Pulmonary Veins in Total Anomalous Pulmonary Venous Connection with Obstruction: Demonstration Using Silicone Rubber Casts

We studied the postmortem vascular anatomy of four patients with total anomalous pulmonary venous connection (TAPVC) with obstruction using a silicone rubber casting method. Two patients were studied clinically but died before therapeutic intervention, one died after corrective surgery, and another was not diagnosed before death. The morphologic types were supracardiac (2), infracardiac (1), and mixed (1). One of the supracardiac forms had a stenosis at the left vertical vein by a bronchoarterial vise, and a collateral channel to the right superior caval vein was also present. In the other supracardiac case, a left upper pulmonary vein was connected to the left vertical vein above the stenotic portion, resulting in postoperative pulmonary hemorrhage into the left upper lobe. The patient with infracardiac TAPVC had a tree-shaped descending vertical vein and small opening at the inferior caval vein. In the mixed type, a right upper pulmonary vein ran between the pulmonary artery and bronchus draining into the superior caval vein, and the rest of the drainage was to the portal vein.     

Supracardiac type total anomalous pulmonary venous connection (TAPVC) with oesophageal varices

Oesophageal varices due to total anomalous pulmonary venous connection (TAPVC) is very rare. Additionally, the infradiaphragmatic type is the most common type of oesophageal varices due to TAPVC. Paraoesophageal varices due to stenosis of the vertical vein of supracardiac TAPVC has not previously been reported. We describe paraoesophageal varices developed as a result of a connection between the left lower pulmonary vein and the umbilicovitelline venous system because of stenosis of the proximal vertical vein in supracardiac type TAPVC in a 3-day-old female newborn who presented with general cyanosis, tachypnoea and dyspnoea.     

Fate of the unligated vertical vein after repair of supracardiac anomalous pulmonary venous connection

OBJECTIVE: To determine the fate of the unligated vertical vein after repair of isolated supracardiac total anomalous pulmonary venous connection (TAPVC). METHODS: We reviewed the outcome of 28 patients who were diagnosed to have isolated supracardiac TAPVC and determined the fate of the unligated vertical vein. RESULTS: Of the 28 patients, four died before surgery. The remaining 24 patients underwent surgical correction of TAPVC with (n = 5) or without (n = 19) ligation of vertical vein at a median age of 20 days (range: 1-574 days). There were no significant differences in age, weight, presence of pulmonary venous obstruction, need for preoperative inotropic and ventilatory support, cardiopulmonary bypass duration, postoperative pulmonary hypertensive crisis and requirement of peritoneal dialysis between patients with and those without vertical vein ligation. The in-hospital surgical mortality was 50% (12/24), with 83% (10/12) of deaths occurring before 1990. Patients who died after surgery were significantly younger (median age: 5.5 days vs 37 days, P = 0.005), lighter (3.3 +/- 0.5 kg vs 3.9 +/- 0.6 kg, P = 0.016), more likely to have pulmonary venous obstruction preoperatively (75% vs 12%, P = 0.039) and have undergone surgery before 1990 (83% vs 33%, P = 0.036). The 12 survivors were followed up for a median of 4.7 years (range: 2.3-18.1 years), 10 of whom had their vertical vein unligated. The vertical vein remained patent in five (50%) patients, while stenosis of pulmonary venous anastomosis was only present in one patient. Of these five patients, three had subsequently undergone surgical ligation of the vertical vein to eliminate a large left-to-right shunt. CONCLUSIONS: Patency of the unligated vertical vein is common after the repair of supracardiac TAPVC, even in the absence of pulmonary venous obstruction. The degree of left-to-right shunt through the patent vertical vein may be so significant as to warrant surgical ligation.     

