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.     

超声心动图诊断胎儿完全型肺静脉异位引流

目的 探讨胎儿完全型肺静脉异位引流(TAPVC)产前诊断线索及超声心动图特征.方法 回顾性分析于我院经超声诊断并经尸体检查或出生后超声心动图证实的14胎TAPVC胎儿的二维及多普勒图像的特征.结果 产前诊断12胎TAPVC,其中心上型9胎,心内型2胎,心下型1胎.TAPVC的产前诊断线索及超声心动图特征:二维超声四腔心切面未显示肺静脉角,左心房后壁光滑;左心房后壁与降主动脉间距离增大;可见共同肺静脉腔和垂直静脉.产前超声心动图漏诊2胎,经出生后超声心动图证实均为心内型TAPVC.14胎TAPVC中,4胎伴肺静脉引流途径梗阻.结论 胎儿超声心动图可诊断TAPVC并准确分型;脉冲和彩色多普勒超声可显示肺静脉回流途径梗阻.     

CT of unusual mediastinal vascular abnormalities

The previously unreported CT findings of supracardiac anomalous pulmonary venous return and Takayasu arteritis are presented. CT demonstration of a left vertical vein and an enlarged superior vena cava strongly suggests a diagnosis of supracardiac anomalous venous return. Dynamic scanning of the mediastinum following bolus injection of urographic contrast with close attention to vascular enhancement patterns is required to suggest a diagnosis of Takayasu arteritis. CT is not the preferred diagnostic modality for either entity, but may prove definitive in some patients and may serve to triage other patients to the appropriate diagnostic modalities.     

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.     

Prenatal diagnosis of total anomalous pulmonary venous connection to the portal vein associated with right atrial isomerism.

We report the prenatal diagnosis of total anomalous pulmonary venous connection to the portal system in a 20-week fetus with right atrial isomerism. The apex of the fetal heart pointed to the left, the fetal stomach was on the right, there was a common atrioventricular valve, the left ventricle was small and the abdominal aorta and inferior vena cava were on the left side; all these features were suggestive of right atrial isomerism. An anomalous vein was connected to the portal vein which ascended above the diaphragm and ended in a confluence of pulmonary veins, posterior to the common atrium. Color Doppler imaging helped confirm the diagnosis of total anomalous pulmonary venous connection. The prenatal findings were confirmed on autopsy.     

区域血流追踪法在超声诊断胎儿肺静脉异位引流中的应用价值

目的探讨区域血流追踪法在超声诊断胎儿肺静脉异位引流中的价值。 方法回顾性选取2015年1月至2019年12月在河北生殖妇产医院进行胎儿超声心动图检查，诊断为肺静脉异位引流的胎儿41例。超声检查胎儿肺静脉时，应用区域血流追踪法，即将肺组织大致分为4个区域，左侧前上部1/2肺野、左侧后下部1/2肺野、右侧前上部1/2肺野、右侧后下部1/2肺野，应用彩色血流技术对41例肺静脉异位引流胎儿4个区域内肺静脉分支进行追踪并定位，观察其近心端与左心房的关系及最终回流部位。 结果41例肺静脉异位引流胎儿，4个区域内肺静脉远端分支分别引流入左上肺静脉、左下肺静脉、右上肺静脉、右下肺静脉。36例完全型肺静脉异位引流胎儿4条肺静脉近心端均未汇入左心房，其中34例呈现左心房后方汇聚征。心上型25例肺静脉最终回流入上腔静脉；心内型7例中6例通过冠状静脉窦回流入右心房，1例直接回流入右心房；心下型2例肺静脉回流入门静脉窦；混合型2例，1例左侧肺静脉汇入上腔静脉，右侧肺静脉汇入下腔静脉，1例左侧肺静脉汇入上腔静脉，右侧肺静脉回流入右心房。5例部分型肺静脉异位引流胎儿，1例右上肺静脉直接回流入右心房，2例右上肺静脉回流入上腔静脉，1例右上、右下肺静脉直接回流入右心房，1例左肺静脉变异为3支，最上支经无名静脉回流入上腔静脉。 结论应用区域血流追踪法可使肺静脉分支检查更全面，定位更加精确，有助于提高胎儿肺静脉异位引流的诊断准确性。     

Two- and four-dimensional echocardiography with B-flow imaging and spatiotemporal image correlation in prenatal diagnosis of isolated total anomalous pulmonary venous connection.

