李文秀 耿 斌 吳 江 張桂珍

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·論著·

單純性完全型肺靜脈異位引流產(chǎn)前8例超聲心動(dòng)圖診斷

李文秀 耿 斌 吳 江 張桂珍

目的 探討胎兒?jiǎn)渭冃酝耆头戊o脈異位引流(TAPVC)的產(chǎn)前超聲心動(dòng)圖特點(diǎn)，提高對(duì)本病的產(chǎn)前診斷準(zhǔn)確率。方法 回顧性分析2011年5月至2014年2月經(jīng)新生兒超聲心動(dòng)圖、手術(shù)或尸解證實(shí)的8例單純性TAPVC的胎兒期超聲心動(dòng)圖檢查結(jié)果，總結(jié)超聲心動(dòng)圖特征。結(jié)果 8例單純性TAPVC胎兒中，心下型2例，心內(nèi)型1例，心上型5例，5例存在垂直靜脈或共同肺靜脈腔與垂直靜脈連接處梗阻；8例胎兒中，引產(chǎn)1例(心下型)，余7例均于出生后行手術(shù)治療，其中1例術(shù)后死亡(心下型)，6例均恢復(fù)良好。TAPVC胎兒超聲心動(dòng)圖特征為：①正常的左心房形態(tài)消失，呈圓形或橢圓形，左心房光滑并多變小，在孕后期較為明顯；②降主動(dòng)脈與左心房間距離明顯增大，多數(shù)在左心房后方可見(jiàn)一異常的腔隙(即共同肺靜脈腔)，可顯示左、右側(cè)上升(心上型)或下降(心下型)的垂直靜脈；③妊娠早期左、右心系統(tǒng)比值早期多正常，但妊娠中、晚期(孕26周后)可出現(xiàn)右心系統(tǒng)輕度擴(kuò)大；④引流入冠狀靜脈竇時(shí)(心內(nèi)型)，冠狀靜脈竇可有擴(kuò)張；引流入上腔靜脈時(shí)(心上型)，上腔靜脈擴(kuò)張；引流入肝內(nèi)血管時(shí)(心下型)，肝內(nèi)血管可有不同程度的擴(kuò)張；⑤彩色多普勒可顯示引流途徑及是否合并垂直靜脈梗阻。結(jié)論 在孕早期左、右心系統(tǒng)比值正常時(shí)，TAPVC易被漏診及誤診，應(yīng)注意多角度、多切面掃查，孕晚期超聲心動(dòng)圖檢查可減少漏診。

完全型肺靜脈異位引流； 胎兒超聲心動(dòng)圖； 產(chǎn)前診斷

隨著胎兒產(chǎn)前檢查的廣泛開(kāi)展，胎兒超聲心動(dòng)圖檢查已成為產(chǎn)前超聲診斷胎兒先天性心臟病(CHD)的重要手段，但因國(guó)內(nèi)各級(jí)醫(yī)療機(jī)構(gòu)對(duì)各種CHD的預(yù)后了解不足，部分出生后臨床治療效果好的CHD胎兒被過(guò)度引產(chǎn)[1,2]。完全型肺靜脈異位引流(TAPVC)為CHD的一種，是指4支肺靜脈均未與左房相連接，而是通過(guò)共同肺靜脈腔直接或間接回流入右心房。根據(jù)是否合并其他心內(nèi)畸形，TAPVC可分為單純性和復(fù)合性。復(fù)合性TAPVC臨床多采用姑息性治療，預(yù)后較差[3,4]；而單純性TAPVC依據(jù)分型的不同、診斷和治療時(shí)間的不同，使得出生后手術(shù)效果差別較大，心內(nèi)型和心上型TAPVC患兒手術(shù)治療預(yù)后較好，心下型因肺靜脈血液回流至右心房行程長(zhǎng)，受外界壓迫的機(jī)會(huì)多，容易導(dǎo)致肺靜脈引流部位梗阻，產(chǎn)生嚴(yán)重的肺淤血，預(yù)后較差[5～7]。國(guó)外對(duì)單純性TAPVC報(bào)道較少，目前國(guó)內(nèi)尚未見(jiàn)針對(duì)單純性TAPVC的產(chǎn)前超聲診斷報(bào)道。本研究回顧性分析首都醫(yī)科大學(xué)附屬北京安貞醫(yī)院(我院)診斷單純性TAPVC胎兒的產(chǎn)前超聲心動(dòng)圖特點(diǎn)，以期提高對(duì)胎兒期單純性TAPVC的診斷準(zhǔn)確率，為孕婦提供治療建議 ，降低臨床過(guò)度引產(chǎn)，并對(duì)出生后患兒的超聲心動(dòng)圖檢查時(shí)間及手術(shù)前治療提供建議。

