完全性肺静脉异位引流的手术治疗(附44例报告)

目的 探讨完全性肺静脉异位引流（TAPVC）的手术方法和治疗经验。方法对44例TAPVC患者施行手术治疗，其中心上型29例，心内型13例，心下型2例。手术在中度低温体外循环下进行．心上型早期行心脏上翻法吻合、中期行左右房联合切口法，近期改良为心上吻合法。心下型采用心脏上翻纵切口吻合。结果手术死亡2例（心上型和心内型各1例）；心律失常12例，出院时恢复安性心律。结论TAPVC一经确诊．必须立即手术，以防止肺血管阻塞性病变的发生。     

改良左心房上吻合法矫治心上型完全性肺静脉异常回流

目的总结从左心房顶部行改良左心房-共同静脉吻合(左心房上吻合法)治疗心上型完全性肺静脉异常回流(total anomalous pulmonary venous connection,TAPVC)的经验。方法 2005年1月至2011年8月高州市人民医院收治心上型TAPVC 17例,17例肺静脉均是经垂直静脉、无名静脉、右上腔静脉后回流至右心房,其中男7例,女10例,年龄中位数为1岁(40 d～24岁);体质量中位数为12.5 kg(3.1～31.0 kg),全组12例合并房间隔缺损,3例合并卵圆孔未闭,2例合并房间隔缺损及动脉导管未闭,均采用左心房上吻合法。结果手术死亡2例,1例死于低心排血量综合征;1例术后反复肺感染,死于呼吸衰竭。1例因术后急性肺水肿,二次手术重新开放垂直静脉延迟闭合治愈;2例心律失常(频发房性期前收缩),治疗一周恢复正常。无肺动脉高压危象。术后随访3个月至6年,平均3.5年,无吻合口狭窄和心律失常,发绀消失,活动耐力显著增加,生长发育良好。结论改良左心房上吻合法治疗心上型TAPVC,手术暴露好、操作简单、心律失常发生率低,术后中期随访结果良好。     

完全性肺静脉异位引流的外科治疗

目的:总结完全性肺静脉异位连接(TAPVC)患者的外科治疗经验,以提高手术疗效。方法:20例患者均在中度低温体外循环下行TAPVC矫治术。心上型12例,心内型7例,混合型1例。合并动脉导管未闭2例,肺动脉瓣狭窄及三房心各1例,三尖瓣关闭不全7例。结果:术后早期(30 d)死亡1例,死于术后严重低心排出量综合征,生存19例均获得随访,随访时间1～5年,心功能恢复良好;术后2周超声示右心房室明显缩小,左心房室扩大。结论:完全性肺静脉畸形引流一经确诊应尽早手术,手术的关键是保证吻合口够大和避免吻合口狭窄及心律失常。心上法可为肺静脉主干与左心房顶部吻合提供良好显露并可降低心律失常发生率,是心上型TAPVC左心房与肺静脉总干吻合良好途径。     

完全性肺静脉异位连接的手术治疗

目的观察完全性肺静脉异位连接（TAPVC）患者的手术治疗效果。方法在中度低温体外循环下对23例TAPVC患者行TAPVC矫治术。结果术后早期死亡1例;并发阵发性结性心律、短阵性房性心动过速、左侧膈肌麻痹各1例。22例出院后均获随访（2.8±1.5）a,1例心上型患者术后8个月因左肺静脉回流梗阻再次入院经手术治愈,1例反复发作房性心律失常经内科治疗无效。结论手术是治疗TAPVC的有效手段。     

