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.     

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: Persistence and atresia of the common pulmonary vein

Summary A unique patient with subdiaphragmatic total anomalous pulmonary venous connection (TAPVC) in whom individual pulmonary veins drain into a myocardium-encircled saccular confluence, which may represent a persistent and atretic common pulmonary vein (CPV), is presented.     

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.     

Common pulmonary vein atresia without anomalous pulmonary venous connection

Common pulmonary vein atresia without pulmonary venous connection is a rare form of congenital heart disease. No communication exists between the confluence of the pulmonary veins and the heart or a major systemic vessel. A case diagnosed antemortem is presented. Correct early diagnosis is imperative as surgery may be corrective.     

小儿完全性肺静脉畸形引流的外科治疗

目的　评判完全性肺静脉畸形引流早期手术矫治的疗效及心上型完全性肺静脉畸形引流各种吻合方法的优缺点。方法　 1976～ 1999年共收治完全性肺静脉畸形引流 14 0例 (男 81,女5 9) ,平均年龄 ( 7.13± 6.88)岁。心上型 87例 ( 62 .14 % ) ,心内型 4 4例 ( 3 1.4 3 % ) ,心下型 2例( 1.4 3 % ) ,混合型 7例 ( 5 % )。 2例经右心导管检查证实为重度肺动脉高压 ,未能实施矫治手术 ,余患儿均在全麻、低温、体外循环下行矫治术。心上型的吻合方法包括 :心外法 ( 2 1例 ) ,经上腔静脉与升主动脉间吻合法 ( 3例 ) ,经房间隔缺损吻合法 ( 18例 ) ,心外吻合加经房缺吻合 ( 2例 ) ,经左右心房联合切口吻合法 ( 4 3例 )。结果　术后早期 ( 3 0d)死亡 14例 ( 10 % ) ,其中 3例合并其他复杂的心内畸形 ,均死亡 ,故单纯TAPVC的手术死亡 11例 ,死亡率为 8.0 3 % ( 11/ 13 7)。术后主要并发症为心律失常 60例 ( 4 2 .86% )。结论　完全性肺静脉畸形引流宜尽早手术 ,心上型矫治术吻合口足够大是保证手术成功的关键 ,左右房联合切口入路可确保吻合口足够大 ,但心律失常发生率高 ,经上腔静脉与升主动脉间吻合法可减少心房损伤 ,降低心律失常发生率。     

Partial anomalous pulmonary venous connection to the azygos vein with intact atrial septum

Summary The case of a 13-year-old girl with an unusual form of partial anomalous pulmonary venous connection is reported. Cardiac catheterization revealed anomalous connection of the right pulmonary veins to the azygos vein and an intact atrial septum. Rationale for nonsurgical management and four-year follow-up results are discussed.     

Functional total anomalous pulmonary venous connection via levoatriocardinal vein

We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the “dead‐end” left atrium, the pulmonary venous flow had a to‐and‐fro pattern along with atrial relaxation and contraction. Postnatal echocardiography and computed tomography confirmed the diagnosis of normally connected but anomalously draining pulmonary veins via the LACV. Surgical creation of an atrial septal defect on the day of birth successfully relieved pulmonary venous obstruction. Normally connected but anomalously draining pulmonary veins via the LACV should be considered for TAPVC differential diagnosis in fetuses with a left‐side heart obstruction.     

采用左心房顶部进路纠治心上型完全性肺静脉异位连接

目的　评估经左房顶部进路方法纠治心上型完全性肺静脉异位连接。方法　 2 0 0 1年11月至 2 0 0 3年 1月 ,采用经左房顶部进路方法纠治心上型完全性肺静脉异位连接 16例。年龄 (2 3d～ 4岁 ) ,其中 6个月以下 8例 ,占 5 0 .0 % ;平均体重 (8.5± 4 .94 )kg ,10kg以下共 11例 ,占全组6 8.7%。结果　纠治心上型完全性肺静脉异位连接 16例 ,无死亡。其中 1例术后吻合口狭窄 ,第 2d再次手术 ,肺静脉回流通畅 ,康复出院。 1例婴儿术后做膈肌折叠术。 2例新生儿术后延迟关胸。术后随访 3～ 16个月 ,所有患儿生长发育良好 ,胸片示心影较术前明显缩小 ,肺充血消失 ,心电图示窦性节律 ,无一例出现肺静脉回流梗阻。结论　采用经左房顶部进路方法纠治心上型完全性肺静脉异位连接 ,取得较好效果。手术成功取决于左房与汇总静脉的吻合口大小 ,保证肺静脉回流无梗阻。     

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.     

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.     

体重5kg以下完全性肺静脉异位引流患儿的外科治疗

目的 探讨体重5 kg以下完全性肺静脉异位引流患儿的外科治疗效果.方法 2002年1月至2009年12月,首都医科大学附属北京儿童医院外科手术矫治体重5 kg以下各型完全性肺静脉异位引流患儿52例,均在全麻、低温体外循环下手术.对心上型采取经左、右心房联合切口或心上入路完成肺静脉共腔与左房的侧侧吻合;心内型剪除冠状静脉...     

