杂交技术在法洛四联症中的应用

目的:总结杂交技术镶嵌治疗儿童法洛四联症(TOF)的经验。方法:外科术前介入治疗:3例有巨大体肺侧支(APCAs)的重症TOF在根治术前予以侧支血管堵塞术。外科术后镶嵌治疗:6例TOF根治术后残余分流,其中4例残余膜周部室间隔缺损,1例残余左室右房通道室间隔缺损,1例残余房间隔缺损,分别予以经导管残余心脏缺损封堵术。结果:3例有巨大APCAs的重症TOF在根治术前予以侧支血管堵塞术后随即进行外科手术,皆获得满意效果。6例TOF根治术后残余分流者行经导管封堵术封堵成功,随访无残余分流及心脏瓣膜异常,未出现心律失常。结论:杂交技术镶嵌治疗伴有巨大APCAs及术后存在残余分流的TOF安全、有效。     

重症法洛四联症的镶嵌手术治疗

目的 总结重症法洛四联症(TOF)镶嵌手术的临床经验,探讨其方法、效果和意义.方法 筛选病例:重症发绀型法洛四联症17例,通过超声心动图和螺旋CT检查确诊.术前介入治疗:重症TOF在根治术前予以侧支血管堵塞术,其中2例采用AGA Plug血管塞堵闭体肺侧支血管,1例采用国产先健动脉导管未闭(PDA)Amplatzer封堵器,其余均采用COOK非可控弹簧圈栓塞体肺侧支血管.侧支血管堵塞术后立即行一期外科根治手术.结果 全组手术皆获得满意效果.随访2.0～2.5年,无严重并发症,患儿发育正常.结论 镶嵌手术治疗重症TOF安全、有效、可行,比单一外科手术效果好.     

杂交技术在法洛四联症合并体肺侧支治疗中的应用

目的评价杂交技术治疗法洛四联症合并体肺侧支的临床效果。方法回顾分析2010年1月至2011年12月期间我院31例合并体肺侧支的法洛四联症病例,均采用侧支栓堵和外科手术联合治疗。结果全组早期死亡1例,死亡率为3．2％。共发现大的体肺侧支66支,栓堵53支,未发生造影及栓堵相关并发症,均痊愈出院。结论介入栓堵体肺侧支与外科手术联合治疗伴有体肺侧支的法洛四联症是安全有效的,可以减轻手术难度,减少术中出血,缩短手术时间,提高手术矫治成功率,减少手术刨伤,可以明显降低术后并发症,提高手术治愈率。     

KMG微球栓塞联合外科矫正术治疗伴丰富侧支血管的法洛四联症

9例重症法洛四联症患者,术时造影发现有体肺侧支血管25支,术中先对其中的20支进行了海藻酸钠（KMG）微球栓塞,部分适于手术结扎的血管在实施外科矫正术同时进行结扎,之后进行心内畸形外科矫正术。术中体外循环灌注压稳定,侧支血管回血量明显减少,术野暴露良好。术后8例痊愈,1例因凝血功能异常于术后第6天失血、心功能衰竭死亡。认为对伴有丰富侧支血管形成的重症法洛四联症患者,在外科矫正术前、术中用KMG微球栓塞体肺侧支血管,可减少术中出血,有利于术野暴露,手术成功率高。     

体肺侧支血管封堵治疗在肺血减少型先天性心脏病中的临床应用研究

目的:探讨肺血减少型复杂先天性心脏病体肺侧支介入封堵治疗的意义、操作技术及安全性。方法:回顾性分析58例并发体肺侧支的肺血减少型先天性心脏病的封堵介入治疗。中位年龄36个月(7~288个月),体质量6.9~64kg,平均15.96kg。本组患儿术前均经超声心动图及心脏CT检查确诊,54例术前封堵侧支血管,4例术后封堵侧支血管。结果:全组58例患者体肺侧支血管共115支,其中63支(54.7%)起源于降主动脉(胸主动脉)。对94支血管进行了封堵(82%),释放弹簧圈204枚。每位患者封堵侧支血管1~6支,放置弹簧圈1~14枚。本组10例因肺动脉未达到根治条件未手术,48例行外科根治手术,死亡3例。死亡原因:1例肝、肾衰竭,1例低心排、灌注肺,1例重症感染、多器官衰竭。结论:本组病例提示对肺血减少型复杂先心合并体肺侧支血管,先期采用经皮介入封堵侧支,再行外科治疗,安全、有效,可减少术后并发症的发生,降低手术病死率。     