1 方法

1.1 納入標(biāo)準(zhǔn) ①2011年5月至2014年2月在我院兒童心血管病中心行胎兒產(chǎn)前超聲心動(dòng)圖檢查診斷為單純性TAPVC的連續(xù)病例；②經(jīng)胎兒尸解或出生后經(jīng)超聲心動(dòng)圖或手術(shù)證實(shí)的病例。

1.2 臨床資料截取

1.2.1 孕婦資料 ①在我院行產(chǎn)前超聲檢查時(shí)的孕周(孕周由妊娠末次月經(jīng)或妊娠早期超聲檢查確定)；②外院胎兒超聲診斷和我院胎兒超聲診斷。

1.2.2 胎兒資料 ①左、右心室結(jié)構(gòu)是否對(duì)稱；②是否存在共同肺靜脈腔；③共同肺靜脈腔是否存在梗阻；④何種類型TAPVC(心上型、心內(nèi)型、心下型和混合型)[8]；⑤是否終止妊娠；⑥是否行手術(shù)治療；⑦手術(shù)后治療效果。

1.3 胎兒產(chǎn)前超聲心動(dòng)圖檢查 使用Philips IE33彩色多普勒超聲診斷儀，選擇探頭C5-2，探頭頻率2～5 MHz，選擇胎兒心臟模式。首先確定胎兒心臟方位，再獲取胎兒上腹部胃泡水平橫切面，確定胎兒心臟與內(nèi)臟的位置關(guān)系，依次顯示胎兒心臟常規(guī)5個(gè)標(biāo)準(zhǔn)切面：胎兒四腔心切面、胎兒五腔心切面、心底短軸及肺動(dòng)脈分叉切面、胎兒左心室流出道及主動(dòng)脈弓長(zhǎng)軸切面、胎兒右心室流出道及動(dòng)脈導(dǎo)管弓長(zhǎng)軸切面；再顯示三血管切面、三血管氣管切面(3VT)、上下腔靜脈切面、膈肌水平矢狀切面、膈肌水平冠狀切面；在這些切面的基礎(chǔ)上，再疊加彩色多普勒和能量多普勒血流成像，以觀察各房室腔、瓣膜和大血管的血流情況。

1.4 隨訪及驗(yàn)證 胎兒超聲診斷由我院2名高年資醫(yī)師共同做出，依據(jù)醫(yī)學(xué)倫理學(xué)原則，均向孕婦及其家屬告知胎兒TAPVC的類型、產(chǎn)后檢查時(shí)間、治療時(shí)間和預(yù)后，胎兒父母自主選擇繼續(xù)妊娠或終止妊娠，所有終止妊娠的胎兒于引產(chǎn)后行尸體解剖病理檢查，繼續(xù)妊娠者于產(chǎn)后行新生兒超聲心動(dòng)圖檢查 。

2 結(jié)果

2.1 一般情況 孕婦8名，年齡24～38歲，平均年齡(28.2±4.7)歲；孕周24～36周，平均(29.5±4.3)周；自然受孕6例，體外受精受孕2例；1例胎兒引產(chǎn)行尸解， 2例早產(chǎn)，其中1例36周因胎膜早破早產(chǎn)，1例于36周因羊水過(guò)少早產(chǎn)，5例足月產(chǎn)。僅2例在外院診斷為T(mén)APVC(1例診斷為肝靜脈異常和心下型TAPVC，1例診斷為心下型TAPVC合并垂直靜脈阻塞)，余6例在外院均未診斷為T(mén)APVC(2例診斷為左心發(fā)育不全綜合征和主動(dòng)脈狹窄，1例診斷為冠狀竇擴(kuò)大和左上腔靜脈，1例診斷為上腔靜脈旁異常血管合并狹窄，1例疑診為左上腔靜脈，1例左心系統(tǒng)內(nèi)徑小)。8例胎兒在我院行胎兒超聲均診斷為T(mén)APVC，活產(chǎn)的7例均于出生后行手術(shù)治療，其中1例術(shù)后1周死亡，其他均恢復(fù)良好。8例胎兒及其母親一般情況見(jiàn)表1。