69例小儿心上型完全性肺静脉异位引流超声诊断与临床分析

目的:总结心上型完全性肺静脉异位引流(TAPVC)超声心动图的诊断特点及对围术期临床转归的指导意义。方法:对2010年6月至2015年6月5年间我院儿童心脏中心收治的心上型TAPVC患儿的超声心动图及临床资料进行回顾性分析。结果:经超声心动图诊断并外科手术证实为心上型TAPVC 69例,男性46例,女性23例,中值年龄5.0个月(21d~180个月)。69例术前超声心动图诊断均与手术诊断一致,符合率100%。设定超声测量房间隔交通≤5mm为限制性房间隔交通(限制性房水平分流)。本组研究提示限制性房间隔交通组就诊年龄(P=0.005)、体表面积(P=0.015),经皮血氧饱和度(P=0.039),房间隔交通直径指数(P0.001)等均明显低于房间隔交通直径5mm患儿组(非限制性分流);而肺动脉收缩压(P=0.002),右心室舒张末径指数,右心室与左心室前后径比值则明显高于非限制性分流组(P0.001),且术后ICU时间明显长于非限制性分流组(P=0.01)。结论:超声心动图是诊断心上型TAPVC首选且准确的方法。心上型TAPVC尽早诊断手术预后良好。房水平分流大小,引流血管是否梗阻,术前右心室与左心室内径比值与心上型TAPVC患儿围术期转归密切相关。     

完全性肺静脉异位连接的外科治疗

目的:探讨完全性肺静脉异位连接(total anomalous pulmonary venous connection,TAPVC)的手术方法及疗效。方法:选取5例TAPVC患者,其中1例心上型患者行左心房共同静脉吻合;1例心内型患者将房间隔缺损扩大,应用补片将异位引流的肺静脉隔至左心房;3例混合型患者中有2例垂直静脉与左心耳吻合,补片将异位引流的肺静脉共同腔隔至左心房,1例扩大房间隔缺损后行心包片补片将异位引流的肺静脉隔至左心房。结果:所有患者均痊愈并获得随访,随访时间8个月～2年,心功能基本正常。结论:TAPVC患者一旦诊断明确应及早手术,正确认识肺静脉复杂的解剖,选择适当的手术方法可获良好的治疗效果。手术治疗的关键在于肺静脉流入左心房足够宽畅,避免狭窄。     

完全性肺静脉异位引流矫治术后肺静脉梗阻的治疗探讨

目的:总结完全性肺静脉异位引流(TAPVC)矫治术后肺静脉梗阻的治疗经验。方法:回顾性分析2011-01至2015-12于青岛市妇女儿童医院心脏中心施行TAPVC矫治术后发生肺静脉梗阻16例患儿的临床资料,其中男性10例。所有患儿均出院前、术后1个月、3个月、6个月、12个月、24个月回院复诊,包括经胸心脏超声多普勒、心电图、X线胸部正侧位片。以经胸心脏超声多普勒检查肺静脉流速2 m/s为梗阻标准,诊断肺静脉梗阻。结合患儿临床表现(反复心力衰竭、生长发育迟缓)确定二次手术时机。二次手术采用sutureless缝合技术和常规补片扩大技术。结果:术后发生肺静脉狭窄16例患儿无失访,其中7例吻合口周围狭窄(混合型1例,心下型3例,心上型2例,心内型1例);7例单支肺静脉狭窄,2例双支肺静脉狭窄,无三支及以上肺静脉狭窄。按术前Darling类型分:混合型2例,心下型5例,心上型5例,心内型4例。11例(68.8%)术后肺静脉狭窄流速增快发生在术后3~6个月。5例二次手术患儿中,4例采用sutureless缝合技术,1例采用常规补片扩大技术,均为吻合口周围梗阻狭窄,2例死亡,3例患儿随访中。结论:TAPVC矫治术后吻合口周围狭窄为主要二次手术适应证,并且需早期即行手术。     

完全性肺静脉异位引流的外科治疗

目的 :探讨完全性肺静脉异位引流 （TAPVD）的手术方法 ,评价其效果。方法 :本组患者 2 9例 ,均在体外循环下行矫治术。心上型采用心房横断法 ,应用补片扩大左房 ;心内型及混合型采用扩大房间隔缺损 ,用大补片将异位引流的肺静脉隔至左房 ;混合型上腔静脉用心包补片加宽。结果 :全组无手术死亡 ,出院时心功能 1级。随访 （2 2 /2 9例 ） 3～ 15年 ,心功能均正常。结论 :按 TAPVD的类型采用不同的手术方法。对 TAPVD的外科治疗只要诊断明确、手术方法得当、术后严密监护可获良好的治疗效果     

