单纯性完全型肺静脉异位引流产前8例超声心动图诊断

李文秀 耿 斌 吴 江 张桂珍



·论著·

单纯性完全型肺静脉异位引流产前8例超声心动图诊断

李文秀 耿 斌 吴 江 张桂珍

目的 探讨胎儿单纯性完全型肺静脉异位引流(TAPVC)的产前超声心动图特点，提高对本病的产前诊断准确率。方法 回顾性分析2011年5月至2014年2月经新生儿超声心动图、手术或尸解证实的8例单纯性TAPVC的胎儿期超声心动图检查结果，总结超声心动图特征。结果 8例单纯性TAPVC胎儿中，心下型2例，心内型1例，心上型5例，5例存在垂直静脉或共同肺静脉腔与垂直静脉连接处梗阻；8例胎儿中，引产1例(心下型)，余7例均于出生后行手术治疗，其中1例术后死亡(心下型)，6例均恢复良好。TAPVC胎儿超声心动图特征为：①正常的左心房形态消失，呈圆形或椭圆形，左心房光滑并多变小，在孕后期较为明显；②降主动脉与左心房间距离明显增大，多数在左心房后方可见一异常的腔隙(即共同肺静脉腔)，可显示左、右侧上升(心上型)或下降(心下型)的垂直静脉；③妊娠早期左、右心系统比值早期多正常，但妊娠中、晚期(孕26周后)可出现右心系统轻度扩大；④引流入冠状静脉窦时(心内型)，冠状静脉窦可有扩张；引流入上腔静脉时(心上型)，上腔静脉扩张；引流入肝内血管时(心下型)，肝内血管可有不同程度的扩张；⑤彩色多普勒可显示引流途径及是否合并垂直静脉梗阻。结论 在孕早期左、右心系统比值正常时，TAPVC易被漏诊及误诊，应注意多角度、多切面扫查，孕晚期超声心动图检查可减少漏诊。

完全型肺静脉异位引流； 胎儿超声心动图； 产前诊断

随着胎儿产前检查的广泛开展，胎儿超声心动图检查已成为产前超声诊断胎儿先天性心脏病(CHD)的重要手段，但因国内各级医疗机构对各种CHD的预后了解不足，部分出生后临床治疗效果好的CHD胎儿被过度引产[1,2]。完全型肺静脉异位引流(TAPVC)为CHD的一种，是指4支肺静脉均未与左房相连接，而是通过共同肺静脉腔直接或间接回流入右心房。根据是否合并其他心内畸形，TAPVC可分为单纯性和复合性。复合性TAPVC临床多采用姑息性治疗，预后较差[3,4]；而单纯性TAPVC依据分型的不同、诊断和治疗时间的不同，使得出生后手术效果差别较大，心内型和心上型TAPVC患儿手术治疗预后较好，心下型因肺静脉血液回流至右心房行程长，受外界压迫的机会多，容易导致肺静脉引流部位梗阻，产生严重的肺淤血，预后较差[5～7]。国外对单纯性TAPVC报道较少，目前国内尚未见针对单纯性TAPVC的产前超声诊断报道。本研究回顾性分析首都医科大学附属北京安贞医院(我院)诊断单纯性TAPVC胎儿的产前超声心动图特点，以期提高对胎儿期单纯性TAPVC的诊断准确率，为孕妇提供治疗建议 ，降低临床过度引产，并对出生后患儿的超声心动图检查时间及手术前治疗提供建议。

1 方法

1.1 纳入标准 ①2011年5月至2014年2月在我院儿童心血管病中心行胎儿产前超声心动图检查诊断为单纯性TAPVC的连续病例；②经胎儿尸解或出生后经超声心动图或手术证实的病例。

