胎儿腹腔大血管异常与内脏异位畸形的关系研究

目的 探讨胎儿腹腔大血管异常与胎儿内脏异位的关系及以腹腔大血管异常作为产前超声标志筛查胎儿内脏异位畸形的价值.方法 在我院行产前系统超声检查的6532例孕妇,超声检查胎儿生长径线、腹腔大血管和脏器结构.以腹腔大血管异常的胎儿为研究对象.结果 产前超声共发现胎儿腹腔大血管异常45例,占0.7%.产后证实内脏异位综合征35例,完全性内脏反位7例,3例失访.腹腔大血管异常胎儿畸形总发生率、内脏异位综合征发生率、完全性内脏反位发生率高于腹腔大血管正常胎儿,差异有统计学意义(P＜0.001).Ⅰ型和Ⅱ型异常内脏异位综合征发生率高于Ⅲ型,差异有统计学意义(P＜0.001);Ⅰ型左侧异构发生率高,Ⅱ型右侧异构发生率高,差异有统计学意义(P＜0.001).结论 腹腔大血管异常胎儿均存在脏器的异位或反位;腹腔大血管观察方法 简单,可以作为产前筛查内脏异位畸形的有效超声标志.

Abstract：

Objective To investigate the relation between anormalities of fetal abdominal great vessels and heterotaxy, the clinical significance of screening fetal heterotaxy by the abnormalities of fetal abdominal great vessels. Methods 6532 fetuses from our hospital were scanned and the fetuses with abdominal great vessels abnormalities were included. Parameters of fetal growth, abdominal great vessels and anatomical survey were detected. Results Forty-five cases (0.7%) of fetal abdominal great vessels abnormalities were detected prenatally. Forty-two cases were confirmed postnatally, including 35 cases of heterotaxy syndrome and 7 cases of situs inversus totalis and 3 cases missed. The incidences of the abnormal fetus,heterotaxy syndrome and situs inversus totalis of the fetuses with abnormal abdominal great vessels were higher than that of the fetuses with normal abdominal great vessels, the differences were statistically significant( P＜ 0.001). The incidence of both type Ⅰ and type Ⅱ of the heterotaxy syndrome were significantly higher than that of type Ⅳ (P＜0.001). There was a high incidence of left isomerism in abnormal type Ⅰ and of right isomerism in abnormal type Ⅱ , the differences were statistically significant (P＜ 0.001). Conclusions Abnormal great vessels abnormalities have strong relations to heterotaxy and situs inversus which can be used as effective and simple indicator in screening heterotaxy syndrome.     

胎儿心脏连续扫查方法在产前诊断圆锥干畸形中的意义

目的探讨胎儿心脏连续扫查方法在产前诊断圆锥干畸形中的价值。方法根据胎儿的不同体位，应用超声对1067例孕18-26周的高危胎儿进行横向、矢状或冠状连续心脏扫查，扫查过程中将主、肺动脉排列次序或内径的改变、交叉结构的消失或不显示作为圆锥干畸形存在的特征性改变，同时对有上述特征性改变的胎儿作进一步的超声心动图检查以明确诊断。结果1067例胎儿失访17例，余1050胎儿中有上述特征性改变的胎儿33例，进一步详细行超声心动图检查后产前诊断圆锥干畸形21例，其中法洛四联症9例，大动脉转位9例，共同动脉干2例，右室双出口1例；生后超声或尸检证实产前漏诊法洛四联症1例，大动脉转位1例，右室双出口误诊为法洛四联症1例。胎儿心脏连续扫查方法在产前诊断圆锥干畸形的敏感性、特异性、阳性预测值和阴性预测值分别为91．3％、98．8％、63．6％和99．8％，产前诊断准确率为87．0％。结论胎儿心脏连续扫查过程中主、肺动脉排列次序或内径改变、交叉结构的消失或不显示对预测胎儿圆锥干畸形有较高的敏感性和特异性，结合不同类型圆锥干畸形的病理解剖特征进一步作超声心动图检查可提高圆锥干畸形的产前诊断率。     