新生儿复杂先天性心脏病外科治疗--一年经验回顾

目的回顾分析过去一年新生儿复杂先天性心脏病手术治疗结果.方法2004年5月～2005年5月我院新生儿手术临床资料.手术共完成21例,其中室间隔完整的大动脉转位(TGA/IVS)13例;合并室间隔缺损的大动脉转位(TGA/VSD)2例;右心室双出口(DORV、Taussing-Bing)1例;完全性肺静脉畸形引流(TAPVC)2例(心下型及心上型各1例);肺动脉闭锁(PAA)3例.分别行动脉调转术(ASO)16例,完全性肺静脉畸形引流矫治术2例,改良Blalock-Taussing手术3例(其中肺动脉瓣闭式扩张术2例).结果全组存活18例,死亡3例,死亡率14.3%.2例完全性大动脉转位,1例为冠状动脉壁内走行,术后死于低心排血量综合征,1例死于肺部感染.另1例为心上型完全性肺静脉畸形,术后因肺部感染导致肾功能衰竭死亡.结论准确认识新生儿复杂先天性心脏病的病理生理,是正确选择手术时机,提高围手术期治疗的关键.     

31例完全性肺静脉异位连接的外科治疗

目的探讨31例完全性肺静脉异位连接的手术方法及术后疗效。方法31例患者（心上型16例、心内型13例、混合型2例）均在体外循环下行矫治术。心上型6例采用传统双心房联合切口，10例采用心上法，后者经上腔静脉与升主动脉之间显露左心房与共同肺静脉干行侧一侧吻合；心内型切开房间隔缺损与冠状静脉窦开口之间的残余房间隔壁，自体心包将肺静脉开口与冠状静脉窦口隔入左房；混合型则根据不同的病理解剖特点行相应的矫治术。结果早期死亡2例，其他患者都痊愈出院。术后随访3个月至14年，恢复良好，无吻合口狭窄发生，心功能均为Ⅰ级，无因心肺功能衰竭死亡患者。结论完全性肺静脉异位连接早期诊断和及早行矫治术极为重要，手术治疗的关键在于左心房与肺总静脉吻合口应足够大，避免狭窄的发生。     

经左房顶部矫治心上型完全性肺静脉异位引流的体会

目的探讨经左房顶部径路矫治心上型完全性肺静脉异位引流的体会。方法2005年1月至2008年1月经左房顶部径路矫治心上型完全性肺静脉异位引流5例,女性3例,男性2例,年龄2-13（7．00±3．85）岁,在全麻体外循环下经左房顶部径路进行矫治。结果均治愈出院,随访0．5～2．5年,生长发育良好,无心律失常和吻合口狭窄。结论经左房顶部径路矫治心上型完全性肺静脉异位引流,手术方便,显露好,减少心律失常和吻合口狭窄的发生。     

Palliative stent placement in vertical vein in a 1.4 kg infant with obstructed supracardiac total anomalous pulmonary venous connection

Temporary relief of obstructed total anomalous pulmonary venous connection (TAPVC) may stabilize the preoperative clinical status. We report a 1.4 kg infant with obstructed supracardiac TAPVC to right sided superior vena cava, double outlet right ventricle, pulmonary atresia, major aortopulmonary collateral arteries, and atrioventricular septal defect. A palliative percutaneous stent was successfully placed and resulted in immediate clinical improvement. We performed a review of literature regarding interventional procedures for relief of obstructed TAPVC and found 17 cases including stent placement (13), balloon angioplasty (3), and Hybrid procedure (1). We conclude that temporary stent placement in obstructed TAPVC is feasible and efficacious in relieving pulmonary venous obstruction. The procedure can lead to subsequent surgical repair in a more favorable clinical status and has special significance in the low birth weight infant. © 2012 Wiley Periodicals, Inc.     