完全性肺静脉异位引流病理谱及个体化手术治疗

目的 描绘完全性肺静脉异位引流(TAPVC)患儿肺静脉走行"路线图"和形态"变异图",阐明肺静脉病理谱的变化规律,促进有效个体化手术.方法 2006年4月至2009年9月,139例TAPVC患儿进行手术,心上型61例,心内型55例,心下型6例和混合型17例.病理诊断依据超声心动图、核磁共振、计算机断层扫描或心导管和心血管造影检查以及术中解剖,根据患儿TAPVC类型和解剖特点选择个体化手术方法.结果 1.病理谱:①肺静脉走行连接"路线图":心上型按照垂直静脉(VV)走行方式分为4种,左行(47例)、右行(9例)、后行(2例)和双行(3例);心内型按照肺静脉回流部位分为3种,汇入冠状静脉窦(49例)、汇入右心房(5例)和同时汇人CS和右心房(1例),再依据肺静脉开口数目分为4个开口、2个开口和1个开口三种亚型;心下型按照VV汇入体静脉方式分为4种:汇入门静脉(1例)、肝静脉(3例)、同时汇入门静脉和肝静脉(1例)以及汇入下腔静脉(1例);混合型按照肺静脉回流双侧是否对称分为双侧对称连接的"2+2"型(5例)、双侧不对称连接的"3+1"型(10例)和"怪异型"(2例).②肺静脉形态"变异图":肺静脉入口狭窄、发育不良或多分支(11例)、共汇肺静脉发育不良或伴内膜增生(4例)、VV扭曲、短小和狭窄(9例).2.手术结果:本组早期死亡6例(4.3%),其中低心排1例,肺静脉梗阻5例.中期随访因肺静脉梗阻再手术6例,术后轻微梗阻2例.结论 TAPVC患儿肺静脉病理谱广,个体差异大;按照肺静脉走行"路线图"和形态"变异图"有利术中肺静脉解剖的探查和个体化手术设计.

Abstract：

Objective To delineate the morphological spectrum of total anomalous pulmonary venous connection (TAPVC) by building the "road map" and the " variation chart" of pulmonary veins,and to advance individualized surgical treatment Methods Between April,2006 to June,2009, 139 consecutive patients with TAPVC underwent operations. There were 61 supracardiac,55 intracardiac,6 infracardiac and 17 mixed types. Pathological diagnosis was made by echocardiogram,magnetic resonance imaging, computerized tomography,or the cardiac catheterization and operative findings during operation. The option of procedure was determined by findings. Results (1) Pathological spectrum:①" the road map"of the pulmonary veins: the patients with supracardiac type were divided into 4 subtypes according to the course of vertical veins:left course (47 cases),right course (9 cases),posterior course(2 cases) and double courses (3 cases). The patients with intracardiac type were divided into 3 subtypes according to the draining site of pulmonary veins: to coronary sinus (49 cases) ,to right atrium (5 cases) or to coronary sinus and right atrium (1 case),and the openings of pulmonary veins may be 4,2 or 1 in each subtype. The patients with infracardiac type were divided into 4 subtypes according to the draining site of vertical vein:to portal vein (1 case),to hepatic vein(3 cases),to portal vein and hepatic vein (1 case) and to inferior vena cava (1 cases). The patients with mixed type were divided into 3 subtypes: bilateral and symmetrical connections "2 + 2" pulmonary venous drainage pattern; bilateral and asymmetrical connections"3 +1" pulmonary venous drainage pattern and bizarre anatomic variants. ② The morphological "variation chart" of pulmonary veins individual pulmonary vein stenosis or excessive tributary veins (11 cases) showed: hypoplastic confluence veins (4 cases) and vertical veins distortion or elongation or forming hemodynamic vise or common pulmonary vein that drained to coronary sinus or right atrium through a narrowed short vertical vein or a small window(9cases).(2)Surgical results：early death was encountered in 6 cases(4.3％).The causes included 10W cardiac output syndrome in 1 case,and pulmonary veins stenosis and associated complications in the other 5 patients.Six patients with pulmonary restenosis underwent reoperation.All survived.Two patients had mild residual obstruction during Intermediate-term follow-up.Conclusions The patients with TAPVC had a wide spectrum of pulmonary veins with high inter-individual variation.It was useful to delineate the anatomy of pulmonary veins and to plan personalized procedures during operations according to the"road map"and"variation chart"of pulmonary veins.     

