征，并复习文献。 结果胎儿超声检查右肾见2个肾盂和2组肾静脉，腹部冠状切面显示下腔静脉在肾脏水平向左跨过腹主动脉，肾水平以下与腹主动脉并行左、右侧各见一支下腔静脉，呈"平行三血管征"，双下腔静脉血流走行一致。彩色多普勒超声显示双下腔静脉与腹主动脉血流方向相反，并记录到静脉频谱。 结论掌握双下腔静脉胚胎学基础及超声声像图特征，有利于提高产前超声检出率。     

肺动脉异常起源于升主动脉产前超声诊断并文献回顾

目的探讨肺动脉异常起源于升主动脉的产前超声图像特征。方法回顾性分析产前超声诊断的2例左肺动脉异常起源于升主动脉胎儿临床资料,总结肺动脉异常起源于升主动脉产前超声诊断特点,与引产后胎儿的高频超声及病理解剖结果进行对比,并对国内外相关文献进行回顾分析。结果 2例产前超声诊断左肺动脉异常起源于升主动脉均经尸体解剖证实,近端型与远端型各1例,1例合并法洛四联症,右位主动脉弓,动脉导管缺如;1例合并肺动脉瓣缺如,室间隔缺损,永存左上腔静脉,三尖瓣反流,右心增大。2例羊水染色体核型均正常。结论肺动脉异常起源于升主动脉有特征性超声表现,产前超声检查可作出诊断。     

Azygous continuation of the interrupted inferior vena cava: A Clue to Prenatal Diagnosis of the Cardiosplenic Syndromes.

We reviewed the prenatal sonographic findings in 11 consecutive fetuses diagnosed as having abdominal situs inversus (stomach on the right) at a single institution. Interruption of the inferior vena cava with azygous continuation was diagnosed by the “double vessel” sign. This sign was considered to be present in nine cases, including all eight fetuses who proved to have the polysplenia syndrome. The only false positive diagnosis of IVC interruption with azygous continuation was made prospectively in one fetus with asplenia. At autopsy, this fetus showed a large left-sided superior vena cava. We conclude that, in combination with cardiac anomalies or situs abnormalities, interruption of the IVC with azygous continuation should suggest a specific diagnosis of a cardiosplenic syndrome, especially polysplenia. This information should be helpful in patient counseling and management.     

Prenatal diagnosis of right atrial isomerism (asplenia-syndrome): case report

Right atrial isomerism (earlier known as asplenia) is a group of malformations including anomalies of the heart and the visceral organs. Complex defects of the heart are normally found, but these are non-specific. The suspected diagnosis can be confirmed most easily by focusing on a specific and segmental examination of the heart. We report on a fetus referred at 22 weeks in whom AV-septal defect was detected. Heart and stomach were on the left side, but the segmental approach allowed the detection of juxtaposed descending aorta and inferior vena cava as typical signs of right isomerism. The suspected malformation could be verified by the additional detection of infra-diaphragmatic total anomalous pulmonary venous drainage by using colour Doppler. Howell-Jolly bodies from fetal blood supported the diagnosis of asplenia. Autopsy after termination of pregnancy demonstrated the typical signs of right isomerism. The paper reviews the typical prenatal findings which lead to the detection of right isomerism in the fetus. Segmental approach should be performed in each fetus with an anomaly of the heart especially in the presence of complex defects.