Hybrid技术治疗合并体肺侧支的法洛四联症

目的:总结应用Hybrid技术治疗合并较大体肺侧支的法洛四联症的临床结果,分析该联合术式的临床疗效。方法:回顾分析阜外医院1996年1月至2008年12月手术联合栓堵治疗的46例伴有体肺侧支的法洛四联症的临床资料:2007年6月前主要采取手术前、后联合介入栓堵13例(分期组),2007年6月后采用Hybrid技术行术中栓堵33例(同期组)。全组造影发现体肺侧支共89支,成功栓堵70支。结果:同期手术组在体外循环时间、升主动脉阻断时间、呼吸机使用时间、ICU时间及住院时间方面均明显小于分期手术组。同期手术组病死率(6.1%)较分期手术组(31%)低。结论:应用Hy-brid技术治疗合并较大体肺侧枝的法洛氏四联症可降低手术难度,减轻手术创伤,提高手术矫治成功率;术中联合栓堵安全有效,优于术前或术后联合栓堵。     

体-肺侧支栓塞术在重症法洛四联症“一站式”杂交治疗中的临床价值

目的评价体-肺侧支栓塞术在重症法洛四联症（F4）患者“一站式”杂交治疗（介入治疗＋外科根治术）中的临床价值。方法2007年5月至12月,共26例重症F4患者（男12例,女14例）,平均年龄5.2岁（5个月~29岁）,在我院Siemens杂交手术室系统内完成了“一站式”治疗：全麻插管下先完成主动脉及心室造影,若发现有明确的体-肺侧支血管即予以经导管栓塞,随后立即完成外科根治术（杂交组）。选择同期住院仅接受单纯外科根治术的26例重症F4患者作为对照组（单纯外科组）。两组患者在性别、年龄、体重组成方面差异无统计学意义。对两组患者的住院时间、术后ICU监护时间、呼吸机辅助时间、体外循环时间等指标进行对比研究。结果杂交组中有20例患者接受了体-肺侧支栓塞术,其中2例因侧支血管太细放弃栓塞、4例未发现明确体-肺侧支血管。共采用Cook不可控弹簧圈50枚栓塞了38支侧支血管,另有两例采用明胶海绵团块栓塞侧支血管,所有患者均获栓塞成功,无异位栓塞发生。杂交组术后恢复良好,无死亡病例。单纯外科组有两例患者外科术后发生“灌注肺”现象,术后2~5d内接受了补救性体-肺侧支栓塞术,1例死于术后心功能不全。杂交组较对照组总住院时间（19.5d比25.6d）、术后ICU监护时间（50.3h比82.6h）、术后呼吸机辅助时间（18.5h比38.6h）和术中体外循环时间（105.1min比131.3min）均要明显缩短（均为P〈0.05）。结论外科术前体-肺侧支栓塞术能缩短手术时间、提供良好手术野、减少术后灌注肺的发生,在F4患者“一站式”杂交治疗能提高外科根治术的疗效及预后。     

复杂紫绀型先天性心脏病并发体肺侧支的内外科镶嵌治疗

目的:总结并发体肺侧支的复杂紫绀型先天性心脏病内外科镶嵌治疗的临床经验,探讨其治疗意义、操作技术及安全性。方法: 回顾性分析41例并发体肺侧支的复杂紫绀型先心病患者,年龄1～28（15±10）岁,体质量（18±8）kg。均行螺旋CT检查确诊,38例术前封堵侧支血管,3例术前漏诊术后封堵侧支血管。全组均行外科一期矫治手术。结果: 全组治愈37例。死亡4例:1例死于肺部感染,3例死于顽固性心力衰竭。侧支血管直径2.5～9.4（5.3±2.1） mm。每位患者放置弹簧圈3～21（10±6）枚。3例术后因侧支血管再通而二次封堵。术后4例并发肺部感染,3例肺水肿,2例灌注肺。结论: 并发体肺侧支的复杂紫绀型先心病,在围手术期应当内外科联合处理侧支血管的问题。可采用经皮介入封堵法,有效、简单、安全,可行多支、多次封堵,有效减少并发症的发生,降低手术死亡率。     