2.2 胎兒超聲心動(dòng)圖檢查結(jié)果 表1顯示，8例胎兒均在左心房后方顯示呈卵圓形或狹長(zhǎng)的共同肺靜脈腔。心上型5例，其中3例共同肺靜脈腔通過(guò)左側(cè)上行垂直靜脈回流入左無(wú)名靜脈，最終回流入右上腔靜脈(2例伴垂直靜脈梗阻)；2例通過(guò)右側(cè)的垂直靜脈直接回流入右上腔靜脈(1例伴垂直靜脈梗阻)；心內(nèi)型1例，共同肺靜脈腔回流入冠狀靜脈竇，后回流入右心房；心下型2例，共同肺靜脈腔均通過(guò)下行的垂直靜脈回流入肝內(nèi)門(mén)靜脈，其中1例伴垂直靜脈梗阻。表1中例1、2、3和4的超聲心動(dòng)圖所見(jiàn)分別見(jiàn)文內(nèi)圖1、2、3和4。

2.3 超聲心動(dòng)圖特征 ①正常的左心房形態(tài)消失(左、右下肺靜脈有棱角的結(jié)構(gòu)消失)，左心房光滑，形態(tài)呈圓形或橢圓形，左心房多變小，在孕后期較為明顯(圖1A，2A，3A)；②降主動(dòng)脈與左心房之間的距離明顯增大(圖1B，2A，3A,4A)，多數(shù)在脊柱與左心房間可見(jiàn)一異常的腔隙，即共同肺靜脈腔(圖1A，1B，2A，2B，3A，3B,4A)，可顯示左、右側(cè)上升(心上型)或下降(心下型)的垂直靜脈(三血管及膈肌水平矢狀切面)(圖1E，圖2D)；③左、右心系統(tǒng)比值早期多正常(圖4A)，但妊娠中、晚期(26周后)可出現(xiàn)右心系統(tǒng)輕度擴(kuò)大，左心室與右心室比值或主動(dòng)脈與肺動(dòng)脈比值減小(圖1A，2A，3A)；④引流入冠狀靜脈竇時(shí)(心內(nèi)型)，冠狀靜脈竇可有擴(kuò)張(圖1C，E)，引流入上腔靜脈時(shí)(心上型)，上腔靜脈擴(kuò)張(圖2C，2D,4C)，引流入肝內(nèi)血管時(shí)(門(mén)靜脈、肝靜脈或下腔靜脈肝內(nèi)段)(心下型)，肝內(nèi)血管不同程度的擴(kuò)張(圖1E)；⑤彩色多普勒可顯示引流途徑及是否合并梗阻(圖1A，1D，2C～E，3D，3F，4A)。

1 29周胎兒心下型TAPVC伴垂直靜脈梗阻超聲心動(dòng)圖(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周胎兒心上型TAPVC伴共同肺靜脈腔與左側(cè)垂直靜脈連接處梗阻超聲心動(dòng)圖(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周胎兒心內(nèi)型TAPVC超聲心動(dòng)圖(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周胎兒心上型APVC伴垂直靜脈梗阻超聲心動(dòng)圖(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 討論

隨著胎兒超聲心動(dòng)圖的廣泛開(kāi)展，各種CHD的產(chǎn)前檢出率及診斷準(zhǔn)確率不斷增加，但單純性TAPVC的發(fā)病率較低，加之多數(shù)超聲檢查醫(yī)師對(duì)本病在不同孕期的超聲心動(dòng)圖表現(xiàn)認(rèn)識(shí)不足，因此產(chǎn)前診斷單純性TAPVC的報(bào)道較少[9～11]。盡管在出生后多數(shù)患兒可被準(zhǔn)確診斷，但由于多數(shù)患兒在出生后需使用藥物治療以保持動(dòng)脈導(dǎo)管的持續(xù)開(kāi)放，而且不同類型單純性TAPVC的手術(shù)治療效果差異較大，TAPVC的類型、是否合并共同靜脈腔梗阻和治療的年齡是影響手術(shù)成功的關(guān)鍵[12,13]。因此產(chǎn)前對(duì)本病做出準(zhǔn)確的診斷對(duì)患兒出生后的治療及指導(dǎo)優(yōu)生優(yōu)育均有重要的意義。