1.2 临床资料截取

1.2.1 孕妇资料 ①在我院行产前超声检查时的孕周(孕周由妊娠末次月经或妊娠早期超声检查确定)；②外院胎儿超声诊断和我院胎儿超声诊断。

1.2.2 胎儿资料 ①左、右心室结构是否对称；②是否存在共同肺静脉腔；③共同肺静脉腔是否存在梗阻；④何种类型TAPVC(心上型、心内型、心下型和混合型)[8]；⑤是否终止妊娠；⑥是否行手术治疗；⑦手术后治疗效果。

1.3 胎儿产前超声心动图检查 使用Philips IE33彩色多普勒超声诊断仪，选择探头C5-2，探头频率2～5 MHz，选择胎儿心脏模式。首先确定胎儿心脏方位，再获取胎儿上腹部胃泡水平横切面，确定胎儿心脏与内脏的位置关系，依次显示胎儿心脏常规5个标准切面：胎儿四腔心切面、胎儿五腔心切面、心底短轴及肺动脉分叉切面、胎儿左心室流出道及主动脉弓长轴切面、胎儿右心室流出道及动脉导管弓长轴切面；再显示三血管切面、三血管气管切面(3VT)、上下腔静脉切面、膈肌水平矢状切面、膈肌水平冠状切面；在这些切面的基础上，再叠加彩色多普勒和能量多普勒血流成像，以观察各房室腔、瓣膜和大血管的血流情况。

1.4 随访及验证 胎儿超声诊断由我院2名高年资医师共同做出，依据医学伦理学原则，均向孕妇及其家属告知胎儿TAPVC的类型、产后检查时间、治疗时间和预后，胎儿父母自主选择继续妊娠或终止妊娠，所有终止妊娠的胎儿于引产后行尸体解剖病理检查，继续妊娠者于产后行新生儿超声心动图检查 。

2 结果

2.1 一般情况 孕妇8名，年龄24～38岁，平均年龄(28.2±4.7)岁；孕周24～36周，平均(29.5±4.3)周；自然受孕6例，体外受精受孕2例；1例胎儿引产行尸解， 2例早产，其中1例36周因胎膜早破早产，1例于36周因羊水过少早产，5例足月产。仅2例在外院诊断为TAPVC(1例诊断为肝静脉异常和心下型TAPVC，1例诊断为心下型TAPVC合并垂直静脉阻塞)，余6例在外院均未诊断为TAPVC(2例诊断为左心发育不全综合征和主动脉狭窄，1例诊断为冠状窦扩大和左上腔静脉，1例诊断为上腔静脉旁异常血管合并狭窄，1例疑诊为左上腔静脉，1例左心系统内径小)。8例胎儿在我院行胎儿超声均诊断为TAPVC，活产的7例均于出生后行手术治疗，其中1例术后1周死亡，其他均恢复良好。8例胎儿及其母亲一般情况见表1。

2.2 胎儿超声心动图检查结果 表1显示，8例胎儿均在左心房后方显示呈卵圆形或狭长的共同肺静脉腔。心上型5例，其中3例共同肺静脉腔通过左侧上行垂直静脉回流入左无名静脉，最终回流入右上腔静脉(2例伴垂直静脉梗阻)；2例通过右侧的垂直静脉直接回流入右上腔静脉(1例伴垂直静脉梗阻)；心内型1例，共同肺静脉腔回流入冠状静脉窦，后回流入右心房；心下型2例，共同肺静脉腔均通过下行的垂直静脉回流入肝内门静脉，其中1例伴垂直静脉梗阻。表1中例1、2、3和4的超声心动图所见分别见文内图1、2、3和4。

