胎儿超声心动图在产前诊断完全型肺静脉异位引流中的应用

目的探讨完全型肺静脉异位引流（TAPVC）胎儿超声心动图特征及产前诊断临床意义。方法对2010年1月至2013年1月在南京医科大学附属苏州医院超声中心经产前及产后超声心动图或引产后尸检确诊的13例TAPVC胎儿超声心动图特征及产前诊断的临床意义进行总结分析。结果产前超声诊断12例TAPVC,其中心上型7例,心下型3例,心内型2例。超声表现为：胎儿左心稍小于右心,卵圆孔较大,心房水平右向左分流丰富,左心房与降主动脉距离增宽,左心房顶部光滑,无肺静脉开口,其后方可见共同肺静脉干,追踪共同肺静脉干走行可显示引流部位,相应引流部位血管内径扩张。产前超声漏诊1例,经产后超声心动图确诊为心内型TAPVC。13例TAPVC胎儿中3例为单发畸形（3/13）,10例合并其他心内或心外畸形（10/13）。结论 TAPVC有特征性胎儿超声心动图表现,胎儿超声心动图是诊断TAPVC的可靠方法;产前诊断TAPVC有利于孕妇作出优生优育选择,使胎儿出生后得到及时治疗。     

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。     

超声心动图评估完全型肺静脉异位连接中肺静脉病变

目的 探讨超声心动图评估完全型肺静脉异位连接（TAPVC）中肺静脉病变的价值。方法 收集55例TAPVC患儿的超声心动图资料,并与CTA及术中所见进行对比。结果 应用超声心动图根据肺静脉引流部位对55例TAPVC患儿进行分类,分为心上型24例、心内型20例、心下型7例及混合型4例。15例垂直肺静脉梗阻中,心上型的梗阻多出现在垂直静脉与无名静脉或上腔静脉之间,心下型的梗阻均出现在垂直静脉与肝静脉或门静脉之间。肺静脉狭窄4例,其中3例局限性狭窄均为心内型,出现在分支肺静脉与共同肺静脉腔或右心房之间,1例弥漫性狭窄为心下型。分支肺静脉异常9例,其中超声心动图仅检出1例,而CTA检出8例。结论 超声心动图能对TAPVC中的肺静脉的引流、梗阻和分支肺静脉近端的狭窄做出更为全面的评价。CTA评估分支肺静脉的汇入和数目异常及远端肺静脉的成像方面优于超声心动图。     

超声心动图诊断胎儿孤立性完全性肺静脉异位引流

目的探讨超声心动图诊断胎儿孤立性完全性肺静脉异位引流(TAPVC)的价值.方法 回顾性分析17例胎儿孤立性TAPVC的产前超声资料,观察分析其二维超声心动图及CDFI表现,测量TAPVC胎儿左心室与右心室横径比值(LV/RV)、左心房与右心房横径比值(LA/RA),并与同孕周正常胎儿预测值进行统计学比较.结果 15例孤立性TAPVC胎儿中,心上型9例,心内型4例,心下型2例;4例见血流梗阻,其中心上型3例,心下型1例,CDFI示梗阻处血流信号明亮,梗阻处流速1.0~1.6 m/s.15例孤立性TAPVC胎儿的LV/RV为0.75~0.99,平均0.88±0.08,小于同孕周正常胎儿预测值(t=-3.305,P=0.005).LA/RA为0.68~1.00,平均0.84±0.11,亦小于同孕周正常胎儿预测值(t=-3.320,P=0.005).结论 超声心动图可用以胎儿期诊断孤立性TAPVC.     

