经皮介入栓堵双向Glenn术后扩张的奇静脉

目的:总结经皮介入栓堵双向Glenn术后扩张奇静脉的结果。方法:8例复杂性先天性心脏病患儿,双向Glenn术后发现奇静脉和/或半奇静脉扩张,平均年龄(7.7±3.3)岁,平均体质量(24±6)kg,距双向Glenn手术1.0～4.7年,平均2.7年。入院时氧饱和度(SO2)平均(0.80±0.08)。心血管造影显示扩张的奇静脉和/或半奇静脉,平均内径(8±2)mm,与上腔静脉内径比值平均0.66±0.14。肺动脉的Mcgoon比值平均2.3±0.3,Nakata指数平均(248±75)mm2/m2,平均肺动脉压(PAP)和上腔静脉压(SCVP)均为(13.4±3.3)mmHg(1 mmHg=0.133 kPa)。1例合并左肺动脉(LPA)起始部中度狭窄。于基础麻醉下经皮导管栓堵奇和/或半奇静脉,1例同时行LPA狭窄部位球囊扩张。结果:栓堵术后SO2升高至平均〔(0.86±0.04),P<0.0001〕,PAP〔(12.9±3.4)mmHg,P=0.53〕和SVCP〔(12.7±3.2)mmHg,P=0.09〕无变化。4例于栓堵术后1 w至1年行全腔静脉-肺动脉连接术(TCPC)。4例随诊1～2年SO2无变化。结论:双向Glenn术后合并奇静脉和/或半奇静脉扩张、SO2降低,但无SCVP或PAP升高者,可经皮介入栓堵奇静脉和/或半奇静脉,以增加肺血流量,提高体循环SO2。     

法洛四联症外科矫治手术前后的介入干预治疗

目的:评价经导管介入治疗措施对法洛四联症(tetralogy of Fallot,TOF)外科矫治术的辅助治疗价值。方法:对我科2010年10月至2013年12月住院的27例,和作者在外院完成的3例TOF经导管介入治疗进行总结分析。结果:男性20例,女性10例,年龄8个月~49岁,平均4.3岁。外科矫治术前行体-肺侧枝血管(major aorto-pulmonary collateral arteries,MAPCAs)栓塞术17例、肺动脉瓣球囊扩张术(percutaneous balloon pulmonary valvuloplasty PBPV)4例;因术后肺出血急诊行MAPCAs栓塞术4例,术后左心衰行MAPCA-肺静脉瘘瘘口封堵1例、肺动脉分支狭窄球囊扩张(percutaneous balloon pulmonary arterioplasty PBPA)3例、PBPV1例。介入治疗均达到预期效果,未发生与操作相关的并发症,1例根治术后,肺出血患者行MAPCAs栓堵术后肺出血改善,但死于感染性心内膜炎,其他病例均痊愈出院。结论:TOF外科矫治术前、术后的介入治疗,可减少TOF根治手术风险并改善预后。     

超声评价肺血减少型复杂先天性心脏病双向Glenn术后肺血管发育的临床价值

目的:探讨超声心动图评价肺血减少型复杂先天性心脏病(先心病)双向Glenn分流术后肺血管发育的临床应用价值。方法:应用二维经胸超声心动图测量101例复杂发绀型先心病患儿双向Glenn分流术前后左、右肺动脉内径,同时测量术前、术后经皮血氧饱和度,评价其术后肺血管发育。根据有无肺动脉前向血流将患儿分为肺动脉闭锁组(30例)和肺动脉狭窄组(71例);另分为婴幼儿组(年龄≤3岁,51例)和儿童组(年龄>3岁,50例),比较各组内及组间手术前后肺动脉分支内径及血氧饱和度变化。结果 :术后应用超声心动图随访12～36个月,左、右肺动脉内径及血氧饱和度较术前有不同程度改善。肺动脉狭窄组较肺动脉闭锁组术后肺动脉生长发育及血氧饱和度的改善更明显;婴幼儿组肺动脉发育及血氧饱和度改善情况优于儿童组(P<0.01)。应用超声定量的肺动脉分支发育与临床血氧相关性回归分析结果满意(P<0.001)。结论:肺血减少型复杂先心病患儿施行双向Glenn分流术后,可在不增加右心室前负荷情况下增加肺血流,促进患者肺动脉血管发育,提高血氧饱和度。超声心动图对术后定量随访评估肺血管发育,评估预后有重要的临床应用价值。     