2.3 超声心动图特征 ①正常的左心房形态消失(左、右下肺静脉有棱角的结构消失)，左心房光滑，形态呈圆形或椭圆形，左心房多变小，在孕后期较为明显(图1A，2A，3A)；②降主动脉与左心房之间的距离明显增大(图1B，2A，3A,4A)，多数在脊柱与左心房间可见一异常的腔隙，即共同肺静脉腔(图1A，1B，2A，2B，3A，3B,4A)，可显示左、右侧上升(心上型)或下降(心下型)的垂直静脉(三血管及膈肌水平矢状切面)(图1E，图2D)；③左、右心系统比值早期多正常(图4A)，但妊娠中、晚期(26周后)可出现右心系统轻度扩大，左心室与右心室比值或主动脉与肺动脉比值减小(图1A，2A，3A)；④引流入冠状静脉窦时(心内型)，冠状静脉窦可有扩张(图1C，E)，引流入上腔静脉时(心上型)，上腔静脉扩张(图2C，2D,4C)，引流入肝内血管时(门静脉、肝静脉或下腔静脉肝内段)(心下型)，肝内血管不同程度的扩张(图1E)；⑤彩色多普勒可显示引流途径及是否合并梗阻(图1A，1D，2C～E，3D，3F，4A)。

1 29周胎儿心下型TAPVC伴垂直静脉梗阻超声心动图(例1)

Fig 1 Findings of infracardiac TAPVC to portal vein with obstruction of descending vertical vein at GA 29 weeks (case 1)

Notes A:Two-dimensional ultrasound image showed the ′confluence′(red arrow) of pulmonary veins posterior to the left atrium (LA) and anterior to the descending aorta (Dao) on fetal four-chamber view with asymmetry ventricle at GA 29 weeks. B: Fetal four-chamber view showed the pulmonary venous confluence (CPV) (red arrows) and increased distance between LA and Dao. C:Color Doppler imaging showed the CPV which left pulmonary venous (LPV) and right pulmonary venous (RPV) draining blood into it. D:Power Doppler imaging also showed the CPV clearly. E:Two-dimensional ultrasound image showed the CPV connected portal vein (POV) through the descending vertical vein (DVV) on fetal coronal view. F: Color Doppler imaging showed DVV draining blood away from the heart and into the POV. G: Fetal sagittal view showed color flow when the DVV passing through the diaphragm (red arrow). H:Spectral Doppler demonstrated the velocity of obstruction of DVV was 90 cm·s-1

2 31周胎儿心上型TAPVC伴共同肺静脉腔与左侧垂直静脉连接处梗阻超声心动图(例2)

Fig 2 Findings of supracardiac TAPVC to the innominate vein in a fetus with the stenosis at the connection of pulmonary venous confluence to the left aescending vertical vein at GA 31 weeks(case 2)

Notes A:Two-dimensional ultrasound image of fetal four-chamber view showed the pulmonary venous confluence (CPV) (red arrow) between the left atrium (LA) and the descending aorta (Dao), and left atrium (LA) was became narrow at 31 weeks. B: CPV(red arrow) was seen posterior to the LA on the approximate apex long axis view of left ventricle outlet tract (LVOT). C: Color Doppler imaging showed the flow direction of CPV was upward and an accelerated flow (white arrow) at the site of CPV near the pulmonary artery (PA). D: Color Doppler imaging showed CPV connected to the left innominate vein (LIV) by the left ascending vertical vein (L-VV), and a vein arch was made with a stenosis (red arrow) at the connection of CPV to L-VV. E: Power Doppler imaging showed the vein arch clearly. LV: left ventricle; RA: right atrium; RV: right ventricle; LIV: left innominate vein; L-VV: left vertical vein; SVC: superior vena cava

3 31周胎儿心内型TAPVC超声心动图(例3)

Fig 3 Findings of cardiac TAPVC in a fetus at GA 31 weeks (case 3)