超声二维斑点跟踪技术测量三尖瓣环位移评价右心室收缩功能

目的 探讨彩色多普勒超声心动图对完全性肺静脉异位连接(TAPVC)的诊断价值。方法 回顾分析19例经手术证实的TAPVC患者的超声心动图诊断情况。结果 首次彩色多普勒超声心动图检查诊断TAPVC与手术诊断的符合率为89.47%(17/19),其中心上型11例,心内型5例,心下型1例。首次超声检查漏诊2例(10.53%),其中包含1例心下型TAPVC以及将1例混合型TAPVC(心上型+心内型)诊断为部分性肺静脉异位连接心内型。这2例患者术前再次复查超声心动图,均正确诊断。19例TAPVC均伴继发孔房间隔缺损及中至重度肺动脉高压。19例TAPVC超声心动图表现共同特点:右心系统扩大,以右心房为主,肺动脉增宽,右心房和(或)左心房增大,左心系统缩小,以左心房缩小显著;彩色多普勒血流显示均为房水平右向左蓝色分流束;不同分型的特征性改变:左心房内不能探及肺静脉的入口部位,左心房缩小,左心房后方可探及一管状无回声结构,即共同静脉干。心上型TAPVC者胸骨上窝主动脉弓长轴切面可显示于主动脉弓前方并环绕主动脉弓的异常“静脉弓”,即共同静脉干经垂直静脉、无名静脉汇入上腔静脉;心内型TAPVC者大多数经冠状静脉窦汇入右心房,此时,在胸骨旁左心室长轴及四腔心切面可显示扩张的冠状静脉窦及其与共同静脉干沟通;心下型TAPVC者剑下主动脉短轴切面显示三条大血管,异常连接的共同静脉干位于下腔静脉左前、腹主动脉的前方,血流呈红色与腹主动脉相同,长轴追踪该共同静脉干于肝右叶后方汇入门静脉。混合型TAPVC(心上型合并心内型)者,胸骨上窝主动脉弓长轴切面显示左上肺静脉与垂直静脉(相对较细)相连,在胸骨旁左心室长轴及四腔心切面可显示右肺静脉及左下肺静脉经冠状静脉窦汇入右心房。合并心内畸形:19例TAPVC患儿中,2例合并心内膜垫缺损,1例合并大动脉转位,1例合并肺动脉瓣狭窄,2例合并动脉导管未闭。结论 二维彩色多普勒超声心动图经多部位、多切面、多方向的探查,对TAPVC能明确诊断及分型,为外科治疗方案提供重要依据,且检查简便易行、无创、可重复,是诊断TAPVC的首选方法。     

超声心动图检查在胎儿完全性肺静脉异位引流畸形诊断中的应用

目的 探讨超声心动图检查在胎儿完全性肺静脉异位引流(TAPVC)诊断中的临床价值.方法 回顾性分析11例TAPVC胎儿的超声心动图特征以及产后超声心动图检查或引产后尸检的结果.结果 11例TAPVC胎儿(心上型7例,心内型3例,心下型1例),3例属于单发畸形,8例并发其他心内外畸形.超声心动图正确诊断9例,准确度约为81.82％ (9/11),漏诊2例.典型的声像图特征:四腔心切面左心稍小于右心,左房顶壁光滑,无肺静脉开口,4条肺静脉在左房后方汇入共同肺静脉干,并经引流血管汇入右房,相应引流血管扩张.结论 超声心动图检查是产前诊断TAPVC的可靠方法,有利于孕妇作出优生优育选择,具有较高的临床应用价值.     

彩色多普勒超声心动图诊断完全性肺静脉异位连接的方法和价值

目的:探讨完全性肺静脉异位连接(TAPVC)的彩色多普勒超声心动图(CDE)图像特征及超声心动图诊断TAPVC的价值。方法:对16例手术证实的TAPVC患儿进行回顾性分析。结果:TAPVC的CDE具有明显的图像特征:①肺静脉未与左房直接连接;②心上型TAPVC可见肺静脉共腔-垂直静脉-左无名静脉-上腔静脉-右心房或肺静脉共腔-上腔静脉近心端;心内型TAPVC可见四根肺静脉引流入扩张的冠状静脉窦-右心房;心下型TAPVC可见肺静脉共腔-降垂直静脉-肝内门静脉相连;混合型TAPVC以心内型+心上型常见;③均有心房水平(经ASD或PFO)右向左分流。根据TAPVC的CDE图像特征该16例作出正确诊断,诊断准确率100%,引流部位正确率94%。结论:彩色多普勒超声心动图对TAPVC的诊断有很高的临床价值。     

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。     

胎儿完全性肺静脉异位引流的产前超声诊断价值

目的分析总结胎儿完全性肺静脉异位引流(TAPVC)的超声心动图表现;探讨产前超声诊断胎儿TAPVC的临床意义。方法回顾性分析总结15例我院产前及产后超声心动图或引产后尸检确诊为TAPVC的胎儿超声心动图表现,并将产前超声心动图与产后超声心动图或引产后尸解结果对照。结果产前超声诊断14例TAPVC中,12例TAPVC均伴复杂型先心病(均为右侧异构综合征),其中心内型3例,心上型5例,心下型4例;1例混合型(心内型+心上型)TAPVC;1例心内型TAPVC;产前超声漏诊1例,产后行超声心动图证实为心内型TAPVC。结论胎儿TAPVC超声心动图有特征性表现;产前超声对胎儿TAPVC有重要的诊断意义。     