伴有体肺侧支血管的发绀型先天性心脏病患者镶嵌治疗的临床探讨

目的 探讨伴有体肺侧支血管的发绀型先天性心脏病患者镶嵌治疗的可行性和效果.方法 回顾性分析7例伴有体肺侧支血管的发绀型先天性心脏病镶嵌治疗患者的临床资料.全组患者于手术当天在导管室行主动脉造影和选择性体肺侧支血管造影,根据情况选择合适的弹簧圈对体肺侧支血管进行栓塞,随后立即移至外科手术室行外科矫治手术.结果 7例患者共发现体肺侧支血管15支,除其中的1支使用ev3公司可控性弹簧圈2枚栓塞外,其余均使用COOK公司非可控弹簧圈共20枚达到完全栓塞,顺利完成外科矫治手术.未出现介入治疗并发症,未发生体肺侧支栓塞后严重低氧血症,无矫治术后脱离体外循环机困难,外科根治术后未出现灌注肺、低心排血量综合征等严重并发症.术后无需行补救性体肺侧支栓塞.结论 伴有体肺侧支血管的发绀型先天性心脏病患者镶嵌治疗是可行、有效的.     

第Ⅱ代动脉导管未闭封堵器栓塞幼儿体肺侧支的临床应用

目的探讨Amplatzer第Ⅱ代动脉导管未闭封堵器(amplatzer duct occluderⅡ,ADOⅡ)栓塞幼儿体肺侧支的可行性及效果。方法入选2014年3月3日至2015年4月1日7例患儿(其中男6例,女1例)选用ADOⅡ栓塞体肺侧支,栓塞后行选择性体肺侧支造影,观察封堵器的位置、形态及封堵效果。结果 7例介入栓塞患儿,年龄5~71个月,体质量4.2~22.0 kg。有6例患儿栓塞成功,1例栓塞后出现严重低氧血症放弃介入治疗,择期行外科体肺侧支结扎术。介入栓塞15支体肺侧支,共用ADOⅡ7枚、COOK公司不可控弹簧圈22枚和波科公司可控微弹簧圈2枚。即刻造影示无残余分流2例,少量残余分流4例,少-中量残余分流1例。6例介入成功患儿立即行外科术,其中5例行肺动脉闭锁根治术,1例行体肺分流术。外科术中回血不多、未见死亡,随访未见溶血及外周血管损伤等并发症发生。结论 ADOⅡ可控性高、输送鞘小、对血管损伤小,应用于幼儿合并较粗大、迂曲体肺侧支的介入治疗是安全可行的。     

并发主肺侧支的Fallot四联症术后通气功能的改变

目的:研究侧支循环对Fallot四联症患者根治术后心肺功能的影响。方法:我们研究了23例Fallot四联症并发主肺侧支的患者(组A),30例没有侧支的Fallot四联症患者(组B),37例行室间隔缺损或房间隔缺损修补的患者(组C)。各组患者于术前和术后一年进行肺功能测试和运动心肺功能,分析肺活量、最大摄氧量、氧脉搏、死腔/潮气量比等。结果:肺功能结果显示各组间第1秒用力呼气容积(FEV1)、第1秒用力呼气容积占预计值百分比(FEV1%pred)、分钟通气量、分钟通气量占预计值百分比无显著性差异,在组A和组B用力肺活量、用力肺活量占预计值百分比与组C相比差异有显著性(P0.01),组A的FEV1/FVC%显著高于组B和组C(P0.01)。运动心肺试验显示组A的最大运动功率、峰值运动时的分钟通气量、呼吸商、最大摄氧量、无氧阈、最大氧脉搏显著低于组B和组C(P0.01),而CO2呼吸当量明显高于组B和组C(P0.01)。结论:组A与组B、组C相比,表现出更显著的限制性通气功能损害,因此术前需积极处理主肺侧支。     

Tetralogy of Fallot with major aortopulmonary collateral arteries.