单纯性完全型肺静脉异位引流产前8例超声心动图诊断

摘要 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。     

完全性肺静脉异位连接的外科治疗

目的 总结10例完全性肺静脉异位连接的外科治疗经验。方法 全组均在全身麻醉中度低温体外循环下进行手术，6例心上型患儿早期1例经后径法矫治，其他5例中3例经右心房切口径路矫治；另2例采用心上法矫治；4例心内型1例采用自体心包片将冠状静脉窦口经扩大的房间隔缺损隔入左心房，后3例用5-0 Prolene线连续缝合房间隔粗糙边缘后再矫治。结果 无手术死亡，发生心律失常3例，一过性肺水肿2例，均经治疗痊愈。全组随访4个月～5年，心功能正常。结论 提高手术成功率和防止术后并发症的关键为：术中吻合口要足够大，扩大左心房容积及术后及时处理心律失常、肺水肿和低心排血量。心内型矫治时消除房间隔粗糙面可防止术后肺静脉梗阻。     

Total anomalous pulmonary venous connection— radiological features

Seventeen cases of total anomalous pulmonary venous connection (TAPVC) were studied radiologically in the last five years. The diagnosis was confirmed by cineangiography in 16 cases and at necropsy in one patient who was found to have infradiaphragmatic variety of TAPVC. Eleven of the 16 cases were found to have supracardiac TAPVC through the left ascending vein. In five cases the anomalous veins connected to the right atrium. Four of these five cases with right atrial connection drained through the coronary sinus and in the fifth case the anomalous veins entered the right atrium directly. Postero-antevior thoracic roentgenogram identified supracardiac anomalous connection in each of the eleven cases. The thoracic roentgenogram was nonspecific in the five cases of the cardiac type of TAPVC. Levophase of the pulmonary angiogram was useful not only in the identification of TAPVC but also indicated whether all or only some of the veins are connected anomalously. Biplane angiogram was found to be useful in cardiac type of TAPVC.     

Atypical total anomalous pulmonary venous connection: Two channels leading to infracardiac terminations

Summary Reported is a rare case of total anomalous pulmonary venous connection (TAPVC) where veins from each lung joined a homolateral confluence. From each confluence, a vein descended into the abdomen, the vein from the right lung joining the ductus venosus, while the vein from the left joined the portal vein.In TAPVC to systemic veins, multiple connections are rare. Multiple connections are most common at supracardiac and cardiac levels, less common at supra- and infracardiac levels, and rare at cardiac and infracardiac levels.From the literature, it is evident that multiple connections at one body level, as in our case, are rare.     

Multidetector CT evaluation of total anomalous pulmonary venous connections: comparison with echocardiography

Background  Although echocardiography is the first-line imaging modality in the diagnosis of total anomalous pulmonary venous connection (TAPVC), multidetector CT (MDCT) could have advantages in the diagnosis of TAPVC in certain cases. Objective  To compare MDCT with echocardiography in the evaluation of TAPVC. Materials and methods  Enrolled in the study were 23 patients with surgically proven TAPVC. The echocardiography and MDCT findings were independently interpreted by a paediatric cardiologist and cardiac radiologist in terms of: (1) the drainage site of the common pulmonary vein, (2) stenosis of the vertical vein, and (3) the course of the atypical vessel into the systemic vein in the case of vertical vein stenosis. The findings from both modalities were correlated with the results obtained at surgery (n=22) or autopsy (n=1). Results  In all patients, MDCT correctly depicted the drainage site of the common pulmonary vein, stenosis of the vertical vein and the course of the atypical vessel into the systemic vein (sensitivity 100%, specificity 100%). The specificity of echocardiography was 100% for the three defined findings. The sensitivity of echocardiography, however, was 87%, 71% and 0%, respectively. Conclusion  MDCT can facilitate the diagnosis of TAPVC in certain cases.     