三血管及三血管气管切面在超声产前诊断胎儿心脏大血管畸形中的应用价值

目的:回顾性分析三血管及三血管气管切面在超声产前诊断胎儿心脏大血管畸形中的应用价值。方法 :统计我院66例大血管畸形胎儿超声心动图检查情况,回顾分析四腔心、流出道、三血管、三血管气管切面在各个疾病的超声心动图特点,对患者进行复诊、随访。结果:大血管畸形在三血管及三血管气管切面有特征性表现,出现大血管排列顺序、内径、血流方向、分支等异常,结合彩色多普勒血流显像,表现出"V""O""C""U""Ioo""o Vo"型结构,该切面在主动脉弓离断、缩窄,右位主动脉弓,永存动脉干,永存左上腔静脉全部出现异常表现。而四腔心、左、右室流出道等切面在部分大血管畸形不出现异常表现。二者联合观察可提高胎儿先心病的诊断率。大血管畸形最常合并的心内畸形是室间隔缺损,最常合并的心外畸形是单脐动脉。结论:三血管及三血管气管切面可提示胎儿大血管畸形,尤其对于主动脉弓离断、缩窄,右位主动脉弓,永存动脉干,永存左上腔静脉有重要的诊断价值。有利于为孕妇及早作出产前诊断,减轻身心痛苦,提高生育质量。     

胎儿心脏超声检查常见误诊与漏诊分析

目的 分析胎儿心脏超声检查中几种较常见易误诊、漏诊疾病的超声特点。方法 回顾性分析接受胎儿心脏超声检查6745名孕妇共6848胎和出生后接受随访的332例新生儿和婴幼儿资料,分析常见易误诊、漏诊疾病的超声特点。结果 心脏畸形胎儿969胎,其中2胎完全型大动脉转位为首诊漏诊,5胎卵圆孔受限误诊为左心室发育不良。出生后于我院随访的新生儿及婴幼儿332例,与出生前检查结果不一致者9例,包括产前可疑轻度主动脉缩窄而出生后正常5例、产前诊断肺动脉瓣狭窄但出生后未见异常1例、漏诊威廉综合征2例、漏诊心内型肺静脉异位引流1例。结论 胎儿心脏超声检查中一些心脏畸形极易漏诊和误诊,对这些疾病的诊断困境和难点进行关注和探讨,有助于提高产前诊断质量。     

胎儿主动脉弓异常的超声诊断线索和方法研究

目的 探讨胎儿主动脉弓异常的超声诊断方法技巧、诊断线索,提高主动脉弓异常的产前超声诊断率.方法 选择2006年1月至2009年12月在我院行产前系统超声检查并获得结果验证的1472例正常和异常胎儿为研究对象,每个胎儿均进行四腔心切面、左右心室流出道切面、三血管气管切面的观察,当怀疑主动脉弓异常时,进一步获得主动脉弓长轴切面和冠状切面及经过气管的冠状切面.结果 产前超声共诊断148例主动脉弓异常,漏诊1例,92例获得结果验证,包括主动脉弓缩窄28例,主动脉弓离断10例,右位主动脉弓及主动脉弓分支异常52例,双主动脉弓2例.24例合并其他严重心脏畸形.主动脉弓缩窄、主动脉弓离断的线索为三血管气管平面均显示主动脉弓内径和动脉导管内径比例失调,主动脉弓内径异常小,四腔心切面显示左心小,左右心不对称;右位主动脉弓及其分支异常在3VTV平面显示主动脉弓位于气管右侧、主动脉弓和动脉导管之间距离增大呈"U"形和(或)在气管后方有发自降主动脉起始段的血管分支-锁骨下动脉.主动脉弓横切面即三血管气管切面、纵切面、冠状切面的显示率分别为 98.4%、90.0%、81.9%.结论 四腔心切面显示左心小,左右心不对称和3VT平面显示主动脉弓内径异常小,主动脉弓与动脉导管内径比例失调是诊断主动脉弓缩窄、离断的线索,主动脉弓和动脉导管之间距离增大是诊断右位主动脉弓的线索,三血管气管平面是诊断主动脉弓异常最易显示和最敏感的切面,主动脉弓冠状切面、纵切面及经过气管的冠状切面对诊断具有补充和鉴别意义.