完全性肺静脉异位引流的发病机制研究进展

完全性肺静脉异位引流（total anomalous pulmonary venous connection，TAPVC）是一类罕见而又严重的先天性心脏病。TAPVC是因肺静脉未能与左心房相连，而直接与右心房或体循环静脉系统连接，导致该类患儿生长发育明显受限，生后全身紫绀及缺氧表现逐渐加重，严重威胁患儿的生命，需尽早手术干预。TAPVC的发病原因和机制尚未完全明确，近年来随着现代分子遗传学的发展，对其的发病机制有了初步的认识，本文就TAPVC发病机制研究进展作一概述。     

Supracardiac total anomalous pulmonary venous connection with cor triatriatum sinister: A rare diagnosis confirmed by saline contrast echocardiography

In cor triatriatum sinister, the left atrium is divided by a membrane into a proximal and a distal chamber. Usually proximal chamber receives all the pulmonary veins and drains through an opening in the dividing membrane into distal chamber, which empties into left ventricle through the mitral valve. Rarely, the two chambers lack a communication and there is associated total anomalous pulmonary venous connection (TAPVC). We report a 1-month-old infant with cyanosis and heart failure, who had cor triatriatum sinister associated with supracardiac TAPVC. The case is reported for rarity of the association with a focus on contrast echocardiographic imaging.     

Detection of shunt flow in total anomalous pulmonary venous connection using 2D-Doppler echocardiography

Two-D-Doppler echocardiography was used to diagnose two cases of total anomalous pulmonary venous connection (TAPVC). The first case presented the supracardiac type, the second the infracardiac type. Blood flow imagings produced by 2D-Doppler echocardiography revealed shunt flow in the common pulmonary veins (CPV) and the vertical, innominate, and portal veins. The shunt flow in the vertical vein was laminar, though the shunt flow in the inflow tract of the right atrium was turbulent. Two dimensional Doppler echocardiography is very useful in the detection shunt flow in TAPVC.     

Echocardiographic diagnosis of total anomalous pulmonary venous return

Between March 83 and November 84, 20 patients with TAPVC were studied by cross-sectional echocardiography. The diagnosis was confirmed by catheterization, surgery or autopsy in each case. The anomalous drainage of the pulmonary veins was supracardiac in 10 patients, to the coronary sinus in 5, infracardiac in 4, mixed (supracardiac and infracardiac) in 1. The correct diagnosis was prospectively achieved in 17 patients (85); the echocardiographic examination could not show the precise site of the drainage in 2 (10); in only one patient (5) with right isomerism, double inlet indeterminate ventricle and severe pulmonary stenosis, the diagnosis of TAPVC infracardiac was missed. Seven patients (4 with TAPVC supracardiac, 3 to the coronary sinus) were sent to surgery without preoperative catheterization; the diagnosis was confirmed in all. Cross-sectional echocardiography proved to be a reliable diagnostic tool in the assessment of TAPVC. The patients in which the site of anomalous drainage is clearly identified by echo, can be sent to surgery without invasive investigation.     

Echo Doppler detection of external compression of the vertical vein causing obstruction in total anomalous pulmonary venous connection

External obstruction of the pulmonary venous return was detected by two-dimensional (2-D) and pulsed Doppler echocardiography and was confirmed by angiography and at surgery in two infants with isolated supracardiac total anomalous pulmonary venous connection (TAPVC) to the left vertical vein. In both infants, the left vertical vein ascended behind the left pulmonary artery and anterior to the left main stem bronchus. This arrangement produces mechanical obstruction to both vertical vein flow and pulmonary venous drainage. Early detection and surgical correction of obstructed pulmonary venous return are necessary to prevent severe right-sided heart failure and death. Doppler echocardiography is recommended when evaluating infants with TAPVC for possible anatomic or mechanical obstruction to pulmonary venous return.