Percutaneous Closure of a Large Unligated Vertical Vein Using the Amplatzer Vascular Plug II After Supracardiac Total Anomalous Pulmonary Venous Connection (TAPVC) Repair

It is well known that the vertical vein (VV) may have to be left open after repair of a total anomalous pulmonary venous connection (TAPVC) in children with preoperative obstruction, whose left heart chambers are small (Cope et al. in Ann Thorac Surg 64:23–29, 3). An unligated VV has been found to reduce pulmonary arterial pressure, decrease perioperative pulmonary hypertensive crisis, and provide better hemodynamics postoperatively (Chowdhry et al. in J Thorac Cardiovasc Surg 133:1286–1294, 2). Although these VVs are expected to close later, they may remain patent in about half of these children (Cheung et al. J Paediatr Child Health 41:361–364, 1).The patent VVs may be a cause for significant left-to-right shunting, and the children may be symptomatic. The case report describes a child who had a large patent VV after repair of supracardiac TAPVC and its closure using the Amplatzer Vascular Plug II device.     

完全型肺静脉异位引流胎儿超声肺静脉频谱特征

目的 探讨胎儿完全型肺静脉异位引流(TAPVC)的肺静脉频谱特征表现.方法 回顾性分析2017年2月至2021年6月经山东省潍坊市妇幼保健院产前超声心动图诊断的TAPVC胎儿的肺静脉频谱表现,总结不同类型TAPVC肺静脉的频谱特征.结果 共诊断19例TAPVC(11例单纯型,8例复合型)胎儿,其中心上型11例,心内型和...     

Successful balloon dilatation of ascending vein stenosis in obstructed supracardiac total anomalous pulmonary venous connection

Summary A newborn infant presenting with severe hypoxia and pulmonary edema was found to have supracardiac total anomalous pulmonary venous connection (TAPVC). There was a severe localized stenosis (gradient >30 mmHg) of the vein ascending from the pulmonary venous confluence. Balloon dilatation of the stenosis provided immediate and effective relief of the obstruction (gradient 4 mmHg) until surgery was performed. In sick neonates with discretely obstructed anomalous pulmonary venous connection, short-term hemodynamic stability may be achieved by balloon angioplasty of the site of obstruction.     

Integrated management during the perinatal period for total anomalous pulmonary venous connection北大核心CSCD

Objective To evaluate the clinical effectiveness of integrated management during the perinatal period for fetuses diagnosed with total anomalous pulmonary venous connection (TAPVC) by prenatal echocardiography. Methods Clinical data of 64 cases of TAPVC fetuses diagnosed by prenatal echocardiography and managed with integrated perinatal care in Qingdao Women and Children's Hospital from January 2017 to December 2021 were retrospectively analyzed. Integrated perinatal care included multidisciplinary collaboration among obstetrics, fetal medicine, ultrasound, pediatric cardiology, pediatric anesthesia, and neonatology. Results Among the 64 TAPVC fetuses, there were 29 cases of supracardiac type, 27 cases of intracardiac type, 2 cases of infracardiac type, and 6 cases of mixed type. Chromosomal analysis was performed in 42 cases, and no obvious abnormalities were found. Among the 64 TAPVC fetuses, 37 were induced labor, and 27 were followed up until term birth. Among the 27 TAPVC cases, 2 cases accepted palliative care, 2 cases were referred to another hospital for treatment and lost to follow-up, while the remaining 23 cases underwent primary repair surgery. One case died within 6 months after the operation due to low cardiac output syndrome, while the other 22 cases were followed up for (2.1±0.3) years with good outcomes (2 cases underwent a second surgery within 1 year after the first operation due to anastomotic stenosis or pulmonary vein stenosis). Conclusions TAPVC fetuses can achieve good outcomes with integrated management during the perinatal period. © 2023 Xiangya Hospital of CSU. All rights reserved.     

Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection

Background

Obstructed total anomalous pulmonary venous connection (TAPVC) is frequently misdiagnosed as pulmonary disease and without operative correction early death is common. It is important to make a correct diagnosis before surgery.

Objective

The purpose of this study was to describe the chest radiographic features of obstructed TAPVC and compare CT angiography with transthoracic echocardiography in the evaluation of obstructed TAPVC.

Materials and methods

Eighteen children with obstructed TAPVC were assessed. Their clinical and imaging data were retrospectively reviewed. The characteristic radiographic findings were analyzed and compared with surgical results, and the diagnostic accuracy of CT angiography and transthoracic echocardiography was evaluated in terms of pulmonary venous drainage and obstruction detection.