The clinical features of eight patients with tetralogy of Fallot and major aortopulmonary collateral arteries were determined and found to be usually unhelpful in differentiating such patients from those with pulmonary atresia with ventricular septal defect. The point of connection between the pulmonary and collateral arteries could usually be demonstrated at cineangiography by observing washin and washout between the two systems. The central pulmonary arteries connected with the entire pulmonary vascular bed in the five patients in whom the anatomy of the pulmonary circulation was clearly demonstrated and were thought to do so in the remaining three patients also. In six out of eight patients only one collateral artery connected with each hilar pulmonary artery. These findings suggest that the anatomy of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries is usually less complex than in pulmonary atresia with ventricular septal defect, making the outlook for complete repair more hopeful. The major determinant of successful complete repair appears to be pulmonary arterial size. Early palliative surgery to increase pulmonary blood flow is recommended to encourage normal growth of the central and intrapulmonary arteries.     

Tetralogy of Fallot with major aortopulmonary collateral arteries

The clinical features of eight patients with tetralogy of Fallot and major aortopulmonary collateral arteries were determined and found to be usually unhelpful in differentiating such patients from those with pulmonary atresia with ventricular septal defect. The point of connection between the pulmonary and collateral arteries could usually be demonstrated at cineangiography by observing washin and washout between the two systems. The central pulmonary arteries connected with the entire pulmonary vascular bed in the five patients in whom the anatomy of the pulmonary circulation was clearly demonstrated and were thought to do so in the remaining three patients also. In six out of eight patients only one collateral artery connected with each hilar pulmonary artery. These findings suggest that the anatomy of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries is usually less complex than in pulmonary atresia with ventricular septal defect, making the outlook for complete repair more hopeful. The major determinant of successful complete repair appears to be pulmonary arterial size. Early palliative surgery to increase pulmonary blood flow is recommended to encourage normal growth of the central and intrapulmonary arteries.     

Tetralogy of fallot with diminutive pulmonary arteries: Preoperative pulmonary valve dilation and transcatheter rehabilitation of pulmonary arteries

Objectives. The study sought to determine the results of a novel transcatheter management approach in tetralogy of Fallot with diminutive pulmonary arteries.

Background. Tetralogy of Fallot with diminutive pulmonary arteries and severe pulmonary stenosis is rare and resembles tetralogy of Fallot with pulmonary stresia: There is a high incidence of aortopulmonary collateral channels, arborization abnormalities, stenoses and need for multiple operations. Because a combined catheter-surgery approach facilitates repair in these patients, such an approach may benefit those with diminative pulmonary arteries and pulmonary stenosis.

Methods. Clinical, catheterization and surgical data were studied retrospectively for 10 such patients undergoing preoperative pulmonary valve balloon dilation, among other transcatheter interventions, from January 1989 to January 1995.

Results. Initially, the Nakata index ranged from 20 to 98 mm²/m² (mean 67 ± 28 mm²/m²). The pulmonary valve was first balloon dilated (mean balloon/annulus 1.5 ± 0.3), and the mean initial valve annulus Z score (−40 ± 1) increased to −3.3 ± 1.1 (p < 0.01). Other interventions included branch pulmonary artery balloon dilation (7 patients, 23 vessels) and coil embolization of aortopulmonary collateral channels (8 patients, 31 collateral channels). At preoperative follow-up catheterization, the mean pulmonary annulus Z score was −3.1 ± 0.7, and the aorta index increased to 143 ± 84 mm²/m² (p < 0.03). All patients underwent complete surgical repair successfully. At a mean follow-up period of 2.6 ± 2 years, right ventricular pressure was < 70% systemic in all patients and <50% systemic in seven.

Conclusions. In patients with tetralogy of Fallot, severe pulmonary stenosis and diminutive pulmonary arteries, initial pulmonary valve balloon dilation increases the annulus Z score and anterograde pulmonary blood flow and facilitates simultaneous coiling of sertopulmonary collateral channels and access for branch pulmonary artery dilation, all of which results in pulmonary artery growth, simpligying surgical management.     