在正常妊娠的早期及中期，由于胎兒尚未建立呼吸，雙肺未膨脹，肺循環(huán)的血流量只占右心系統(tǒng)排血量的7%左右，通過(guò)肺靜脈引入左心房，其他93%的血流量通過(guò)開(kāi)放的動(dòng)脈導(dǎo)管進(jìn)入體循環(huán)，同時(shí)由于胎兒的血氧交換是通過(guò)母體的胎盤(pán)而不是肺組織，因此即使胎兒期存在完全型肺靜脈異位回流入右心房，通常也不會(huì)引起明顯的胎兒血流動(dòng)力學(xué)異常，左、右心系統(tǒng)比值正常；妊娠晚期存在TAPVC的胎兒，20%～25%的左右心室(混合)排血量進(jìn)入肺組織，隨著肺循環(huán)血流量逐漸增加，可占到右心系統(tǒng)排血量的40%～50%，因此孕晚期胎兒的右心系統(tǒng)(管狀靜脈、上腔靜脈)擴(kuò)張明顯，左心系統(tǒng)縮小[14,15]。

本研究8例TAPVC胎兒超聲心動(dòng)圖檢查顯示，心內(nèi)結(jié)構(gòu)、房室及大動(dòng)脈連接、大動(dòng)脈關(guān)系均未見(jiàn)明顯異常。其中例5、6和8為孕27周前來(lái)我院會(huì)診的胎兒，心下型、心上型和心上型伴垂直靜脈梗阻各1例，就診時(shí)孕周分別為24、25和26周。例6心下型TAPVC胎兒在外院診斷為：肝臟內(nèi)異常血管，心下型TAPVC不除外；在我院行超聲心動(dòng)圖檢查顯示，左、右心系統(tǒng)比值接近正常，但在膈肌水平矢狀切面顯示在肝臟內(nèi)與門(mén)靜脈系統(tǒng)相連接的異常血管，通過(guò)彩色多普勒、頻譜多普勒及能量多普勒模式的檢查，顯示該異常血管為靜脈血管，其血流方向與降主動(dòng)脈血流方向一致，為下行的垂直靜脈，四腔心切面顯示降主動(dòng)脈與左心房之間的距離明顯增大，經(jīng)多角度反復(fù)掃查確認(rèn)脊柱與左心房間存在一狹長(zhǎng)的腔隙，即異常回流的肺靜脈形成的共同肺靜脈腔；引產(chǎn)后胎兒尸解證實(shí)了超聲心動(dòng)圖所見(jiàn)。例1和4心上型TAPVC胎兒在我院超聲心動(dòng)圖檢查顯示左、右心系統(tǒng)比值接近正常，例4在外院診斷為降主動(dòng)脈旁異常血管，考慮為永存左上腔靜脈，我院檢查確認(rèn)降主動(dòng)脈旁的異常血管內(nèi)為靜脈樣頻譜，血流與降主動(dòng)脈血流方向相反，為上行的血流，考慮為上行的垂直靜脈，該垂直靜脈回流通暢，并回流入左側(cè)的無(wú)名靜脈，最終回流入右上腔靜脈，導(dǎo)致無(wú)名靜脈及右上腔靜脈內(nèi)徑增寬，多角度反復(fù)掃查在左心房后方顯示了共同肺靜脈腔；例1胎兒在外院診斷為右側(cè)上腔靜脈旁異常血管并狹窄，我院超聲心動(dòng)圖顯示脊柱與左心房間距離增大，其間可見(jiàn)一共同肺靜脈腔，共同肺靜脈腔通過(guò)一短小的上行垂直靜脈與右側(cè)的上腔靜脈相交通，垂直靜脈匯入上腔靜脈處梗阻，彩色多普勒顯示梗阻處呈花色血流信號(hào)，上腔靜脈局部血管腔膨大，最后診斷為單純性TAPVC(心上型)合并垂直靜脈梗阻。例1和4心上型TAPVC胎兒出生后新生兒期均在我院進(jìn)行了超聲心動(dòng)圖檢查及手術(shù)治療，均證實(shí)了產(chǎn)前超聲心動(dòng)圖的診斷，術(shù)后隨訪患兒恢復(fù)良好。例1和4心上型TAPVC的胎兒在外院誤診原因考慮與孕婦就診時(shí)胎兒左、右心系統(tǒng)比值接近正常，與孕晚期及出生后的TAPVC超聲表現(xiàn)存在明顯的不同，加之超聲檢查醫(yī)師對(duì)本病的認(rèn)識(shí)有限有關(guān)。