肺血减少型复杂先天性心脏病姑息术后介入治疗的临床效果研究

目的:探讨肺血减少型复杂先天性心脏病姑息术后,应用经皮球囊扩张术治疗的临床效果。方法:收集自2012年7月至2015年2月间,肺血减少型复杂先心病姑息手术(体-肺动脉分流或双向Glenn术)后1年肺动脉发育仍未达根治条件的病例共23例,男性14例、女性9例,年龄1.3~13.0岁(中位年龄3.1岁),体质量8.0~48.0kg,平均(17.9±7.7)kg,身高72.0~173.0cm,平均(97.8±23.6)cm,经皮血氧饱和度(Sa O2)74%~89%,平均(80.0±7.0)%。术前常规行超声心动图、心电图、胸部X线、心导管和造影检查,所有病例行经皮球囊肺动脉瓣或肺动脉扩张,于术后1个月、3个月、6个月及1年进行随访。评价经皮球囊肺动脉扩张术治疗肺血减少型复杂先心病姑息术后肺动脉发育仍未达根治条件的患儿的临床效果。结果:所有患儿均成功行介入治疗,无严重并非症。23例患儿术后即刻跨肺动脉瓣压力阶差(PTG)、右心室收缩压(RVSP)较术前明显减少,Sa O2较术前明显升高,差异有统计学意义(P0.05)。在随访过程中经超声心动图或CT检查发现患儿肺动脉瓣、主肺动脉、Nakata指数、Mc Goon比值、PTG等均较术前有明显改善(P0.05)。12例患儿因肺动脉发育较好达到根治条件而最终行根治手术,恢复良好。9例行未跨环手术,3例行跨环手术。余患儿仍在随访中,病情平稳,发绀较前有所减轻,活动耐量较前有所增加。结论:经皮肺动脉瓣或肺动脉球囊扩张术可有效增加肺血减少型复杂先心病外科姑息术后患儿前向肺血流,促进其肺血管发育,改善心室功能,为进一步的根治治疗创造条件。     

经皮支架置入在先天性心脏病治疗中的应用

经皮导管介入治疗先天性心脏病（先心病）已广泛应用于临床。随医用材料和介入器材的改进和发展,介入治疗先心痛的适应证不断扩大,从早期的瓣膜球囊成形术和间隔缺损封堵术逐渐发展到支架置入术。当前,支架置入已经能够治疗各个年龄段先天性和获得性血管狭窄、闭塞,如右室漏斗部狭窄、主动脉缩窄、肺动脉和肺静脉狭窄、受限的动脉导管、各种狭窄的外科人工管道血运重建等,有效避免单纯球囊扩张术后弹性回缩所致的再狭窄,为其后外科矫治手术创造条件。     

经皮支架置人在先天性心脏病治疗中的应用

经皮导管介入治疗先天性心脏病(先心痛)已广泛应用于临床.随医用材料和介入器材的改进和发展,介入治疗先心病的适应证不断扩大,从早期的瓣膜球囊成形术和间隔缺损封堵术逐渐发展到支架置入术.当前,支架置入已经能够治疗各个年龄段先天性和获得性血管狭窄、闭塞,如右室漏斗部狭窄、主动脉缩窄、肺动脉和肺静脉狭窄、受限的动脉导管、各种狭窄的外科人工管道血运重建等,有效避免单纯球囊扩张术后弹性回缩所致的再狭窄,为其后外科矫治手术创造条件.     

