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.     

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.     

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.     

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...     

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.     

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.     

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.     

Successful management of cor triatriatum associated with anomalous pulmonary/systemic venous connection in an infant

Summary Diagnosis and successful surgical repair of cor triatriatum, associated with anomalous pulmonary/systemic venous connection in a 6-month-old infant, is described. Follow-up at 2 years revealed no abnormalities. The clinical findings, hemodynamics, and surgical treatment are discussed, together with a review of the literature. This is a rare, life-threatening situation presenting significant diagnostic difficulties in infancy. It is, however, amenable to surgery provided that an early diagnosis is achieved.     

Unusual form of total anomalous pulmonary venous connection with double drainage

A patient with complex congenital heart disease was diagnosed by two-dimensional echocardiography. Total anomalous pulmonary venous connection (TAPVC) was suspected because of the results of two-dimensional echocardiography, but the exact anatomy was delineated by cineangiocardiography as an unusual form of TAPVC with double drainage or connections to the left superior vena cava at nearly the same level. The clinical implications and possible embryogenesis for such a condition are discussed.     

Late pulmonary venous obstruction after surgical repair of infradiaphragmatic total anomalous pulmonary venous return

Summary Twelve years after an apparently successful surgical correction of infradiaphragmatic (obstructed) total anomalous pulmonary venous drainage, a 12-year-old boy developed evidence of pulmonary artery hypertension secondary to pulmonary venous obstruction due to an apparent lack of growth at the anastomotic site.     

“Superimposition” digital subtraction angiography: Evaluation of total anomalous pulmonary venous connection

Summary Total anomalous pulmonary venous connection (TAPVC) has a relatively high surgical mortality, especially the infracardiac variety. A small left atrium may limit postoperative cardiac output. Superimposition of digitally subtracted angiographic (DSA) images may be used to define pulmonary venous anatomy, left atrial size, and its spatial relationship to the common pulmonary vein. The technique for acquiring superimposed digitally subtracted images and the results of two cases with infracardiac TAPVC are presented. We have found that this superimposition technique is easily performed and may provide important preoperative information in infants with TAPVC.     

完全性肺静脉异位引流手术预后的病例对照研究

目的 探讨完全性肺静脉异位引流(TAPVD)的临床特点和手术治疗效果。方法 2012年6月至2018年6月于我院住院行外科手术治疗并符合纳入标准的TAPVD患儿,分为事件组(发生终点事件)和非事件组(未发生终点事件)。采用SPSS 25软件进行数据分析,计量资料用中位数表示。组间比较用Mann-Whitney U检验。应用COX多因素回归分析发生终点事件的危险因素。结果 138例TAPVD患儿纳入分析,心上型65例,心内型44例,心下型19例和混合型10例,手术年龄中位数50 d,<6月龄123例(89.1%),<1月龄60例(43.5%)。事件组42例,非事件组96例。术前存在肺静脉梗阻(PVO)患儿63例(45.7%),术后发生PVO患儿41例(29.7%)。心下型、混合型、手术年龄、术前存在POV、需要急诊手术及体外循环时间在事件组与非事件组之间分布差异有统计学意义。COX多因素回归分析结果显示心下型(P=0.005)、混合型(P=0.014)、术前存在PVO(P=0.020)、手术年龄<1月龄(P=0.009)及体外循环时间长(P=0.024)是发生终点事件的危险因素。结论 心上型是最常见的TAPVD类型,心下型、混合型、术前存在PVO、手术年龄<1月龄及体外循环时间长是预后差的危险因素。     

完全性肺静脉异位引流手术预后的病例对照研究

目的 探讨完全性肺静脉异位引流(TAPVD)的临床特点和手术治疗效果。方法 2012年6月至2018年6月于我院住院行外科手术治疗并符合纳入标准的TAPVD患儿,分为事件组(发生终点事件)和非事件组(未发生终点事件)。采用SPSS 25软件进行数据分析,计量资料用中位数表示。组间比较用Mann-Whitney U检验。应用COX多因素回归分析发生终点事件的危险因素。结果 138例TAPVD患儿纳入分析,心上型65例,心内型44例,心下型19例和混合型10例,手术年龄中位数50 d,<6月龄123例(89.1%),<1月龄60例(43.5%)。事件组42例,非事件组96例。术前存在肺静脉梗阻(PVO)患儿63例(45.7%),术后发生PVO患儿41例(29.7%)。心下型、混合型、手术年龄、术前存在POV、需要急诊手术及体外循环时间在事件组与非事件组之间分布差异有统计学意义。COX多因素回归分析结果显示心下型(P=0.005)、混合型(P=0.014)、术前存在PVO(P=0.020)、手术年龄<1月龄(P=0.009)及体外循环时间长(P=0.024)是发生终点事件的危险因素。结论 心上型是最常见的TAPVD类型,心下型、混合型、术前存在PVO、手术年龄<1月龄及体外循环时间长是预后差的危险因素。     

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.     

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.     

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.     

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.     

超声心动图对儿童完全性肺静脉异位引流诊断价值的探讨

目的　旨在评价超声心动图对完全性肺静脉异位引流 (TAPVD)的诊断价值。方法　对 1987年 9月至2 0 0 2年 11月收治的TAPVD患儿 70例进行分析。均有完整的临床及超声心动图资料 ,其中 35例施行了手术治疗。结果　 70例TAPVD的解剖分型为 :心内型 5 2例、心上型 16例、心下型 2例 ,未发现混合型。 35例施行手术 ,32例与术前超声心动图的诊断一致 ;3例与超声心动图诊断不完全一致 ,其超声心动图诊断分别为 :三房心伴继发孔房缺、左房隔膜伴继发孔房缺、原发孔房缺伴卵圆孔未闭 ,而手术结果均显示为心内型TAPVD伴继发孔房缺。结论　超声心动图检查是诊断TAPVD重要且准确的方法 ,并能确定其分型 ,提供精确的解剖及血流动力学资料。在应用超声心动图诊断TAPVD时 ,应注意与三房心、左房隔膜等疾病进行鉴别。