TAPVC胎兒在妊娠早期多不出現(xiàn)左、右心系統(tǒng)比例的失調(diào)，考慮其可能的原因?yàn)椋孩僭缙诜戊o脈血流較少，不足以引起右心系統(tǒng)增大；②垂直靜脈回流梗阻可致肺靜脈回流梗阻，從而使肺動(dòng)脈阻力增加，而經(jīng)開(kāi)放的動(dòng)脈導(dǎo)管進(jìn)入體循環(huán)的血流量增多，肺血量減少，回流入右心系統(tǒng)的血流量減少；③心下型肺靜脈異位引流的血流通過(guò)門(mén)靜脈系統(tǒng)最終進(jìn)入下腔靜脈后，通過(guò)開(kāi)放的卵圓孔回流入左心房，當(dāng)卵圓孔較大或存在比較大的房間隔交通時(shí)，可使左心系統(tǒng)的血流量減少不明顯，最終左、右心系統(tǒng)比例基本正常[16]。

本研究另5例TAPVC胎兒均是孕29周后來(lái)我院會(huì)診，其中例1為心下型，就診時(shí)孕29周；例3為心內(nèi)型，就診時(shí)孕31周；例2、5和7為心上型，就診時(shí)孕周分別為36、31和34周。這5例胎兒在就診時(shí)超聲心動(dòng)圖均有不同程度的左、右心系統(tǒng)比值異常，孕周越大，右心系統(tǒng)擴(kuò)大越明顯，主動(dòng)脈與肺動(dòng)脈比值越小。例1和6心下型胎兒的超聲心動(dòng)圖表現(xiàn)相似，均為肝臟內(nèi)與門(mén)靜脈連接的異常靜脈血管，但例1下行垂直靜脈在穿膈肌處梗阻，彩色多普勒顯示該處血流呈花色信號(hào)，流速增快，外院也考慮為心下型TAPVC合并下行垂直靜脈梗阻，我院檢查結(jié)果與外院診斷基本一致。本研究?jī)H有例3為心內(nèi)型TAPVC，外院誤診為冠狀靜脈竇增寬，永存左上腔靜脈不除外，在我院反復(fù)掃查顯示冠狀靜脈竇增寬并不是由于永存左上腔靜脈匯入造成，例3無(wú)明確的共同肺靜脈腔，僅見(jiàn)左、右肺靜脈血流回流入冠狀靜脈竇，并最終回流入右心房，因此冠狀靜脈竇明顯增寬。例2在當(dāng)?shù)蒯t(yī)院檢查以左心系統(tǒng)內(nèi)徑小來(lái)我院會(huì)診，我院超聲心動(dòng)圖檢查顯示在左心房后方可見(jiàn)一狹長(zhǎng)的共同肺靜脈腔，其與左側(cè)上行垂直靜脈連接，并且連接處內(nèi)徑狹窄，彩色多普勒顯示該處血流增快，左側(cè)垂直靜脈上行回流入左無(wú)名靜脈，最終回流入右側(cè)的上腔靜脈，導(dǎo)致右上腔靜脈和無(wú)名靜脈內(nèi)徑增寬，診斷為心上型TAPVC并共同肺靜脈腔與垂直靜脈連接處梗阻；例5和7心上型胎兒就診時(shí)孕周較大，典型的表現(xiàn)為右心系統(tǒng)擴(kuò)大明顯，左心系統(tǒng)內(nèi)徑小，主動(dòng)脈與肺動(dòng)脈比值減小，在外院均被誤診為左心室發(fā)育不良，主動(dòng)脈縮窄，來(lái)我院會(huì)診后均首先排除了左心室發(fā)育不良的診斷，我院超聲心動(dòng)圖反復(fù)掃查顯示胎兒的二尖瓣及主動(dòng)脈均未見(jiàn)異常，開(kāi)放及啟閉良好，血流未見(jiàn)異常，因此考慮左心系統(tǒng)內(nèi)徑小為回流入左心房?jī)?nèi)的血流量減少所導(dǎo)致，此時(shí)正常的左心房形態(tài)消失(左右下肺靜脈有棱角的結(jié)構(gòu)消失)，左心房變得光滑，形態(tài)呈圓形或橢圓形，其中例5在降主動(dòng)脈旁可見(jiàn)一上行的靜脈血管，其走行至與左肺動(dòng)脈相交叉的位置處可見(jiàn)局部血流加速所致的花色血流信號(hào)，最后這支異常血管的血流注入到左側(cè)的無(wú)名靜脈，并最終回流入右上腔靜脈，致右上腔靜脈及無(wú)名靜脈內(nèi)徑增寬，根據(jù)這一征象最后診斷為心上型TAPVC并垂直靜脈梗阻；例7經(jīng)反復(fù)掃查在降主動(dòng)脈旁并無(wú)上行的垂直靜脈，當(dāng)時(shí)初步考慮為主動(dòng)脈縮窄所致右心系統(tǒng)擴(kuò)大，但反復(fù)掃查后顯示右側(cè)的奇靜脈旁存在一上行的靜脈樣血管，其血流通暢，并回流入右上腔靜脈，最終回流入右心房，根據(jù)這些征象最后診斷為心上型TAPVC；例5和7均在左心房的后方可見(jiàn)一共同靜脈腔，但因?yàn)樵兄茌^大，胎兒體位受限，共同靜脈腔顯示非常困難。這5例孕29周后來(lái)我院會(huì)診的胎兒出生后均在我院進(jìn)行了超聲心動(dòng)圖檢查，證實(shí)了產(chǎn)前超聲心動(dòng)圖檢查的診斷，并均進(jìn)行了手術(shù)治療，例1心下型患兒于術(shù)后1周死亡，其他患兒術(shù)后隨訪恢復(fù)良好。