OBJECTIVES: To explore whether the use of four dimensional (4D) ultrasound examination with B-flow imaging and spatiotemporal image correlation (STIC) can supply additional information with respect to two-dimensional (2D) gray-scale and color Doppler echocardiography in the prenatal characterization of isolated total anomalous pulmonary venous connection (TAPVC). METHODS: The study population comprised a group of three TAPVC fetuses that had been examined exclusively by conventional echocardiography, and a group of four additional cases initially identified by conventional echocardiography and examined further by 4D ultrasonography; a thorough postnatal work-up was available for all fetuses. RESULTS: At our center, isolated TAPVC was found in seven of 1040 fetuses with cardiac defects (0.67%). Anomalous drainage was supracardiac to the innominate vein in three cases, cardiac to the coronary sinus in two, and infracardiac to the portal vein in the remaining two cases. An evident asymmetry between left heart and right heart structures was observed in 4/7 cases. The confluence of the anomalous pulmonary veins (PVs) was visualized in 4/7 cases and the connecting vertical vein was identified in 3/5 cases at 2D echocardiography. 4D ultrasound imaging with B-flow imaging and STIC clearly visualized the anomalous PV confluence and the draining vertical vein in all four cases examined. CONCLUSION: 2D and color Doppler echocardiography appears to diagnose reliably TAPVC, albeit with some limitations in thorough assessment of the pathology, depending on the anatomy of the defect, on the technical adequacy of the equipment used and on the experience of the operator. 4D ultrasound examination with B-flow imaging and STIC is apparently able to facilitate identification of the anatomical features of TAPVC, thus supplying additional information over that provided by 2D fetal sonography.     

经胸超声心动图诊断部分型肺静脉异位引流的规律性研究

目的 探讨经胸超声心动图诊断部分型肺静脉异位引流 （PAPVC）的特征及规律.方法 回顾性分析我院68例经手术确诊为PAPVC患者的经胸超声心动图资料.结果 68例 PAPVC中,心上型占14.7%（10/68）,心内型占82.4%（56/68）,心下型占1.5%（1/68）,混合型占1.5%（1/68）;68例中,单支异常连接占26.5%（18/68）,双支异常连接占69.1%（47/68）,三支异常连接占4.4%（3/68）.PAPVC合并房间隔缺损占85.3%（58/68）,单支右上肺静脉异常连接11例中合并上腔型房间隔缺损占81.8%（9/11）.68例 PAPVC经胸超声心动图诊断57例,诊断符合率为83.8%.结论 经胸超声心动图通过观察肺静脉进入位置和血流方向诊断PAPVC可部分代替心血管造影检查     

完全性肺静脉畸形引流的超声心动图诊断

目的：评价超声心动图对完全性肺静脉畸形引流（TAPVC）的诊断价值。方法：回顾分析16例超声诊断为TAPVC的超声心图特征，其中11例超声结果与手术结果相对照，超声检查内容包括二维及彩色多普勒显像，分别经胸骨旁、胸骨上窝，剑突下等部位多切面观详细观察肺静开口部位，引流途径，共同肺静脉干形态及存在的合并畸形等。结果：按Darling等的分型，16例TAPVC患者中超声诊断分型为：心上型（Ⅰ型）11例，其中ⅠA型10例，ⅠB型1例，心内型（Ⅱ型）5例，其中ⅡA型2例，ⅡB型3例。11例经手术证实的TAPVC患者超声诊断及分型与手术结果一致，同时超声心动图还可以估计肺动脉压力，发现合并存在的其他畸形等。结论：超声心动图是TAPVC患者术前首选检查方法，如能多部位多切面仔细观察，则可对TAPVC作出准确的定性及分型诊断。     

Two-dimensional echocardiographic and color flow Doppler detection of an unusual pulmonary venous anomaly: anomalous right pulmonary venous return to the right atrium by way of three separate orifices

In this report, we describe the echocardiographic findings of a patient with anomalous right pulmonary venous return to the right atrium by way of three separate pulmonary vein orifices. The color flow Doppler examination was essential for identification of the pulmonary veins and their connection to the right atrium.     

超声诊断完全性肺静脉畸形引流20例

目的 评价超声心动图对完全性肺静脉畸形引流（TAPVC）的诊断价值。 方法 分析20例TAPVC的超声表现。 结果 本组病儿共20例，其中心上型10例；心内型7例，其中引流入冠状静脉窦5例、引流入右心房2例；心下型2例；混合型1例。 结论 超声心动图对TAPVC能做出明确的诊断和分型，方便、快速且准确率高。     

Color Doppler flow mapping in the ultrasound diagnosis of total anomalous pulmonary venous connection

With color Doppler flow mapping (CFM), we studied 16 children with total anomalous pulmonary venous connection (TAPVC), which was confirmed at cardiac catheterization, surgery, or autopsy in all but one case. The drainage was supracardiac in nine children, cardiac in four, and infracardiac in three. Obstruction to pulmonary venous return was present in seven children. Increased variance, reflecting disturbed blood flow, as well as increased velocities and aliasing were present in all patients. In patients without obstruction turbulence was present in the right atrium and throughout the common pulmonary venous structures. In patients with obstruction a discrete site of increased turbulence and velocity was identified at the site of obstruction. CFM allows rapid differentiation between normal and abnormal venous and arterial structures in TAPVC. In patients suspected of having TAPVC with obstruction, CFM complemented by pulsed Doppler facilitates the determination of the site of obstruction. CFM allows a more rapid appreciation of the anatomy in TAPVC than can be achieved by two-dimensional imaging alone.     