Abstract：

Objective To study the ultrasonographic clues and methods for fetal anomalies of the aorta arch and improve prenatal detection of anomalies of the aorta arch.Methods One thousand four hundred and seventy-two cases fetus who were carried out detailed scan and whose results were confirmed were chose as study objects.Every routine fetal echocardiography included four chamber and left and right outflow tract and three-vessel trachea view(3VT).The more views which included longitudinal and coronary view of the aorta arch and coronary view of the trachea and main bronchus were obtained when the abnormality of aorta arch was suspected.Results One hundred and forty-eight cases with anomalies of aorta arch were diagnosed by ultrasonography.One case was misdiagnosed.Ninety-two fetus with anomalies of aorta arch which included 28 aortic coarctation(CoA) and 10 interrupted aortic arch (IAA) and 52 right-side aortic arch and abnormal aortic branch and 2 double aortic arch were confirmed by postmortem or postnatal echocardiography and surgery.Of the 92 confirmed cases,24 had prenatally diagnosed additional complex intracardiac anomalies.All cases with CoA and IAA presented ventricular and/or great arterial disproportion with smaller left ventricle and aorta diameter on four chamber view and 3VT.Right aortic arch (RAA) and abnormal aortic branch(AAB) displayed aortic arch located on the right side of the trachea and increased distance between the aortic arch and arterial duct and abnormal aortic arch branch-subclavian artery originating from the beginning section of the descend aorta which coursed behind the trachea with U-shaped appearance on the 3VT plane.The display rate of the transverse and longitudinal and coronary view of the aorta arch was 98.4%,90.0%,81.9%,respectively.Conclusions Disproportional ventricular and /or great arterial with smaller left ventricle and aorta diameter are the clues for CoA and IAA.Increased distance between the aortic arch and arterial duct is the clue for RSA.The transverse view of the aortic arch 3VT is the most sensitive for detecting the anomalies of the aortic arch and the most easily be obtained.The longitudinal and coronary view of the aorta arch and coronary view of the trachea and main bronchus are helpful in differentiating the anomalies of the aortic arch.     

胎儿复杂先天性心脏病超声诊断与病理解剖结果的对比研究

目的 探讨胎儿超声心动图诊断复杂先天性心脏病的临床价值.方法 对49例胎儿复杂性先天性心脏病超声诊断结果与引产后胎儿尸检心脏病理解剖结果进行对比分析.结果 产前超声诊断与病理解剖符合42例(85.71%),产前超声诊断与病例解剖结果不一致7例,其中3例产前诊断不符合,4例为不完全符合.24例合并心脏外其他畸形,9例染色体异常.结论 胎儿超声心动图诊断胎儿复杂先天性心脏病具有较高的准确性,但部分心脏畸形产前难以检出.     

胎儿心脏右室双出口12例产前超声诊断分析

右室双出口(DORV)的主要特征是两条大动脉完全或大部分起源于右心室,有肺动脉骑跨的右室双出口称为Tanssig-Bing畸形.有时也有两组半月瓣均骑跨在室间缺损上,很难判定两大动脉发自左或右心室,称之为双心室双出口.几乎所有病例均伴有室间隔缺损.肺动脉狭窄较常见,主动脉狭窄、缩窄、主动脉弓离断相对少见.     