Notes A:Two-dimensional ultrasound image showed the suspected pulmonary venous confluence (CPV) (red arrows) posterior to the left atrium (LA) on fetal four-chamber view, and the right heart was bigger and LA was small. B: Color Doppler imaging showed the site of pulmonary veins draining blood flow was close to the posterior wall of LA (red arrows), but the CPV was not very clear. C: Two-dimensional ultrasound image demonstrated a dilated coronary sinus (CS) (red arrow); D: Color Doppler imaging showed the blood flow (white arrow) from CS to right atrium (RA). E: Two-dimensional ultrasound image demonstrated a dilated CS which connected left and right pulmonary vein (red arrows) on the CS sagittal view. F: Color Doppler imaging showed the blood flow of left and right pulmonary vein draining into CS. G: The three vessels and trachea view (3VT) demonstrated the diameter of superior vena cava (SVC) was normal and the linear arrangement of pulmonary artery (PA), aorta (AO) and SVC was normal, and this view did not show left superior vena cava (LSVC). The supracardiac TAPVC was excluded. Dao: descending aorta; LV: left ventricle; RV: right ventricle;PFO: patent foramen; AO: aorta

4 26周胎儿心上型APVC伴垂直静脉梗阻超声心动图(例4)

Fig 4 Findings of supracardiac TAPVC to SVC with obstruction of ascending vertical vein at GA 26 weeks(case 4)

Notes A:Fetal four-chamber view with symmetry ventricle at 26 weeks, showing two signs of TAPVC: the presence of a small pulmonary venous confluence (CPV) posterior to the left atrium (LA), and increased distance between LA and the descending aorta (Dao) (red arrow). B: A short ascending vertical vein (red arrow) was shown between CPV and right superior vena cava (SVC). C: Color Doppler imaging showed flow turbulence at the site of obstruction of ascending vertical vein (white arrow). D: Spectral Doppler demonstrated high-velocity continuous flow at the junction of the vertical vein to the SVC. The velocity was 180 cm·s-1.

3 讨论

随着胎儿超声心动图的广泛开展，各种CHD的产前检出率及诊断准确率不断增加，但单纯性TAPVC的发病率较低，加之多数超声检查医师对本病在不同孕期的超声心动图表现认识不足，因此产前诊断单纯性TAPVC的报道较少[9～11]。尽管在出生后多数患儿可被准确诊断，但由于多数患儿在出生后需使用药物治疗以保持动脉导管的持续开放，而且不同类型单纯性TAPVC的手术治疗效果差异较大，TAPVC的类型、是否合并共同静脉腔梗阻和治疗的年龄是影响手术成功的关键[12,13]。因此产前对本病做出准确的诊断对患儿出生后的治疗及指导优生优育均有重要的意义。

在正常妊娠的早期及中期，由于胎儿尚未建立呼吸，双肺未膨胀，肺循环的血流量只占右心系统排血量的7%左右，通过肺静脉引入左心房，其他93%的血流量通过开放的动脉导管进入体循环，同时由于胎儿的血氧交换是通过母体的胎盘而不是肺组织，因此即使胎儿期存在完全型肺静脉异位回流入右心房，通常也不会引起明显的胎儿血流动力学异常，左、右心系统比值正常；妊娠晚期存在TAPVC的胎儿，20%～25%的左右心室(混合)排血量进入肺组织，随着肺循环血流量逐渐增加，可占到右心系统排血量的40%～50%，因此孕晚期胎儿的右心系统(管状静脉、上腔静脉)扩张明显，左心系统缩小[14,15]。

