完全性肺静脉异位引流的外科治疗体会(附16例报告)

完全性肺静脉异位引流 ( TAPVC)为一种少见的先天性心脏畸型 ,发病率占先天性心脏病的 1 %～3% ,自然病程预后极差 ,其中 5 0 %于生后 3个月内死亡 ,80 %于 1岁以内死亡 ,存活至 1岁以上的患儿常合并较大的房间隔缺损。 1 995年 6月至 2 0 0 2年 6月 ,我院共实施 TAPVC矫正术 1 6例 ,取得较好的效果。现总结报告如下。1　资料与方法1 .1　临床资料　本组 TAPVC患儿 1 6例 ,男 7例 ,女 9例 ;年龄 3个月至 6岁 ,平均 1 .2岁。均经心脏超声和多普勒检查明确诊断 ,1例行心导管检查和心血管造影。按 Darling分类法均为心上型 ,均合并房间…     

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。     

单心室合并完全性肺静脉异位引流的外科治疗

目的:总结单心室合并完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法:入选2008-09至2016-03期间,我院手术治疗的单心室合并TAPVC患儿25例,年龄5天~14岁,体重3.3~49.5kg,心上型TAPVC 22例,心下型TAPVC 1例,混合型TAPVC 2例;合并完全性房室间隔缺损17例,三尖瓣闭锁2例,二尖瓣闭锁2例;中度以上房室瓣反流4例,肺动脉闭锁5例,肺静脉梗阻9例。结果:院内早期死亡5例,死亡率20%(5/25),死亡原因均为低心排血量综合征。存活20例患儿随访1~65个月,7例再次手术,其中5例完成二期改良Fontan术,1例因重度房室瓣反流行房室瓣置换术,另1例因肺静脉梗阻及重度房室瓣反流行肺静脉狭窄矫治+房室瓣成形术;余13例患儿未再次手术。20例出院患者随访期间3例死亡,死亡原因分别为低心排血量综合征、蛋白丢失性肠病和神经系统损伤。余存活患儿一般情况好,未发现残余肺静脉梗阻。结论:单心室合并TAPVC常合并右心房异构,心内畸形复杂,手术治疗仍具有较高死亡率。     

单心室合并完全性肺静脉异位引流的外科治疗

目的:探讨单心室合并完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法:2009-05至2011-09,我科共收治单心室合并TAPVC患儿11例,年龄(30.1±21.4)个月,体重(11.0±3.2)kg。例2行改良B-T分流术,例1、3～6、8、9、11行一侧或双侧双向腔肺分流术,例7、10行一期改良Fontan术。例1、6～9、11同期行TAPVC矫治术。结果:例6、10围术期死亡(18.2%,2/11),死亡原因分别为中枢神经系统感染和多器官功能衰竭。存活的患儿随访1～29个月,例4死亡,余患儿氧饱和度改善,未发现残余肺静脉梗阻,例5行二期改良Fontan术并顺利出院。结论:单心室合并TAPVC患儿仍具有较高早期死亡率,术前明确诊断TAPVC并制定合理的个体化治疗方案是提高手术疗效的关键。     

137例婴幼儿完全性肺静脉异位引流各年龄段临床特点和外科疗效分析

目的:探讨婴幼儿完全性肺静脉异位引流(TAPVC)各年龄段的临床特点和外科治疗效果.方法:2000-01至2006-12 诊断TAPVC并在我院进行手术治疗的婴幼儿(除外合并法乐四联症、右心室双出口、完全性房室通道、单心室等复杂畸形的患儿)共137例.分为≤28天新生儿组(组1,n=7)、＞28天～≤6个月组(组2,n=70)、＞6个月～≤1岁组(组3,n=26)、＞1岁～≤3岁组(组4,n=34)4个年龄组.137例患儿伴房间隔缺损119例,卵圆孔未闭18例,动脉导管未闭27例,室间隔缺损2例,三尖瓣关闭不全103例.合并重度肺动脉高压50.4%(69/137).其中≤6个月患儿(组1、组2)占71.0%(49/69).心上型49.6%(68/137),心内型37.2%(51/137).心下型1.5%(2/137),混合型11.7%(16/137).合并肺静脉狭窄18.2%(25/137),其中≤6个月患儿(组1、组2)占88.0%(22/25).结果:手术死亡率11.7%(16/137),组1和组2占87.5%(14/16).主要死亡原因为肺部感染和低心排综合征.有随访资料99例,随访率81.8%(99/121),随访期限2～86个月,平均(25.5±22.3)个月.晚期死亡9例,均为组1和组2患儿,晚期死亡率9.1%(9/99).结论:年龄＞6个月的TAPVC患儿可获得满意的外科治疗效果,而年龄≤6个月尤其是新生儿患儿病情重,合并限制性房间隔缺损、肺静脉狭窄和重度肺动脉高压比例高,往往需要急诊手术,手术及远期死亡率较高.     