先天性心脏病介入治疗在外科术后的应用

目的:评价肺血减少性先天性心脏病(先心病)外科术后,补充性介入治疗的价值。方法:对我科2010年5月至2011年6月住院的15例患者,肺血减少性先心病外科根治或减状手术后的补充性介入治疗进行总结分析。男性10例,女性5例,年龄6个月～14岁,平均3.7岁。病种及外科手术方式包括法洛四联症根治术或减状术5例,右心室双出口Glenn术3例,肺动脉闭锁根治术3例,Glenn术1例,单心室Glenn术2例及共同动脉干根治术1例。介入治疗情况:1.体肺侧枝血管封堵术10例,其中因肺出血而急诊封堵术3例,体肺侧枝血管肺静脉瘘1例。2.因Glenn术后奇静脉扩张窃血、血氧饱和度下降行奇静脉封堵1例。3.外科术后肺动脉狭窄行狭窄部位球囊扩张术4例。结果:介入治疗达到预期效果,未发生与操作相关的并发症。结论:介入治疗可作为一些复杂先心病外科术后,有效的补充性治疗手段。     

发绀型先天性心脏病伴体肺动脉侧支循环的内外科联合治疗

目的 评价内、外科联合治疗对发绀型先天性心脏病伴体肺动脉侧支循环的临床效果.方法 回顾性分析27例经内、外科联合治疗的发绀型先天性心脏病伴体肺动脉侧支循环患者的临床资料,着重分析侧支血管栓堵方法 、外科手术方法 、治疗结果 及并发症.结果 24例存活,3例死亡,病死率11.1%(3/27),死因分别为心室颤动1例、低心排血量综合征1例、呼吸衰竭1例.66支侧支血管为完全阻断,11支血管为部分阻断.栓堵相关并发症:2例缺氧发作:3支侧支血管渗漏,放置弹簧圈后侧支循环均达完全阻断.结论 内、外科联合治疗伴有体肺动脉侧支循环的发绀型先天性心脏病是行之有效的方法.     

分流术结合介入治疗分期根治肺动脉发育不良型重症法洛四联症

目的:总结体肺分流手术结合内科介入,治疗不适于一期根治的肺动脉发育不良型重症法洛四联症(TOF)的临床经验。方法:收集分析2010年3月至2013年8月,我中心行体肺分流术结合介入并最终完成根治手术的14例(男性10例、女性4例)肺动脉发育不良型重症TOF患儿资料。行分流术时,年龄3~34个月(中位年龄6个月),体质量5~16kg,平均(9.2±3.35)kg;行球囊扩张术时年龄18~48个月,(中位年龄27个月),行封堵术及根治术时,年龄12~72个月(中位年龄25个月,30个月),体质量9~24kg,平均(13.88±4.77)kg。合并心血管畸形包括:房间隔缺损3例,永存左上腔静脉2例,单冠畸形1例,合并粗大体肺侧枝9例。14例均行姑息性中心分流式体肺分流术,其中2例同期行侧枝融合术(3支)。姑息性外科术后8例行内科介入封堵体肺侧枝(19支),8例行内科介入肺动脉瓣球囊扩张术,2例同期行内科介入封堵及肺动脉瓣球囊扩张术。结果:全组无死亡病例及严重并发症。14例患儿根治手术前Nakata指数(mm3/mm2)和McGoon比值分别147.2~214.0,平均(173.2±24.53)和1.49~2.24,平均(1.78±0.26)相比体肺分流术前79.9~103.6,平均(93.03±7.58)和0.93~1.18,平均(1.00±0.01)明显升高(P0.05)。根治术前末梢血氧饱和度75%~98%,平均(85.36±6.12)%较体肺分流术前60%~85%,平均(72.86±6.32)%显著改善。血红蛋白浓度由体肺分流术前112~263g/L,平均(171.93±38.93)g/L下降至根治术前110~175g/L,平均(132.93±17.31)g/L。8例体肺分流术结合内科介入封堵体肺侧枝患儿根治术中,术后未见体肺侧枝相关并发症。8例行体肺分流术结合内科介入肺动脉瓣球囊扩张术患儿根治手术前Nakata指数(mm3/mm2)和McGoon比值150.5~210.3,平均(181.1±26.22)和1.59~2.24,平均(1.83±0.25)较肺动脉瓣球囊扩张术前94.4~146.7,平均(120.18±17.3)和1.21~1.4,平均(1.32±0.06)明显增加,差异具有统计学意义(P0.05)。全部14例患者均最终成功实施根治手术,术后随访结果良好。结论:采用体肺分流术结合内科介入技术的分步治疗方法能有效改善肺动脉发育程度、提高根治手术成功率、降低病死率及术后并发症发生率。     