本研究8例TAPVC胎儿超声心动图检查显示，心内结构、房室及大动脉连接、大动脉关系均未见明显异常。其中例5、6和8为孕27周前来我院会诊的胎儿，心下型、心上型和心上型伴垂直静脉梗阻各1例，就诊时孕周分别为24、25和26周。例6心下型TAPVC胎儿在外院诊断为：肝脏内异常血管，心下型TAPVC不除外；在我院行超声心动图检查显示，左、右心系统比值接近正常，但在膈肌水平矢状切面显示在肝脏内与门静脉系统相连接的异常血管，通过彩色多普勒、频谱多普勒及能量多普勒模式的检查，显示该异常血管为静脉血管，其血流方向与降主动脉血流方向一致，为下行的垂直静脉，四腔心切面显示降主动脉与左心房之间的距离明显增大，经多角度反复扫查确认脊柱与左心房间存在一狭长的腔隙，即异常回流的肺静脉形成的共同肺静脉腔；引产后胎儿尸解证实了超声心动图所见。例1和4心上型TAPVC胎儿在我院超声心动图检查显示左、右心系统比值接近正常，例4在外院诊断为降主动脉旁异常血管，考虑为永存左上腔静脉，我院检查确认降主动脉旁的异常血管内为静脉样频谱，血流与降主动脉血流方向相反，为上行的血流，考虑为上行的垂直静脉，该垂直静脉回流通畅，并回流入左侧的无名静脉，最终回流入右上腔静脉，导致无名静脉及右上腔静脉内径增宽，多角度反复扫查在左心房后方显示了共同肺静脉腔；例1胎儿在外院诊断为右侧上腔静脉旁异常血管并狭窄，我院超声心动图显示脊柱与左心房间距离增大，其间可见一共同肺静脉腔，共同肺静脉腔通过一短小的上行垂直静脉与右侧的上腔静脉相交通，垂直静脉汇入上腔静脉处梗阻，彩色多普勒显示梗阻处呈花色血流信号，上腔静脉局部血管腔膨大，最后诊断为单纯性TAPVC(心上型)合并垂直静脉梗阻。例1和4心上型TAPVC胎儿出生后新生儿期均在我院进行了超声心动图检查及手术治疗，均证实了产前超声心动图的诊断，术后随访患儿恢复良好。例1和4心上型TAPVC的胎儿在外院误诊原因考虑与孕妇就诊时胎儿左、右心系统比值接近正常，与孕晚期及出生后的TAPVC超声表现存在明显的不同，加之超声检查医师对本病的认识有限有关。

TAPVC胎儿在妊娠早期多不出现左、右心系统比例的失调，考虑其可能的原因为：①早期肺静脉血流较少，不足以引起右心系统增大；②垂直静脉回流梗阻可致肺静脉回流梗阻，从而使肺动脉阻力增加，而经开放的动脉导管进入体循环的血流量增多，肺血量减少，回流入右心系统的血流量减少；③心下型肺静脉异位引流的血流通过门静脉系统最终进入下腔静脉后，通过开放的卵圆孔回流入左心房，当卵圆孔较大或存在比较大的房间隔交通时，可使左心系统的血流量减少不明显，最终左、右心系统比例基本正常[16]。