胎儿静脉导管异位引流至冠状静脉窦产前超声表现:3例报告及文献复习

目的 结合文献及3例临床所见,观察胎儿静脉导管（DV）异位引流至冠状静脉窦（CS）产前超声表现。方法 回顾性分析3胎DV异位引流至CS胎儿,结合文献复习观察其产前超声特征。结果 产前超声于3胎四腔心后切面均见CS扩张,内径分别为3.6、8.2及4.8 mm;DV异位引流至扩张的CS。于1胎（序号1）见4支肺静脉汇入左心房（LA）,DV经房间隔后方于左心房顶绕行至扩张的CS;1胎（序号2）三血管气管切面肺动脉主干左侧见永存左上腔静脉（PLSVC）,旋转探头追踪观察发现DV与CS相连;1胎（序号3）4支肺静脉均回流入LA,三血管气管切面见主动脉弓位于气管右侧、左锁骨下动脉起自降主动脉起始部。结论 产前超声观察胎儿DV异位引流至CS四腔心后切面可见CS扩张,并可合并其他血管畸形。     

Partial and total anomalous pulmonary venous connection in the fetus: two-dimensional and Doppler echocardiographic findings.

OBJECTIVE: Prenatal diagnosis of total (TAPVC) or partial (PAPVC) anomalous pulmonary venous connection in isolation or associated with other cardiac disease is important for appropriate prenatal counseling and perinatal management. We sought to assess the echocardiographic clues to the fetal diagnosis of TAPVC and PAPVC in a cohort of affected fetuses. METHODS: We retrospectively reviewed 29 fetal echocardiograms performed in 16 pregnancies with fetal TAPVC or PAPVC, systematically analyzing heart chamber size, presence of a confluence behind the left atrium or of a vertical vein, and Doppler flow patterns. RESULTS: Prenatal diagnosis was made at a mean gestational age of 27 +/- 7 weeks. TAPVC was found in 11 cases; five cases for each of supracardiac and infracardiac types and one mixed type. PAPVC was diagnosed in five fetuses, four of which had scimitar syndrome. Ten fetuses had an additional major cardiac defect, including hypoplastic left heart syndrome and right atrial isomerism. In three cases the prenatal diagnosis was only made at follow-up assessment. Among TAPVC cases, visualization of a confluence behind the left atrium (10/11) and a vertical vein (11/11) were the most consistent echocardiographic clues. Dextrocardia and a small right pulmonary artery suggested scimitar syndrome. The diagnosis was confirmed postnatally or at autopsy in 12 cases. In six fetuses with TAPVC and obstruction confirmed postnatally, continuous turbulent flow in the vertical vein and monophasic continuous flow in the pulmonary veins were demonstrated by color and spectral Doppler. CONCLUSIONS: Fetal echocardiography permits prenatal diagnosis of TAPVC or PAPVC. Spectral and color Doppler provide clues to the presence of an obstructed pulmonary venous pathway.     

产前超声诊断胎儿孤立完全性肺静脉异位连接

目的探讨本中心32例胎儿孤立完全性肺静脉异位连接(TAPVC)产前超声的主要特征。方法选择我院经产前超声心动图检出孤立TAPVC病例32例,经出生后检查或尸检证实诊断,回顾性分析超声检查的图像和结果。结果本研究胎儿共诊断TAPVC 75例,孤立TAPVC 35例。单发病例诊断时孕周为22~38周,平均(27.5±4.5)周,年龄为21~38岁,平均(28.0±4.0)岁,其中引产23例,12例出生。出生后证实诊断的9例,假阳性3例。出生的9例TAPVC病例8例在新生儿期手术治疗,平均手术年龄8.6d,平均随访40.6个月(18~75个月)。引产病例由尸检证实诊断。总结可作为超声诊断特征的直接征象和间接征象:包括肺静脉共同腔、肺静脉回流完整途径(是否存在梗阻)、右心优势、左房后壁-降主动脉的距离、引流入血管(腔静脉和冠状窦)扩张等。结论胎儿孤立TAPVC产前超声具有特征性表现。产前系统检查可对TAPVC的分型及梗阻做出准确的判断,为孕妇制定最佳的生产计划和手术方案。     

Totally anomalous pulmonary venous connection and complex congenital heart disease: prenatal echocardiographic diagnosis and prognosis.