Results

The common radiographic features included pulmonary venous congestion or edema or both (16 of 18 cases, 89%), and absence of cardiomegaly (12 of 18 cases, 67%). CT angiography correctly diagnosed TAPVC and clearly revealed the draining sites in all children (five with supracardiac TAPVC, three with cardiac TAPVC, eight with infracardiac TAPVC and two with mixed TAPVC). The diagnostic agreement between CT angiography and surgery was 100%. Transthoracic echocardiography only correctly revealed the draining sites in 11 children (5 with supracardiac TAPVC, 2 with cardiac TAPVC and 4 with infracardiac TAPVC). The diagnostic agreement between transthoracic echocardiography and surgery was 61%. The diagnostic accuracy of CT angiography was higher than that of transthoracic echocardiography (P?=?0.0156). Thirty-four sites of obstruction were correctly detected by CT angiography (11 in the mediastinum, 1 at the diaphragmatic level, 9 below the diaphragm and 13 stenotic individual pulmonary veins in the lung). The diagnostic agreement between CT angiography and surgery was 92%. Transthoracic echocardiography only correctly detected 15 sites of obstruction (11 in the mediastinum, 1 at the diaphragmatic level and 3 below the diaphragm). The diagnostic agreement between transthoracic echocardiography and surgery was 41%. The rate of detection for sites of obstruction with transthoracic echocardiography was much lower than that of CT angiography (P?=?0.0002).

Conclusion

In children with obstructed TAPVC, plain radiographs usually show a characteristic pattern of pulmonary venous congestion or edema, or both, and a normal cardiac silhouette. CT angiography is superior to transthoracic echocardiography in the evaluation of pulmonary venous drainage and obstruction, especially in children with infracardiac and mixed TAPVC.     

经心包斜窦行扩大吻合口心上型全肺静脉异位引流矫治术

目的 探讨经心包斜窦入路行心上型全肺静脉异位引流(TAPVC)矫治术,利用垂直静脉延长共同静脉切口,从而扩大共同静脉与左心房的吻合口.方法 2007年12月至2009年1月,我院共收治27例TAPVC患儿在体外循环下行畸形矫治手术,其中男17例,女10例;年龄2 d至13岁;体重2.3～21 kg,平均(5.16±3.49)kg;其中心上型TAPVC 11例,占40.7%;心下型1例,占3.7%;心内型12例,占44.4%;混合型3例,占11.1%.在全部接受手术的11例心上型TAPVC患儿中,10例采用经心包斜窦人路行扩大吻合口的矫治技术,其中7例同时采用无内膜接触缝合技术.此10例患儿中,男7例,女3例;年龄2 d至13岁;体重2.4～21 kg,中位数为6.0 kg.术后行心脏超声随访,随访时间为1～14个月,中位数为7个月,常规随访时间点分别设在出院前、术后3个月、术后半年及术后1年.手术选择心包斜窦入路,沿长轴横向剖开共同静脉,并将此切口上延至垂直静脉的心包返折处,使用7-0 PDS缝线将左心房后壁切口与共同静脉切口或其周边的心包组织吻合.结果 1例患儿因凝血功能障碍于术后第二天死亡;另1例患儿由于家庭原因于术后第五天放弃治疗死亡,其他8例患儿均存活.在8例存活患儿中,1例患儿因术前并发双侧肺实变,另1例患儿因合并重度肺动脉高压,术后机械辅助呼吸超过7 d,其他患儿均无并发症发生.至目前为止,心脏超声随访提示:全部存活患儿肺静脉回流通畅,血流速度介于0.65～1.2 m/s之间.结论 在治疗心上型TAPVC时,选择心包斜窦入路不仅可以获得良好的术野,还可以利用垂直静脉扩大吻合口内径,同时方便采用无内膜接触缝合技术,以预防术后肺静脉梗阻的发生.但本技术路线尚有待大宗病例的远期随访进行验证.     