影响完全性肺静脉异位引流术病死率的危险因素

目的 分析完全性肺静脉异位引流(TAPVC)纠治术的手术结果,探讨影响其病死率的危险因素.方法 对比分析郑州大学第三附属医院河南省小儿先心病诊疗中心2001年9月至2011年9月收集的TAPVC患儿临床资料.其中男37例,女20例;年龄15 d～6.5岁[(4.27±8.63)个月];体质量4.0 ～21.0(6.33 ±2.70) kg.收集患儿住院病历、超声心动图和手术记录等资料.应用x2检验和Logistic多变量回归分析对患者的手术年龄、体质量、TAPVC分型、术前急症、主动脉钳夹时间、手术时间、体外循环时间及术后呼吸机辅助时间、监护时间、住院时间等因素进行分析,分析影响病死率的危险因素.结果 57例TAPVC患儿纠治术中,围手术期死亡7例(12.2％).单变量分析结果表明,与TAPVC纠治术病死率相关的因素有体质量(P =0.035)、TAPVC分型(P=0.037)、术前急症(P=0.021)、主动脉钳夹时间(P =0.046).Logistic多变量回归分析结果表明,术前急症是影响TAPVC纠治术病死率的独立危险因素(P =0.003).结论 TAPVC患儿术前急症会增加患儿术后的病死率.     

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.     

The value of early repair for total anomalous pulmonary venous drainage

Summary A total of 20 children with total anomalous pulmonary venous drainage (TAPVD) underwent complete repair within the past six years. The drainage was supracardiac in 11, infracardiac in seven, and into the coronary sinus in two. At repair mean age was nine weeks, and weight was 3.7 km. Operative technique in extracardiac types included complete mobilization of the common vein with division of anomalous channel when possible. The incision in the confluent vein was extended into the lobar veins when necessary to permit extensive incorporation of this structure into the posterior wall of the left atrium and resulted in a nonobstructing stellate-type anastomosis.Operative mortality was 10% (2 of 20). Deaths occurred only in the group of infants in whom severe obstruction to pulmonary venous return required emergency operation, and one of these patients has persistent neurologic deficit. Late cardiac catheterization has been performed in 11 of 18 survivors. Nine had no or only minor abnormalities and two required reoperation. There have been no late deaths with follow-up of 2–8 years (mean=4 years). Currently, all of the survivors are without cardiac symptoms and only one requires cardiac medication.Our experience identifies pulmonary venous obstruction with critical symptoms as the major operative risk factor in patients with TAPVD. With early operation prior to onset of critical symptoms, mortality is low and functional results are excellent.     

Double total anomalous pulmonary venous connection

We present a newborn infant with right atrial isomerism, complex congenital heart malformation and anomalous pulmonary venous connection, reliably diagnosed by 2-D Doppler color echocardiography. The infant had no significant obstetric antecedents. The neonatal clinical picture included cyanosis, heart murmur and respiratory distress. The infant was treated with prostaglandin from the age of 24 hours until his death after surgery. The 2-D echo Doppler color flow mapping showed findings that suggested right atrial isomerism, severe left ventricular hypoplasia, pulmonary atresia and ductus arteriosus. The pulmonary veins flowed together to a posterior cardiac chamber from which an emissary vertical venous vessel connected with a left superior aneurysmal sack. Two venous channels emerged from this sack: one connecting to the innominate vein and the other to the atrium. The malformations were confirmed by cardiac catheterization. On the sixth day of life, the patient underwent anastomosis between the posterior venous chamber with the atrium, a modified Blalock-Taussig shunt implant, and ductus closure but died during surgery. The association between complex cardiac anomalies and uncommon obstructive total anomalous pulmonary venous connection in the context of right atrial isomerism is lethal and few neonates survive surgical repair. Two-dimensional echo color flow Doppler is a reliable diagnostic technique and an indispensable guide in angiography.     

心下型完全性肺静脉异位引流的急诊手术和早中期随访结果

目的 回顾完全性肺静脉异位引流(TAPVC)心下型的急诊手术经验和早中期随访结果。方法 2000年9月至2003年6月，手术证实的4例心下型TAPVC，平均年龄27d。肺静脉回流的部位包括：门静脉2例，下腔静脉1例，另1例不详。3例临床上有肺静脉回流梗阻表现，所有患儿均行急诊手术。结果 无手术死亡。1例术后合并肺炎，长时间呼吸机支持，术后19d自动出院。其余3例均痊愈出院。3例患儿得到随访，随访时间14个月～3年。2例恢复良好，心功能Ⅰ级；1例术后4个月起出现肺静脉梗阻，仅作保守治疗但效果不理想，术后3年失访。结论 心下型TAPVC病情危重，急症手术可挽救生命，手术早期效果良好。术后肺静脉梗阻一般内科治疗无效，应积极手术干预。     

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...