超声诊断胎儿胸外心脏2例

病例例1,孕妇,26岁。孕1产0,妊娠25周。身体健康,月经周期规律,妊娠早期无感染及药物史,无家族心脏病遗传史。从事制鞋业8年,长期接触苯、甲苯有机溶剂（黏合剂内含有）,婚前1年脱离原生产环境,产前常规超声检查。超声检查显示：胎儿双顶径55mm,胎儿颅内结构未见异常,脊柱排列整齐自然,胎儿胸腔狭小,心脏位于胸腔外并在羊水中跳动,心脏外无任何组织覆盖,胸部横切面示：双心室对称、双心房对称,房室连接正常;股骨长约38mm,羊水适量,深约45mm（图1）。CDFI：心底部可见2条大动脉血管与胸腔相连（图2）。超声提示：胎儿胸外心脏。     

四维超声在胎儿心脏圆锥动脉干畸形的应用进展

自20世纪80年代,二维超声一直是筛查和诊断胎儿先天性心脏病(CHD)的主要方法[1],其临床诊断价值毋庸置疑.随着胎儿超声检查设备和新技术的不断发展,近年来,从静态三维超声成像发展到动态三维成像技术,为产前胎儿超声诊断CHD开辟了新的领域,目前已成为国内外研究的新方向.现就四维超声在胎儿圆锥动脉干畸形(CTD)诊断方面的临床应用进展作一综述.     

产前超声观测胎儿主动脉瓣下圆锥连接在筛查胎儿完全型大动脉转位中的应用价值

目的 探讨产前超声观测胎儿主动脉瓣下圆锥连接对诊断胎儿完全型大动脉转位的临床价值.方法 回顾分析22例经引产后尸检证实的完全型大动脉转位胎儿的超声心动图资料,总结其异常声像图特点.结果 22例中二维超声心动图均显示主动脉瓣下圆锥连接声像图特征,其中18例在左室长轴观显示两条大动脉前后平行走行,17例心底短轴观显示右室流出道包绕主动脉根部形态消失,9例显示室间隔完整,13例合并室间隔缺损,2例合并左室流出道狭窄.22例中20例主动脉与肺动脉呈右前左后排列,2例呈正前正后排列.结论 产前超声观测主动脉瓣下圆锥连接对诊断胎儿完全型大动脉转位具有重要临床价值.

Abstract：

Objective To investigate the value of prenatal ultrasound observation of subaortic conical connection in the diagnosis of complete transposition of great arteries(TGA).Methods Echocardiography findings of 22 cases with complete TGA confirmed by autopsy were retrospectively analyzed and summarized.Results All cases were shown subaortic conical connection on two-dimensional echocardiography,the two great arteries were parallel in 18 cases on left ventricular long-axis view,the character of normal right ventricular outflow tract surrounding the aortic root disappeared in 17 cases.There were 13 cases with ventricular septal defect and 2 cases with left ventricular outflow tract obstruction.Aorta and pulmonary artery were shown in right anterior and left posterior position among 20 cases,and in anterior and posterior position in 2 cases.Conclusions Prenatal ultrasound observation of subaortic conical connection is of great value in the diagnosis of complete TGA.     

胎儿先天性心脏病的超声诊断及分型

目的 探讨胎儿心血管畸形的常见类型、合并畸形及分型价值.方法 行胎儿超声心动图检查孕妇3201例,明确诊断胎儿先天性心脏病239例,其中8例系双胎妊娠中的一胎存在心血管畸形.胎儿出生后半年内进行超声复查.结果 239例中检出复杂先天性心脏病155例,其中检出率较高病种依次为单心室59例、右室双出口29例、房室间隔缺损19例、法洛四联症或五联症12例、永存动脉干11例、右心发育不良综合征6例、完全性大血管转位6例.239例中引产100例,其中病理证实41例;出生16例;待产及失访123例.结论 胎儿心血管畸形以复杂和多种畸形并存多见;本组胎儿先天性心脏病检出率7.47％,复杂先天性心脏病占64.85％.根据胎儿先天性心脏病类型,确定出生后能否手术、矫治术式和预后综合评估分为可根治型、可治姑息型和无法治疗型,这一新的理念将有助于孕妇及家庭作出合理的抉择.     