Post-op systemic hypertensive crises in an infant with total anomalous pulmonary venous connection

Systemic hypertension is not usually a complication following repair of total anomalous pulmonary venous connection (TAPVC). We report an infant with supracardiac TAPVC with hypertensive crises post-operatively resulting in pulmonary edema. We feel this might have been related to the pre-operative hemodynamics as described. Beta-blockers improved the hypertensive crises.     

完全性肺静脉异位引流的超声心动图诊断价值

自１９９０年６月至１９９７年１月７年中,我院彩超室共诊断完全性肺静脉异位引流１２３例,其中４５例行心导管及造影检查并证实。其中男２８例,女１７例,年龄７天至９岁。超声心动图完全性肺静脉异位引流的敏感性为９７．７％,特异性为１００％,异位引流部位诊断的准确率为１００％,其中心内型１８例,心上型２３例,     

Reversed differential cyanosis in the newborn: a clinical finding in the supracardiac total anomalous pulmonary venous connection

The newborn can experience two types of differential cyanosis (DC). The common type of DC occurs when oxygen saturation in the right hand is greater than in the foot. The second type of DC, reversed differential cyanosis (RDC), occurs when oxygen saturation is lower in the right hand than in the foot. This phenomenon is observed in transposition of the great arteries (TGA) with patent ductus arteriosis (PDA) and elevated pulmonary vascular resistance or in TGA with PDA and preductal aortic interruption or coarctation. This report describes a case of RDC not previously described involving an infant with supracardiac total anomalous pulmonary venous connection (TAPVC). In supracardiac TAPVC, RDC results from streaming of highly saturated superior vena cava (SVC) blood into the right ventricle, out the main pulmonary artery, through a PDA, and to the descending aorta, with streaming of more desaturated blood from the inferior vena cava (IVC) into the left atrium across the atrial septal defect (ASD)/foramen ovale. Therefore, as part of a neonatal examination to rule out congenital heart disease (CHD), simultaneous pre- and postductal oxygen saturations should be documented. The presence of RDC should initiate immediate full cardiac evaluation for CHD. Supracardiac TAPVC should be included in the differential diagnosis if RDC is observed.     

Obstructed total anomalous pulmonary venous connection

Summary With the advent of echocardiography, total anomalous pulmonary venous connection (TAPVC) can be readily diagnosed without much difficulty. However, noninvasive detection of the presence of pulmonary venous obstruction in TAPVC remains a difficult issue. During a 5.5-year period, 42 patients were found to have TAPVC by catheterization, surgery, and/or autopsy: 17 had supracardiac drainage, 13 paracardiac drainage, nine infracardiac drain-age, and three mixed drainage. Obstruction to pulmonary venous drainage was found in 24 patients (57%). Patients with right isomerism tended to have a higher incidence of pulmonary venous obstruction than those with the usual atrial arrangement (80% vs. 44%,p<0.05). Color Doppler combined with cross-sectional echocardiography provided accurate delineation of drainage sites in 93% cases (39 of 42). Among the 39 cases with correct echocardiographic delineation of the drainage site, obstruction was detected by echocardiography in 22 cases with a sensitivity of 100% (22 of 22) and a specificity of 85% (17 of 20). Therefore, complete echocardiography, including cross-sectional images and color Doppler proved to be a reliable tool in the detection of drainage sites and pulmonary venous obstruction in TAPVC.     

Surgical treatment of total anomalous pulmonary venous connection

Fourteen infants ranging in age from 18 days to 10 months (median age = 4 months) underwent surgical repair of total anomalous pulmonary venous connection (TAPVC) between September, 1988 and December, 1989. The anomalous drainage was supracardiac in nine, cardiac in two, infracardiac in one and mixed in one. One patient had a complex type of TAPVC. There were two hospital deaths, one a critically ill three week old infant with obstructed infracardiac TAPVC and the other with complex TAPVC. There was no late death. All twelve survivors are in NYHA functional Class 1. Postoperative echocardiography revealed unobstructed pulmonary blood flow in all of them. Surgical correction of TAPVC in infancy has been performed with gratifying results. A high index of suspicion, early diagnosis, prompt referral and an aggressive surgical approach are essential for success in this otherwise lethal condition.