OBJECTIVE: The purpose of this study was to determine the accuracy of prenatal cardiac diagnosis, prognosis, and outcome of totally anomalous pulmonary venous connection (TAPVC) and to determine echocardiographic clues in the prenatal diagnosis of isolated TAPVC or TAPVC in association with other complex congenital heart disease (CHD). METHODS: We reviewed our 13-year experience of prenatal diagnosis of TAPVC. Thirteen fetuses were identified with the diagnoses of TAPVC. We systematically analyzed the individual pulmonary veins by color and pulsed Doppler imaging, the presence of a pulmonary venous confluence, the pulsed and color Doppler evaluation of the vertical vein, and sites of connections. Prenatal diagnosis was confirmed by postnatal echocardiography, cardiac catheterization, surgery, or autopsy. RESULTS: The mean gestational age at diagnosis of TAPVC was 26.3 weeks (range, 20-33 weeks). There were 8 fetuses with TAPVC and right isomerism, 3 fetuses with other associated CHD, and 2 with isolated TAPVC. There were 7 fetuses with supracardiac TAPVC, 4 with infracardiac TAPVC, and 2 with mixed TAPVC. Pulmonary vein color and pulsed Doppler data were available in 10 of 13 fetuses. The pulmonary venous confluence was visualized in all fetuses except 1. The vertical vein was visualized in all fetuses. Five fetuses had suspected signs of obstruction. The diagnosis was confirmed postnatally or at autopsy in 12 cases. Eight patients underwent surgery; 6 died, and 2 were alive. Two patients had compassionate care and died; 3 pregnancies were terminated. CONCLUSIONS: It is possible to diagnose accurately complex CHD, including the pulmonary venous connections. When diagnosed prenatally, TAPVC carries a poor prognosis.     

超声产前诊断胎儿完全性肺静脉畸形引流的临床研究

目的 探讨产前超声诊断胎儿完全性肺静脉畸形引流的临床价值.方法 回顾性分析我院诊断的10例胎儿完全性肺静脉畸形引流的产前超声表现,并与病理结果进行对照.结果 10例胎儿产前超声诊断完全性肺静脉畸形引流,7例终止妊娠后病理解剖证实,失访3例.7例经解剖证实的完全性肺静脉畸形引流病例产前超声检查均可见左心房显著缩小,彩色多普勒不能显示任何一支肺静脉血流引流入左心房,其中5例显示共同肺静脉腔并测及高速静脉血流频谱、引流途径管腔增宽,3例合并其他心外畸形.结论 产前超声对胎儿完全性肺静脉畸形引流的诊断具有重要的临床价值.     

胎儿超声心动图在诊断永存左上腔静脉中的应用

目的 总结永存左上腔静脉胎儿超声心动图特征及临床意义.方法 对2008年5月至2011年1月在我院诊断为永存左上腔静脉的33例胎儿超声心动图表现进行总结分析,并与引产后病理或产后超声心动图诊断结果进行对照分析,总结永存左上腔静脉胎儿超声心动图特征及临床价值.结果 33例永存左上腔静脉胎儿中引产后病理或产后超声心动图证实32例,超声表现为:四腔观可见扩张的冠状静脉窦,三血管观在肺动脉左侧可见一圆形血管回声,为永存左上腔静脉,同时可见右上腔静脉变细,追踪走行可见其与冠状静脉窦相连续.其中18例为单发畸形(18/32,56.25%),14例合并其他畸形(7例合并心内畸形,2例合并心外畸形,5例同时合并心内和心外畸形).胎儿超声心动图将内脏反位综合征合并完全型房室间隔缺损误诊为同时合并永存左上腔静脉1例.结论 胎儿超声心动图是诊断永存左上腔静脉的可靠方法.永存左上腔静脉常合并其他畸形,产前超声发现永存左上腔静脉需进一步检查以排除其他合并畸形.     

Changes in pulmonary venous Doppler parameters in fetal cardiac defects.

OBJECTIVES: Recent observations in pediatric cardiological studies have prompted discussion on the possible role of intrauterine pulmonary venous changes in neonatal and postoperative outcome of infants with congenital heart defects. This study analyzes changes of Doppler blood flow velocity waveforms in the pulmonary veins of fetuses with different cardiac defects. METHODS: Eighty fetuses (mean gestational age, 27 weeks) with prenatally diagnosed cardiac defects were classified into one of five groups: obstructed left atrium, other left ventricular outflow tract obstruction, right ventricular outflow tract obstruction, miscellaneous cardiac defects and total anomalous pulmonary venous drainage. Doppler examination of the pulmonary veins was performed and the time velocity integral (TVI), end-diastolic (A) velocity, and pulsatility index for veins (PIV) were compared with reference ranges. RESULTS: Fetuses with infradiaphragmatic total anomalous pulmonary venous drainage showed a continuous Doppler flow pattern instead of the typical pulsatile waveform pattern. In fetuses with obstructed left atrium and restrictive foramen ovale, a reversed A-velocity and increased PIV were found. In five of the eight fetuses with left outflow tract obstruction but patent mitral valve, PIV was increased. In the other groups there were no obvious changes in Doppler parameters. CONCLUSIONS: We suggest routine examination of the pulmonary veins with pulsed Doppler ultrasound in every fetus with a prenatally diagnosed heart defect. Such Doppler parameters could be used in future as cut-offs for the recently reported in-utero atrial septostomy to decompress an obstructed left atrium.