产前超声筛选胎儿中晚期心脏病的临床研究

目的探讨四腔心观（FCV）加三血管-气管观（3VT）在胎儿中晚期心脏病产前超声筛选中的临床意义。方法应用Sequoia512彩色多普勒超声诊断仪，检查孕18～40周胎儿共3211例，与尸体解剖证实和产后超声追踪对照，比较单纯FCV、FCV加左、右室流出道观（FCV＋VOTV）、FCV加大动脉短轴观（FCV＋SAV）、FCV＋3VT筛选胎儿中晚期心脏畸形的敏感性。结果FCV筛选胎儿心脏病的敏感性为54％（91／169），FCV＋VOTV的敏感性为70％（118／169），FCV＋SAV的敏感性为66％（112／169）；FCV＋3VT的敏感性为85％（143／169），明显高于前三者（P〈0、05）。结论FCV＋3VT可以明显提高筛选技术的敏感性，缩短检查时间，可以作为筛选妊娠中晚期胎儿心脏病的首选方法。     

胎儿心脏三血管气管平面在先天性心脏病筛查中的价值

目的 探讨胎儿三血管气管平面的声像图特点及其筛查胎儿先天性心脏病的价值。方法 回顾性分析2003年4月至2005年4月1212例接受产前胎儿心脏超声筛查孕妇资料，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例胎儿超声心动图检出胎儿心脏结构异常47例，22例合并心外畸形。33例在本院引产，20例尸检，其中18例尸检与产前超声诊断相符，1例单心房单心室并大动脉位置正常误诊为单心房单心室并大动脉转位，1例单心房单心室并永存动脉干误诊为完全性心内膜垫缺损并永存动脉干。15例胎儿抽脐带血检查染色体，3例染色体异常。结论 三血管气管平面在筛查胎儿大动脉和心室流出道异常的先天性心脏病中有重要作用。     

超声心动图评价动脉导管对胎儿先天性心脏病的诊断价值

目的 探讨超声心动图评价动脉导管在胎儿先天性心脏病中的诊断价值.方法 以三节段诊断法进行胎儿超声心动图检查,依胎位不同在不同切面检查动脉导管,切面包括动脉导管与右肺动脉形成的"V"形征切面,动脉导管与主动脉弓峡部形成的"V"形征切面,动脉导管与左、右肺动脉形成的"三指征"切面,左肺动脉、动脉导管、主动脉弓峡部形成的"Z"形征切面,测量动脉导管的内径及流速.结果 检出先天性心脏病胎儿31例,13例经尸体解剖得到证实,其中动脉导管狭窄者4例,均为右心梗阻性疾病;动脉导管增宽3例,均为左心梗阻性疾病;动脉导管内测及反向血流者2例;动脉导管流速增高者5例,其中包括4例右心梗阻性疾病、1例左心梗阻性疾病,5例胎儿动脉导管未见异常;1例胎儿动脉导管缺如.结论 超声心动图评价动脉导管是筛查胎儿先天性心脏病的重要内容,有助于快速确定诊断.