Relation of genotype 22q11 deletion to phenotype of pulmonary vessels in tetralogy of Fallot and pulmonary atresia-ventricular septal defect

Objective—To compare the morphology of the pulmonary vessels in tetralogy of Fallot or pulmonary atresia-ventricular septal defect (PA-VSD) with (del22q) and without 22q11 deletion (non-del22q).
Patients—94 consecutive infants (54 with tetralogy of Fallot, 40 with PA-VSD) were studied using ultrasound and catheterisation.
Molecular investigations—Identification of the 22q deletion was performed either by fluorescent in situ hybridisation or polymerisation chain reaction genotyping.
Results—25 patients were del22q (16/40 (40%) PA-VSD v 9/54 (17%) tetralogy of Fallot; p < 0.02). Major aortopulmonary collateral arteries was more common in patients with PA-VSD-del22q (p < 0.03). Such collaterals were identified in 13 patients: 10 del22q and three non-del22q (p < 0.001). The size of the right and left pulmonary arteries expressed as a standard deviation (SD) difference of the normal range was −4.2 (quartiles −5.3 and −2.9) for PA-VSD del22q, and −2.6 (−3.1 and −1.8) for PA-VSD non-del22q (p = 0.02). The mean (SD) difference between the measured and theoretical Nakata index was −373 (94) for PA-VSD del22q v −245 (93) in PA-VSD non-del22q (p = 0.0002). In tetralogy of Fallot patients with and without del22q, the size of the pulmonary arteries was similar (p = 0.6).
Conclusions—A "specific" phenotype could be defined in patients with deletion: PA-VSD, major aortopulmonary collateral arteries with complex loop morphology, and small central pulmonary arteries. Differences in the morphology of the pulmonary vessels may indicate a different timing of the faulty developmental pathway in patients with and without 22q11 deletion.

Keywords: 22q deletion;  tetralogy of Fallot;  congenital heart disease     

Pulmonary atresia with ventricular septal defect: the significance of collateral lung perfusion for the prognosis of corrective operations

From 1970 to 1986, 43 patients with pulmonary atresia and ventricular septal defect (PA-VSD) were diagnosed in our hospital. 19 children had a collateral lung perfusion via a ductus botalli (DB), three children had a DB and systemico-pulmonary collateral arteries (SPCA), and 21 children had only SPCA. Patients with a DB alone had significantly larger pulmonary arteries. Children with SPCA always had hypoplastic pulmonary arteries and intrapulmonary arborization anomalies and in a high percentage (19 patients) they showed intrapulmonary stenoses. 26 palliative operations were performed in 24 children. After palliative operations in 15 cases (patients with DB: RVOT-Patch: one patient, aortopulmonary shunt: three patients, Brock procedure: one patient, patients with SPCA: RVOT-Patch: five patients, aortopulmonary shunt: four patients, Brock procedure: one patient) we checked the possibility of corrective surgery with the equation of Alfieri. According to this equation, corrective surgery without excessive right ventricular pressure would have been possible in all post-operatively catheterized patients with DB after primary palliative operations but, due to the pulmonary artery anomalies, only in seven out of nine patients with SPCA (one patient of the latter group was operated twice, Brock procedure and RVOT-Patch). In four cases we could prove the value of the Alfieri equation after corrective operations. There was an excellent correlation between the predicted relation of PRV/PLV and the actually measured pressures. In our opinion, the Alfieri equation forms a valuable aid in the preoperative assessment of children with PA-VSD.     

Tetralogy of fallot and absence of left pulmonary artery

Unilateral absence of the pulmonary artery is a rare congenital lesion usually caused by backward displacement of the conical artery of the truncus arteriosus. The purpose of this report is to describe and discuss the treatment of an 8-year-old patient who presented with cyanosis and was diagnosed with tetralogy of Fallot together with an absence of the left pulmonary artery and major aortopulmonary collateral arteries.