新生儿完全性肺静脉异位连接外科治疗的临床分析

目的:评估外科手术治疗新生儿完全性肺静脉异位连接(TAPVC)的近中期临床疗效。方法:2010-11至2014-01,我院确诊为TAPVC的新生儿20例(其中男性15例,女性5例)均接受外科手术治疗。平均手术年龄(10.2±4.8)d,体重(3.2±0.4)kg。所有患儿术前均行超声心动图检查,部分患儿行心脏计算机断层摄影术(CT)检查。术后1个月、3个月、6个月、12个月及24个月定为临床随访时间,复查以超声心动图为主,必要时复查心电图及X线胸片,怀疑肺静脉梗阻者加做心脏CT检查。结果:全组无手术早期死亡,晚期死亡1例。平均体外转流时间(83.7±255)min,平均主动脉阳断时间(41.0±19.4)min。术后中位机械通气时间4d(范围2~128 d),中位正性肌力药物支持时间3 d(范围2~128 d),中位住院时间21 d(范围13~128 d)。19例存活者中平均随访(19.5±11.8)个月,其中2例术前有双侧肺静脉发育不良仍伴重度肺动脉高压、心脏功能Ⅲ级,其余17例恢复良好,心脏功能Ⅰ~Ⅱ级。结论:外科手术治疗新生儿TAPVC近中期疗效满意,伴双侧肺静脉发育不良者预后不佳。     

先天性心脏病婴幼儿外科治疗对策分析(附115例报告)

目的 探讨婴幼儿先天性心脏病外科围术期处理对策.方法我院于2008年6月至2011年12月对115例患先天性心脏病的婴幼儿实施外科手术治疗,男性73例,女性42例,年龄3个月至3岁,体重3.5～20.0 kg;其中室间隔缺损(VSD)54例,法洛四联症(TOF)14例,室间隔缺损合并房间隔缺损(VSD+ASD)10例,房间隔缺损(ASD)9例,动脉导管未闭(PDA)8例,肺动脉瓣狭窄(PS)7例,室间隔缺损合并动脉导管未闭(VSD+PDA)5例,完全型肺静脉异位引流(TAPVC)4例,右室双出口(DORV)3例,主动脉缩窄合并室间隔缺损(CoA+VSD)1例.结果全组死亡5例,死亡率为4.3%.死亡原因:低心排出量综合征1例,肺高压危象2例,呼吸道梗阻2例.结论先天性心脏病患儿在婴幼儿期外科治疗可以取得满意疗效,根据婴幼儿生理、病理特点采取个体化治疗对策是成功的关键.     