本研究另5例TAPVC胎儿均是孕29周后来我院会诊，其中例1为心下型，就诊时孕29周；例3为心内型，就诊时孕31周；例2、5和7为心上型，就诊时孕周分别为36、31和34周。这5例胎儿在就诊时超声心动图均有不同程度的左、右心系统比值异常，孕周越大，右心系统扩大越明显，主动脉与肺动脉比值越小。例1和6心下型胎儿的超声心动图表现相似，均为肝脏内与门静脉连接的异常静脉血管，但例1下行垂直静脉在穿膈肌处梗阻，彩色多普勒显示该处血流呈花色信号，流速增快，外院也考虑为心下型TAPVC合并下行垂直静脉梗阻，我院检查结果与外院诊断基本一致。本研究仅有例3为心内型TAPVC，外院误诊为冠状静脉窦增宽，永存左上腔静脉不除外，在我院反复扫查显示冠状静脉窦增宽并不是由于永存左上腔静脉汇入造成，例3无明确的共同肺静脉腔，仅见左、右肺静脉血流回流入冠状静脉窦，并最终回流入右心房，因此冠状静脉窦明显增宽。例2在当地医院检查以左心系统内径小来我院会诊，我院超声心动图检查显示在左心房后方可见一狭长的共同肺静脉腔，其与左侧上行垂直静脉连接，并且连接处内径狭窄，彩色多普勒显示该处血流增快，左侧垂直静脉上行回流入左无名静脉，最终回流入右侧的上腔静脉，导致右上腔静脉和无名静脉内径增宽，诊断为心上型TAPVC并共同肺静脉腔与垂直静脉连接处梗阻；例5和7心上型胎儿就诊时孕周较大，典型的表现为右心系统扩大明显，左心系统内径小，主动脉与肺动脉比值减小，在外院均被误诊为左心室发育不良，主动脉缩窄，来我院会诊后均首先排除了左心室发育不良的诊断，我院超声心动图反复扫查显示胎儿的二尖瓣及主动脉均未见异常，开放及启闭良好，血流未见异常，因此考虑左心系统内径小为回流入左心房内的血流量减少所导致，此时正常的左心房形态消失(左右下肺静脉有棱角的结构消失)，左心房变得光滑，形态呈圆形或椭圆形，其中例5在降主动脉旁可见一上行的静脉血管，其走行至与左肺动脉相交叉的位置处可见局部血流加速所致的花色血流信号，最后这支异常血管的血流注入到左侧的无名静脉，并最终回流入右上腔静脉，致右上腔静脉及无名静脉内径增宽，根据这一征象最后诊断为心上型TAPVC并垂直静脉梗阻；例7经反复扫查在降主动脉旁并无上行的垂直静脉，当时初步考虑为主动脉缩窄所致右心系统扩大，但反复扫查后显示右侧的奇静脉旁存在一上行的静脉样血管，其血流通畅，并回流入右上腔静脉，最终回流入右心房，根据这些征象最后诊断为心上型TAPVC；例5和7均在左心房的后方可见一共同静脉腔，但因为孕周较大，胎儿体位受限，共同静脉腔显示非常困难。这5例孕29周后来我院会诊的胎儿出生后均在我院进行了超声心动图检查，证实了产前超声心动图检查的诊断，并均进行了手术治疗，例1心下型患儿于术后1周死亡，其他患儿术后随访恢复良好。

通过分析总结本文病例及查阅相关文献，认为在胎儿超声心动图检查过程中，应利用二维、彩色多普勒和能量多普勒仔细观察肺静脉。由于胎儿期肺循环血流量非常少，而且胎儿肺静脉管壁薄、管腔细小、血流速度低，所以超声检查胎儿肺静脉具有一定的困难，但胎儿期肺脏没有呼吸功能，肺组织呈塌陷状态，肺内无气体的干扰，肺静脉血流的显示相对成人较容易，而能量多普勒及高清彩色多普勒有助于显示肺静脉[17,18]，因此在检查过程中至少应观察到1支肺静脉引流入左心房，因为只有TAPVC对新生儿的生命构成威胁，只要识别到1支肺静脉进入左心房就可以排除TAPVC的诊断。

TAPVC胎儿在孕晚期最常见的间接征象是右心系统增大，左、右心系统比例减小，但部分正常胎儿作为一种正常生理的变异，在妊娠晚期也常出现上述征象，因此应注意鉴别[19]；此外在胎儿左心系统梗阻性畸形，如主动脉缩窄、主动脉弓离断、左心发育不良综合征等也常见到上述征象，这些因素都是导致TAPVC诊断假阳性或假阴性的常见原因[20]，此外本病须注意与永存左上腔静脉及引起肺静脉梗阻的三房心相鉴别[11]。胎儿期存在永存左上腔静脉时心脏左右系统内径比值正常，而且左心房的形态正常，利用彩色多普勒和能量多普勒可以显示肺静脉回流入左心房而非冠状静脉窦；引起肺静脉梗阻的三房心在胎儿期可以显示左心房内的异常膜状样回声，膜状回声存在交通口，彩色多普勒显示交通口处血流呈花色信号，而且肺静脉仍回流入左心房。

