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。     

胎儿期完全型大动脉转位超声诊断的特征

目的探讨胎儿完全型大动脉转位(C-TGA)超声心动图的特征及诊断价值。方法分析6例经手术或尸解证实的C-TGA的新生儿超声心动图检查,并回顾分析其胎儿期漏诊误诊原因,总结胎儿C-TGA超声心动图的诊断体会。结果6例新生儿大动脉转位,其中胎儿期超声漏诊5例,误诊1例。其中产前仅诊断室间隔缺损或右室双出口,经出生后手术证实的C-TGA4例,尸解证实C-TGA 1例。结论C-TGA在胎儿期已有明确超声声像特征,加强医师对先天性心脏病知识的系统学习,降低漏诊误诊率是提高筛查质量的关键。     

新生儿及婴儿完全型肺静脉异位引流的外科治疗

目的　回顾性探讨新生儿及婴儿完全型肺静脉异位引流 (TAPVC)的早期外科手术效果。方法　 2 0 0 1年 3月～ 2 0 0 3年 4月 ,共收治 8kg以下完全型肺静脉异位引流患儿 13例 ,男 11例 ,女 2例。体重 (3.5～ 8)kg ,平均体重 (5 .0 3± 1.15 )kg。年龄 2 0d～ 11个月 ,平均年龄 (3.73± 3.30 )个月。心上型 6例 (4 6 .15 % 6 /13) ,心内型 4例 (30 .77% 4 /13) ,心下型 2例 (15 .38% 2 /13) ,混合型 1例 (7.6 9% 1/13)。所有患儿均在全麻低温体外循环下行矫治术。结果　术后早期死亡 2例(15 .38% ) ,均为心上型 ,2例心下型全部存活 ,随访 9例 ,时间为 2个月～ 2年 ,心功能均良好。结论　完全型肺静脉异位引流早期矫治效果良好 ,心下型肺静脉异位引流一旦确诊应急诊手术 ,不增加手术死亡率。     

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,     

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。     

胎儿超声心动图检查的多中心临床评价

目的 评价高危人群中进行胎儿超声心动图检查的诊断价值。方法 2003年1月至2004年12月,在上海两家三级妇产科医院进行高危人群中胎儿先天性心脏病产前诊断前瞻性研究。运用二维和彩色／脉冲多普勒超声进行详细化胎儿超声心动图检查,随访并评价诊断灵敏度、特异度及患儿预后。结果研究期间共2063例胎儿接受检查,检查时平均孕周为26,5周（16—42周）。产前诊断胎儿先天性心脏病43例,诊断时平均孕周27．3周（16—40周）,异常病例检查指征中“产科超声检查发现胎儿心脏可疑异常”占67．4％。产前诊断灵敏度92.1％,特异度99．9％。准确性86．1％。随访病例中,52．1％终止妊娠,2．2％宫内死亡,13％新生儿或婴儿期死亡,28．3％存活。结论（1）目前临床实践中胎儿超声心动图检查是在高危人群中进行先天性心脏病产前诊断的有效方法。（2）产科超声筛查提示胎儿心脏畸形是检查的重要指征。（3）完整的分段诊断是提高诊断准确性的关键。（4）患有先天性心脏病的胎儿预后不佳,由产科与儿科医生参与的多学科合作可能有助于改善胎儿预后。     

完全性肺静脉异位回流的诊断：附15例分析

报道１９８９年１月～１９９３年８月收治完全性肺静脉异位回流（ＴＡＰＶＣ）１５例，心上型８例、心内型６例、心下型１例。９例经尸检证实。诊断（包括回流部位）符合率１００％。此病男性多见；临床表现及心电图无特异性；胸部Ｘ线检查心影呈“雪人”型有助心上型诊断；心血管造影和心脏超声诊断最可靠；心房正位引流部位单一旦不合并其他心内畸形的病例手术前可免作心血管造影检查。     

肺静脉异位引流12例

肺静脉异位引流 (ＡＰＶＣ)是一种少见的先天性心脏病 ,临床表现不典型。 1995年 10月～ 2 0 0 1年 12月我院心脏外科共收治ＡＰＶＣ患儿 12例 ,现报告如下。临床资料一、一般资料　ＡＰＶＣ患儿 12例 ,男 9例 ,女 3例 ;年龄 8个月～ 10岁 ,平均 4 .2±1.6岁。 3岁以下 6例 ,体重 5～ 2 5ｋｇ。均经Ｘ线胸片、心电图 (ＥＫＧ)、彩色超声心动图 (ＵＣＧ)检查 ,其中 5例经心导管及心血管造影 ,均以手术结果为诊断依据。二、分型[1] 　 12例中部分型肺静脉异位引流 (ＰＡＰＶＣ) 8例 ,其中 7例并房间隔缺损 (ＡＳＤ) ,1例同时并左上腔 ;另 1例…     

完全性肺静脉异位引流1例

完全性肺静脉异位引流（total anomonous pulmonary venous drainage,TAPVD）是一种少见的先天性心脏畸形,占全部先天性心脏畸形的1．5％-3％。我院近期收治1例,现报告如下。     

混合型完全性肺静脉异位连接17例的诊断与治疗

目的探讨儿童混合型完全性肺静脉异位连接(TAPVC)的诊治要点及影响预后的主要因素。方法近5 a来,在广东省心血管研究所确诊为混合型TAPVC的患儿共17例,收集该17例患儿的临床资料和随访结果,进行回顾性分析。结果 17例混合型TAPVC患儿中心上型+心内型14例,心上型+心下型3例,并肺静脉回流梗阻6例。17例术前全部行超声心动图检查,其中13例行多排螺旋CT扫描,14例行手术治疗。行CT扫描者,10例经手术探查证实此10例患儿CT能够准确显示所有肺静脉回流径路。行超声检查者,12例超声报告了所有4条肺静脉回流径路,5例超声未能报告肺静脉全部回流径路。超声报告所有肺静脉回流径路者,经CT或手术探查,证实其中7例超声显示的所有肺静脉回流径路均准确,4例超声准确显示了3条肺静脉回流径路,1例超声显示的肺静脉回流径路完全错误。并肺静脉回流梗阻的6例患儿,超声心动图发现6例,而CT仅发现1例。14例行手术治疗者,急诊手术6例,择期手术8例。围术期死亡2例,均为急诊手术者。12例康复出院者,随访时间2～53(28.8±17.4)个月,中位随访时间30个月。术后3例发生吻合口狭窄,其中1例术后2个月死于严重吻合口狭窄导致的急性肺水肿。结论混合型TAPVC以心上型+心内型最多见。结合超声心动图和多排螺旋CT扫描是术前评估的最佳策略。对肺静脉回流径路的显示多排螺旋CT优于超声心动图,对肺静脉回流梗阻的显示超声心动图优于多排螺旋CT。急诊手术可能是围术期死亡的危险因素。术后随访主要并发症为吻合口狭窄。     

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.     

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.     

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.     

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.     

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.     

Chest X-ray film patterns in children with isolated total anomalous pulmonary vein connection

Chest-X-ray films of 61 children with isolated total anomalous pulmonary vein connection (TAPVC) were re-evaluated retrospectively. A small heart silhouette with normal pulmonary perfusion and signs of pulmonary oedema is typical of TAPVC with obstruction. There is no typical pattern in TAPVC without obstruction. The snowman or figure of eight does not develop within the first months of life.     

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

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.     

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.