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。     

肺静脉异位引流的经胸超声心动图影像与手术对照分析

目的评价经胸超声心动图(TTE)诊断175例肺静脉异位引流(APVC)的价值并分析误诊原因,提高诊断率。方法回顾性分析我院175例经胸超声心动图诊断APVC患者的超声影像资料并与手术结果对照,分析诊断价值及误诊原因。结果 175例患者均接受手术,166例最终确诊为APVC。部分型比例更高(PAPVC 61.4%vs TAPVC 38.6%)。超声诊断符合率为94.9%,分型准确率分别为94.1%(PAPVC)、100%(TAPVC)。9例误诊均系单纯静脉窦型房间隔缺损,误诊原因为旁四腔切面右上肺静脉假性骑跨于房顶(房缺较大、位高)、右上肺静脉血流与过隔血流混淆所致(9/9,100%)。分型错误最常见者为心上型误诊为心内型。与PAPVC组相比较,TAPVC组合并其他心内畸形率更高,右心增大及肺动脉高压者更多(P<0.05)。结论 TTE诊断APVC及其伴发畸形具有较高的准确率,但对于单支右侧肺静脉易误判,需注意甄别右上肺静脉假性骑跨、过隔血流混淆并注意观察上腔静脉近心端,减少漏诊与误诊,必要时结合其他影像学检查。     

Fetal Isolated Prominent Left Brachiocepalic Vein

The objectives of this series were to describe the prenatal sonographic findings of a fetal isolated prominent left brachiocephalic vein with its in utero natural history and neonatal outcome and to discuss the differential diagnosis, especially with a total or partial anomalous pulmonary venous return malformation. We reviewed all cases referred to 2 tertiary medical centers with the presenting finding of a prominent, upper thorax transverse vein entering the superior vena cava. The primary suspicion of a subjective dilated left brachiocephalic vein, as a part of a supracardiac anomalous pulmonary venous return malformation, was investigated by a systematic anatomic evaluation. After exclusion of other cardiac and structural anomalies, we followed the pregnancies and their outcomes. Eight cases were recruited during a 7 year period. The mean maternal age was 31 years, and the mean gestational age at the time of diagnosis was 17 weeks. In 7 cases, the dilatation was not evident during 6 weeks of follow up. In 1 case, the dilatation was evident until delivery at 39 weeks and was not apparent on postnatal echocardiography or spiral computed tomography. All neonates developed without any heart or other complications. We conclude that after exclusion other malformations, a prominent left brachiocephalic vein is a benign transient phenomenon that does not persist post‐partum in the neonate.     

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。     

The role of cardiac magnetic resonance in the diagnosis of anomalous pulmonary venous return with subsequent Amplatzer device treatment.

We report the case of a young girl with a mixed total anomalous pulmonary venous return (cardiac and supracardiac) treated sequentially by partial neonatal surgery, and then catheterization at age 19 with installation of an Amplatzer device as a treatment of the remaining anomaly. We describe the usefulness of magnetic resonance imaging in both the diagnosis and follow-up of this anomaly.     

经胸超声心动图诊断儿童肺静脉异位连接

目的 探讨儿童肺静脉异位连接的超声表现以及经胸超声心动图(TTE)诊断价值.方法 回顾性分析经手术证实的31例肺静脉异位连接患儿,其中男22例,女9例,年龄22天～10岁,完全型肺静脉异位连接(TAPVC)18例,部分型肺静脉异位连接(PAPVC)13例.结果 31例APVC在TTE中均有右心负荷增加表现.18例TAPVC的解剖分型为心上型9例(50%),心内型9例(50%),16例(88.89%)经胸超声心动图诊断与手术结果完全或基本符合,2例(11.11%)误诊为部分型房室间隔缺损.13例PAPVC的解剖分型为心内型12例(92.31%),心上型1例(7.69%),均合并房间隔缺损,其中9例(69.23%)合并静脉窦型房间隔缺损;9例(69.23%)TTE诊断与手术结果完全或基本符合,4例(30.67%)误诊为单纯房间隔缺损.结论 超声心动图发现右心增大时,无论是否伴有房间隔缺损,均应注意常规检查4条肺静脉入口位置及血流方向.     

完全性肺静脉畸形连接的超声心动图诊断价值

目的探讨超声心动图对完全性肺静脉畸形连接(total anomalous pulmonary venous connection TAPVC)的扫查方法及特征性表现.方法应用彩色多普勒超声或造影检查14例TAPVC.结果 14例TAPVC中,心上型5例,心内型6例,心下型1例,混合型2例.除心下型1例(7.1%)漏诊外,诊断准确率为78.6%(11/14),基本符合率(混合型)14.3%(2/14),总符合率为92.9%.结论应用超声心动图可确定本病的诊断及分型,判断有无肺静脉狭窄、肺动脉高压及预后.对混合型和较少见的心上型TAPVC的诊断准确率有待于进一步提高.     

Isolated partial right lower lobe pulmonary venous drainage into the right atrium as a cause of pulmonary hypertension in a middle-aged woman

Partial anomalous pulmonary venous connection is a rare congenital disease characterized by drainage of one or several pulmonary veins into the systemic venous system. It is extremely rare as an isolated anomaly. We report the case of a middle-aged woman with isolated drainage of an enormously enlarged right lower pulmonary vein into the right atrium with significant left-to-right shunt, severe right ventricular enlargement, and pulmonary hypertension.