Abstract：

Objective To explore the value of ductus arteriosus in ultrasound diagnosis of fetal congenital heart disease by echocardiography. Methods The fetal echocardiography was performed with three segments diagnosis method. According to the fetal position, the ductus arteriosus was examined in different sections which include the "V" shape sign section formed by ductus arteriosus and right pulmonary artery,the "V" shape sign section formed by ductus arteriosus and the gorge of aortic arch,the three fingers sign section formed by ductus arteriosus,left and right pulmonary artery,the "Z" shape sign section formed by left pulmonary artery, ductus arteriosus and the gorge of aortic arch. The flow velocity and the inner diameter of the ductus arteriosus were measured. Results Thirty-one cases with congenital heart disease were evaluted. Thirteen cases were approved by autopsy. Among those fetus, 4 cases went with ductus arteriosus stenosis and they all accompanied with right heart obstructing disease,3 cases went with dilated ductus arteriosus and they all accompanied with left heart obstructing disease. Retrograde were explored in 2 fetus. The flow velocity of 5 fetus ductus arteriosus were higher than the normal fetus and 4 cases of them with right heart obstruction, 1 cases with left heart obstruction. Five fetal ductus arteriosus were normal. One fetal ductus arteriosus was absent. Conclusions Assessant of ductus arteriosus by echocardiography is an important content in screening fetal congenital heart disease. It can help us to diagnose quickly.     

胎儿心脏畸形在高危与低危孕妇中超声检出率的研究

目的 通过比较胎儿心脏畸形在高危与低危孕妇人群中的超声检出率,为制定胎儿心脏超声筛查的策略提供依据.方法 将7165例受检孕妇分为高危组(2147例)和低危组(5018例).以四腔心、左右室流出道为主要筛查切面,对疑有异常者行详细超声心动图检查.统计分析两组胎儿心脏畸形的超声检出率以及有关危险因素的分布.结果 共检出胎儿心脏畸形95例(1.33%),其中高危组检出32例(1.49%),高龄妊娠位居高危因素的第一位.低危组检出63例(1.26%).高危组与低危组孕妇其胎儿心脏畸形的超声检出率比较差异无统计学意义(X2=0.635,P=0.426).对23例心脏畸形胎儿进行了脐血或羊水穿刺染色体检查,其中染色体异常11例(47.83%).结论 胎儿心脏畸形在高危与低危人群中的超声检出率相近,应提倡对所有胎儿行心脏超声筛查,以便在产前尽早发现胎儿严重的心脏畸形.     

超声心动图对复杂胎儿先天性心脏畸形的评价及临床意义

目的 探讨超声心动图对复杂胎儿先天性心脏畸形的评价及临床意义.方法 对产前超声检查发现的复杂胎儿先天性心脏畸形声像图特征行产后尸解和病理验证.结果 超声心动图产前诊断复杂胎儿先天性心脏畸形20例.各类畸形包括:单心室4例,大动脉转位2例,右室双出口2例,永存动脉干2例,左心发育不良综合征3例,心内膜垫缺损3例,右心室发育不良2例,法洛四联症1例,肺动脉闭锁1例.超声产前诊断率100%.结论 超声心动图可以对复杂胎儿先天性心脏畸形进行综合评价,对帮助临床采取相应的决策和产前孕妇及家属知情具有重要意义.     

胎儿心脏畸形超声筛查漏诊误诊原因分析

目的 探讨如何更好使用超声心动图筛查中晚孕胎儿心脏畸形,避免漏诊.方法 超声筛查中晚期孕妇7020例,对检出的胎儿心脏畸形及可疑先天性心脏病者进行每4周一次追踪观察,对产前超声未发现心脏结构异常的胎儿进行每8周一次追踪观察,出生后每6个月一次追踪观察,至出生后6～12个月.所有异常胎儿均经尸检、出生后手术结果证实(部分术前行心血管造影).对比分析同类畸形产前超声诊断正确的经验与超声漏诊的教训.结果 通过引产后病理证实或出生后心血管造影和(或)手术证实的53例心脏大血管异常中,产前超声检出并诊断正确43例,误诊2例,漏诊8例.误诊2例包括1例肺动脉瓣闭锁和1例法洛四联症.产前超声漏诊8例包括6例小于5 mm的室间隔缺损和2例下腔静脉异位引流.产前超声筛查胎儿心脏大血管畸形正确率为81.1％ (43/53).结论 采用高频探头、图像的缩放功能以及注重右室流出道-肺动脉长轴切面和静脉-心房连接关系的扫查是避免漏诊或不全面诊断的重要因素.