单纯性TAPVC虽然临床发病率低，但是仍具有其特征性的超声心动图特点。心下型TAPVC由于在肝内存在异常走行的血管，因此不易被误诊及漏诊；由于胎儿在母体内体位多变，再加上颈部的血管较多，胎儿期不易辨认，因此心上型和心内型TAPVC在临床的诊断中仍然存在一定的困难，容易造成漏诊及误诊，这就要求检查者完成心脏常规扫查切面后，注意有无异常的血管，并对异常走行的血管进行多切面、多角度的扫查，并要对本病不同孕周的胎儿血流动力学特点及本病异位引流的途径有全面的认识，以提高本病的产前诊断率。

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(本文编辑：张萍)

Prenatal diagnosis of isolated total anomalous pulmonary venous connection by fetal echocardiography

LIWen-xiu,GENGBin,WUJiang,ZHANGGui-zhen
(PediatricCardiovascularCenter,BeijingAnzhenHospitalaffiliatedtotheCapitalMedicalUniversity,Beijing100029,China)

GENG Bin，E-mail: geng_bin1@163.com

ObjectiveTo improve the prenatal diagnosis accuracy of isolated total anomalous pulmonary venous connection (TAPVC) by analyzing and accumulating fetal echocardiography features accurately diagnosed by fetal echocardiography.MethodsFetal echocardiographic signs and accumulated fetal echocardiography features were retrospectively analyzed in 8 cases with prenatal diagnosis of isolated TAPVC which was confirmed by neonatal echocardiography, surgery or autopsy from May 2011 to February 2014.ResultsDiagnosis of TAPVC was made in 8 fetuses, including 5 with supracardiac connection, 2 with infracardiac connection and 1 with cardiac connection. Only 2 fetuses were diagnosed TAPVC in other hospitals before, including 1 fetus diagnosed as abnormal vessel in liver and infracardiac TAPVC, 1 fetus diagnosed as infracardiac TAPVC combined with descending vertical vein obstruction. Other 6 fetuses were all misdiagnosed, including 2 with hypoplastic left heart syndrome and coarctation of aorta, 1 with dilated coronary sinus and left superior vena cava, 1 with abnormal vessal nearby superior vena cava combined with stenosis, 1 with left superior vena cava and 1 with small diameter of left heart. Stenosis along the vertical vein pathway or the connection of the vertical vein to pulmonary venous confluence was identified in 5 fetuses. In 1 terminated of pregnancy case with infracardiac connection, autopsy confirmed the prenatal diagnosis. Other 7 fetuses were born and performed surgery after birth, 1 case with infracardiac connection died in 1 week after surgery and other cases recovered well. The echocardiography characters of fetuses diagnosed with TAPVC：① The normal shape of left atrium disappeared and left atrium became round or oral and narrow especially in the latter phase of pregnancy. ② The distance was increased between left atrium and the descending aorta (Dao) and an abnormal pulmonary venous confluence presented posterior to the left atrium in most cases, and the ascending vertical vein or descending vertical vein could be seen. ③ The ventricular proportion was normal at the earliest gestation, but the right heart was dilated after 26 weeks. ④ A dilated coronary sinus could be seen in fetuses with cardiac TAPVC and a dilated superior vena cava in the fetus with supracardiac TAPVC and the dilated hepatic vessel in infracardiac TAPVC fetuses as well. ⑤ Color Doppler imaging could show the draining trace and whether existed vertical vein obstruction.ConclusionThe isolated TAPVC is easy to be misdiagnosed when the ventricular proportion is normal at the earliest gestation. More attention should be paid on more angle and more exam views when performing fetal echocardiography. The fetal echocardiography can reduce misdiagnosis at the latter phase of pregnancy.

Total anomalous pulmonary venous connection; Fetal echocardiography; Prenatal diagnosis

首都医科大学附属北京安贞医院儿童心血管病中心 北京，100029

耿斌，E-mail:geng_bin1@163.com

10.3969/j.issn.1673-5501.2015.02.012

2014-11-20

2015-01-17)