小儿混合性完全型肺静脉异位引流的超声诊断经验

目的总结经胸超声心动图(TTE)在小儿混合型完全性肺静脉异位引流(TAPVC)术前诊断、术后随访的价值和经验。方法选择自2010年1月-2016年12月在中国医学科学院北京阜外医院小儿中心经手术证实为混合型TAPVC的22例患者,将术前术后超声资料与CT检查及手术结果对照进行回顾性研究。结果本组混合型TAPVC约占总体TAPVC的5.4%。22例患儿均成功接受了手术矫治,其中包括:心上型+心内型19例,心上型+心下型3例;其中合并肺静脉回流梗阻7例(含单侧肺静脉发育不良1例),共占约31.8%。超声术前准确探查全部回流途径的病例占(13/22)59.1%,误判为心上型TAPVC 2例,误判为心内型TAPVC 5例,术前漏诊1例为法鲁氏四联症合并肺静脉发育不良患儿。超声对肺静脉回流途径梗阻部位的诊断率为(8/13)61.5%。术前共12例患儿(54.5%)接受了增强螺旋CT检查,CT对各支肺静脉回流途径均判断准确,对肺静脉回流梗部位检出率约38.5%。所有患儿术后6个月内均接受至少一次超声复查,中位随访时间为4个月。截止最后一次超声随访资料显示有5例患儿术后仍存在肺动脉高压,其中3例出现肺静脉与左房吻合口或分支狭窄,发生于术后2个月-4个月。结论超声心动图可为儿童混合型TAPVC提供重要的诊断线索和随访信息,熟悉本病不同类型、了解少见的变异情况有助于提高超声诊断水平,有利于评估患儿手术风险及预后。     

1岁以内完全性肺静脉异位引流外科治疗86例疗效分析

目的:回顾性总结婴儿完全性肺静脉异位引流(TAPVC)外科治疗经验及其效果分析,提高手术疗效。方法:回顾分析河北省儿童医院2012年1月至2015年12月TAPVC患儿86例的临床资料,其中男性66例,女性20例,年龄5d~12个月,平均5.11个月;体质量2.3~9 kg,平均(5.67±2.08)kg,均经心脏彩超、胸部X线片、心电图检查确诊,矫治手术均在常规体外循环中低温(28℃左右)下完成。结果:当时住院死亡4例,术后肺静脉狭窄再手术死亡1例,总病死率5.81%。术中心力衰竭死亡2例,严重感染、多器官衰竭1例,低心排出量综合征2例。81例存活者术后复查结果满意,出院后随访3~36个月,身体发育、活动能力正常,无二次手术者。结论:完全性肺静脉异位引流是一种危重先天性心脏病,尤其伴梗阻性的需急症手术治疗,手术矫治可以明显改善患儿的生存质量。     

67例新生儿完全性肺静脉异位引流的外科治疗及早中期结果

目的:回顾总结67例新生儿完全性肺静脉异位引流的手术治疗经验。方法:自2011年1月至2018年1月,本中心共完成新生儿完全性肺静脉异位引流矫治手术67例。其中男性46例,女性21例;手术中位年龄12 d(1～28 d);手术平均体质量(3.2±0.5)kg(2.1～4.5 kg)。Darling分型:心上型30例,心内型7例,心下型29例,混合型1例。合并卵圆孔未闭18例,合并继发孔房间隔缺损49例,合并动脉导管未闭39例,术前肺静脉梗阻50例(74.6%)。全部患儿均在全麻中浅低温体外循环下手术。结果:全组术后早期死亡6例(9.0%)。随访59例,中位随访时间45个月(6个月～7年)。术后肺静脉梗阻5例(8.5%)。4例吻合口狭窄行吻合口扩大术,术后恢复良好。1例肺静脉开口及分支狭窄采用sutureless技术,术后死于低心排。余患儿发育良好。结论:完全性肺静脉异位引流自然预后差,应尽早手术。新生儿完全性肺静脉异位引流的手术治疗早期结果满意,术后肺静脉梗阻需要长期随访。     

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。     

255例完全性肺静脉异位引流手术治疗的单中心回顾性研究

目的:分析单中心完全性肺静脉异位引流(TAPVC)手术治疗的早中期效果.方法:连续入组2009年1月至2019年10月首都医科大学附属北京儿童医院心脏外科收治并进行手术治疗的0～3岁TAPVC患儿共255例.按照年龄将患儿分为两组:0～6个月组(A组,n=195)和6个月以上～3岁组(B组,n=60),总结其临床特点,...     

BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease.