通過(guò)分析總結(jié)本文病例及查閱相關(guān)文獻(xiàn)，認(rèn)為在胎兒超聲心動(dòng)圖檢查過(guò)程中，應(yīng)利用二維、彩色多普勒和能量多普勒仔細(xì)觀察肺靜脈。由于胎兒期肺循環(huán)血流量非常少，而且胎兒肺靜脈管壁薄、管腔細(xì)小、血流速度低，所以超聲檢查胎兒肺靜脈具有一定的困難，但胎兒期肺臟沒(méi)有呼吸功能，肺組織呈塌陷狀態(tài)，肺內(nèi)無(wú)氣體的干擾，肺靜脈血流的顯示相對(duì)成人較容易，而能量多普勒及高清彩色多普勒有助于顯示肺靜脈[17,18]，因此在檢查過(guò)程中至少應(yīng)觀察到1支肺靜脈引流入左心房，因?yàn)橹挥蠺APVC對(duì)新生兒的生命構(gòu)成威脅，只要識(shí)別到1支肺靜脈進(jìn)入左心房就可以排除TAPVC的診斷。

TAPVC胎兒在孕晚期最常見(jiàn)的間接征象是右心系統(tǒng)增大，左、右心系統(tǒng)比例減小，但部分正常胎兒作為一種正常生理的變異，在妊娠晚期也常出現(xiàn)上述征象，因此應(yīng)注意鑒別[19]；此外在胎兒左心系統(tǒng)梗阻性畸形，如主動(dòng)脈縮窄、主動(dòng)脈弓離斷、左心發(fā)育不良綜合征等也常見(jiàn)到上述征象，這些因素都是導(dǎo)致TAPVC診斷假陽(yáng)性或假陰性的常見(jiàn)原因[20]，此外本病須注意與永存左上腔靜脈及引起肺靜脈梗阻的三房心相鑒別[11]。胎兒期存在永存左上腔靜脈時(shí)心臟左右系統(tǒng)內(nèi)徑比值正常，而且左心房的形態(tài)正常，利用彩色多普勒和能量多普勒可以顯示肺靜脈回流入左心房而非冠狀靜脈竇；引起肺靜脈梗阻的三房心在胎兒期可以顯示左心房?jī)?nèi)的異常膜狀樣回聲，膜狀回聲存在交通口，彩色多普勒顯示交通口處血流呈花色信號(hào)，而且肺靜脈仍回流入左心房。

單純性TAPVC雖然臨床發(fā)病率低，但是仍具有其特征性的超聲心動(dòng)圖特點(diǎn)。心下型TAPVC由于在肝內(nèi)存在異常走行的血管，因此不易被誤診及漏診；由于胎兒在母體內(nèi)體位多變，再加上頸部的血管較多，胎兒期不易辨認(rèn)，因此心上型和心內(nèi)型TAPVC在臨床的診斷中仍然存在一定的困難，容易造成漏診及誤診，這就要求檢查者完成心臟常規(guī)掃查切面后，注意有無(wú)異常的血管，并對(duì)異常走行的血管進(jìn)行多切面、多角度的掃查，并要對(duì)本病不同孕周的胎兒血流動(dòng)力學(xué)特點(diǎn)及本病異位引流的途徑有全面的認(rèn)識(shí)，以提高本病的產(chǎn)前診斷率。

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(本文編輯：張萍)

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

首都醫(yī)科大學(xué)附屬北京安貞醫(yī)院兒童心血管病中心 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)