The aim of the present study was to determine if patients with both pulmonary arterial hypertension (PAH), due to pulmonary vascular obstructive disease, and congenital heart defects (CHD), have mutations in the gene encoding bone morphogenetic protein receptor (BMPR)-2. The BMPR2 gene was screened in two cohorts: 40 adults and 66 children with PAH/CHD. CHDs were patent ductus arteriosus, atrial and ventricular septal defects, partial anomalous pulmonary venous return, transposition of the great arteries, atrioventicular canal, and rare lesions with systemic-to-pulmonary shunts. Six novel missense BMPR2 mutations were found in three out of four adults with complete type C atrioventricular canals and in three children. One child had an atrial septal defect and patent ductus arteriosus; one had an atrial septal defect, patent ductus arteriosus and partial anomalous pulmonary venous return; and one had an aortopulmonary window and a ventricular septal defect. Bone morphogenetic protein receptor 2 mutations were found in 6% of a mixed cohort of adults and children with pulmonary arterial hypertension/congenital heart defects. The current findings compliment recent reports in mouse models implicating members of the bone morphogenetic protein/transforming growth factor-beta pathway inducing cardiac anomalies analogous to human atrioventricular canals, septal defects and conotruncal congenital heart defects. The small number of patients studied and the ascertainment bias inherent in selecting for pulmonary arterial hypertension require further investigation.     

Obstructed pulmonary venous drainage with total anomalous pulmonary venous connection to the coronary sinus

Obstructed pulmonary venous drainage is generally considered extremely rare with total anomalous pulmonary venous connection (TAPVC) to the coronary sinus (CS). A retrospective review of 27 operated patients with TAPVC to CS revealed 6 cases of obstruction (22%). Two of 6 patients who died early had evidence of obstruction at the pulmonary vein confluence at autopsy. Among the 21 hospital survivors, obstruction proximal to the point of CS unroofing developed in 4 (19%), necessitating reoperation and resulting in death in 3. One other patient died late. Mean follow-up of the 17 long-term survivors, who are generally doing well, is 85 months (range 2 to 212). There have been no cases of late obstruction at the site of unroofing among 10 patients who underwent the fenestration procedure suggested by Van Praagh. The incidence of early bradyarrhythmias (60%) does not appear to be decreased by this procedure. Review of 13 autopsy specimens suggests that if the right and left pulmonary veins did not drain directly to the CS but converged to form a short common vertical vein (4 cases), obstruction was likely. When pulmonary artery pressure approaches systemic levels preoperatively, careful echocardiographic and intraoperative assessment of the junction of the pulmonary vein confluence with the CS should be made. If there is evidence of obstruction, consideration should be given to anastomosing the horizontal right and left pulmonary veins directly to the left atrium rather than performing a simple unroofing procedure.     

Heart malformations in trisomy 13 and trisomy 18]

Congenital heart diseases were studied in children diagnosed of trisomy 13 and trisomy 18 in our hospital between January 1973 and July 1990. Twenty patients with trisomy 18 were diagnosed (18 females and two males). All had cardiac malformations. The findings were: ventricular septal defect in 16 cases (80%), valvular anomalies in 12 (63%), patent ductus arteriosus in nine (47%) and atrial septal defect or patent foramen ovale in 7 cases (36%). We found some complex congenital cardiac diseases: one atrioventricular canal, one tetralogy of Fallot, one hypoplastic left ventricle with mitral atresia and double outlet right ventricle, one case of univentricular heart with aortic outlet from a rudimentary cavity, a right ventricular atresia with pulmonary and tricuspid valves atresia. Nine cases of trisomy 13 were diagnosed (seven females and two males). We found: ventricular septal defect in 7 cases (77%), valvular disease in five (100% of the necropsy studies), secundum atrial septal defect in 4 patients (80%) and patent ductus arteriosus in two. Two cases presented hypoplastic left ventricle with aortic arch hypoplasia, one of them had subaortic stenosis and left superior vena cava being connected to the right atrium via coronary sinus; one case showed fibroelastosis. Our results have been similar to the previously reported and confirm the invariably presence of cardiac malformations in these syndromes. These malformations are an important sign of suspicion in fetal ultrasonography.     

Non-invasive diagnosis in clinically suspected atrial septal defect of secundum or sinus venosus type. Value of combining chest x-ray,phonocardiography, and M-mode echocardiography.

Twenty-three consecutive patients with clinical (auscultatory and electrocardiographic) signs of uncomplicated atrial septal defect of secundum or sinus venosus type were examined by chest x-ray, phonocardiography, and echocardiography, before right heart catheterisation. Seventeen (74%) had atrial septal defect, two patients (9%) had insignificant pulmonary stenosis, and four subjects (17%) were normal. No false positive diagnosis of atrial septal defect was made by chest x-ray examination, whereas increased vascular markings were incorrectly interpreted as pulmonary congestion in one case. Four patients had x-ray films showing questionable signs of left-to-right shunt. Six of 15 patients with a large left-to-right shunt were correctly selected for surgery based on radiological findings. One false negative but no false positive diagnosis of atrial septal defect was made by phonocardiography. Four cases with and four cases without atrial septal defect were classified as having questionable phonocardiographic signs of atrial septal defect. Echocardiographic distinction between those with atrial septal defect and those without atrial septal defect was correct in all cases; quantitative measurement of left-to-right shunt, however, was unsatisfactory. Combined normal findings by x-ray film and echocardiography appeared adequate in all cases for the exclusion of atrial septal defect (six patients). When the six patients who were correctly identified for surgery from the radiological findings are included, there was a total of 12 patients out of 23 (52%:95% confidence limits 31 to 73%) who were evaluated definitively by the non-invasive tests.     

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。     

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.     

房水平单向活瓣式补片在先天性心脏病并发重度肺动脉高压术中的应用

目的:探讨并发重度肺动脉高压的先天性心脏病患者的外科手术方式。方法: 采用房水平单向活瓣补片对48例本病患者进行手术治疗。其中,室间隔缺损30例,房间隔缺损3例,室间隔缺损并发房间隔缺损8例,室间隔缺损伴动脉导管未闭7例。结果: 术后1例（2%）死于低心排综合征。术后早期均有明显低氧血症。术后1周仍出现跨单向活瓣补片分流40例,术后3个月活瓣仍开放10例,术后26个月提示活瓣均关闭,分流消失,经皮血氧均为95%以上,肺动脉压均明显下降。结论: 房水平单向活瓣式补片治疗先天性心脏病并发肺动脉高压,使患者度过术后危险期,提高手术成功率。     

四种超声心动图技术评价正常人心肌生物力学参数及其与心功能相关关系

目的: 比较直接二维测量法(D2D)、解剖M型(AMM)、组织速度成像技术(TVI)及二维斑点追踪技术(STI)评价正常人心肌生物力学参数及其与心功能相关关系的方法,比较他们在临床运用过程优点及存在问题。方法: 分别应用D2D、AMM、TVI及STI测量并比较40例正常人收缩、舒张期心肌在长轴方向、短轴和圆周方向的心脏生物力学参数。结果: D2D显示:正常人收缩期纵向应变(LS)及应变率(LSR):下壁>后间隔>前间隔>后壁>前壁>侧壁（P>005）;收缩期LS、LSR与左室射血分数（LVEF）相关系数(r)分别为-0523及-0550。AMM显示:正常人收缩末期径向应变(RS)及径向应变率(RSR)游离壁大于间隔:收缩期RS、RSR与左LVEF(r)分别为0533及0495。TVI显示:收缩期LS:心尖段<基底段、中间段(P<005);舒张早期LS中间段>基底段>心尖段;舒张晚期LS心尖段<基底段、中间段(P<005);收缩期LS、LSR与LVEF(r)分别为-0562及-0550。STI显示:收缩期LS从基底段到心尖段逐步递增;左室扭转运动表现为心底部顺时针旋转和心尖部逆时针旋转,心脏整体表现为心动周期内逆时针方向为主的扭转运动。心肌收缩期平均LS及LSR与左室收缩功能参数间r的绝对值均超过055。结论: D2D通过直接测量左室长轴方向心肌长度的变化,间接计算LS与LSR;AMM克服了M型超声测量的角度限制,比较适合体位不合作的患者;TVI适合评价长轴方向室壁运动特征;STI对二维图像的超声斑点回声进行逐帧追踪,可以更准确测量局部室壁V、S等参数,定量评价局部心肌的收缩和舒张功能,测量参数与传统超声测量LVEF相关性较好,但设备要求较高。