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。     

经皮支架置入在先天性心脏病治疗中的应用

经皮导管介入治疗先天性心脏病（先心病）已广泛应用于临床。随医用材料和介入器材的改进和发展，介入治疗先心痛的适应证不断扩大，从早期的瓣膜球囊成形术和间隔缺损封堵术逐渐发展到支架置入术。当前，支架置入已经能够治疗各个年龄段先天性和获得性血管狭窄、闭塞，如右室漏斗部狭窄、主动脉缩窄、肺动脉和肺静脉狭窄、受限的动脉导管、各种狭窄的外科人工管道血运重建等，有效避免单纯球囊扩张术后弹性回缩所致的再狭窄，为其后外科矫治手术创造条件。     

介入及手术联合治疗伴有体肺侧枝的法鲁氏四联症和肺动脉闭锁

目的评价介入栓堵侧枝合并手术矫治伴有体肺侧枝的重症法鲁氏四联症和肺动脉闭锁的临床结果,总结临床应用经验。方法回顾1992年至2006年我院介入加外科手术治疗25例伴有体肺侧枝的重症发鲁氏四联症和肺动脉闭锁,其中法四12例,肺动脉闭锁13例,年龄6月～17岁,体重7～55公斤,男性16例,女性9例,造影发现体肺侧枝共65支,平均2.6/例。外科根治手术23例,2例肺动脉闭锁行姑息手术,术前栓堵20例,术后栓堵5例,共栓堵侧枝53支,完全栓堵率86.8%。结果全组病例死亡6例,总死亡率24%,肺动脉闭锁根治术11例,死亡4例,死亡率36.4%,肺动脉闭锁姑息手术2例,死亡1例,法四根治术12例,死亡1例,死亡率8.3%。介入栓堵后根治术共18例,死亡3例,均为肺动脉闭锁,死亡率13%,根治术后介入栓堵5例,死亡2例,死亡率40%,其中法四4例死亡1例,肺动脉闭锁1例死亡1例。死亡原因为充血性心衰1例,低心排1例,肺出血1例,广泛肺渗出1例,肺缺血坏死1例,下腔静脉梗阻致多器官功能衰竭1例。结论介入栓堵体肺侧枝与外科手术联合治疗伴有体肺侧枝的重症法鲁氏四联症和肺动脉闭锁安全有效,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,减少手术创伤。术前栓堵侧枝对于手术成功尤为重要,术前明确体肺侧枝与固有肺动脉交通情况,对于单独供血的大体肺侧枝不宜拴堵,应于术中进行融合重建。与国外同期治疗结果比较我们的总死亡率较高。     

Glenn手术治疗合并静脉畸形引流的复杂先天性心脏病

目的探索双向Glenn手术治疗合并静脉畸形引流的复杂先天性心脏病（先心病）的临床效果。方法 2010年3月—2013年12月我科共对29例合并静脉畸形引流的复杂先心病病例实施双向Glenn手术。20例合并体静脉回流异常,6例合并肺静脉畸形引流,3例同时合并体肺静脉畸形引流。结果全组病例均行单侧或双侧双向Glenn手术,并有3例结扎细小的左上腔静脉,1例左侧改良B-T分流术,3例肺静脉畸形引流矫治,4例房室瓣成形术,3例肺动脉环缩术。围术期全组1例（3.4%）因恶性心律失常死亡,2例开胸止血;2例心包积液,1例脑栓塞,1例切口裂开。随访17.4个月±10.9个月无死亡,血氧饱和度（84±6）%,2例行完全性腔静脉肺动脉连接术,1例因预激综合征行射频消融术,2例需降肺动脉压力药物治疗。结论合并体静脉和/或肺静脉畸形引流的复杂先心病病例行双向Glenn手术早中期效果满意,肺静脉畸形情况和是否梗阻将直接影响手术方式,联合多种术式可能是改善手术效果的重要方法。     

Interventional catheterization in surgically treated patients with congenital heart disease

Interventional catheterization is an alternative to surgery for some congenital heart defects. For other malformations, the surgeon and the interventionist will join in an effort to obtain an optimal result: the typical example is pulmonary atresia with VSD and aortopulmonary collaterals. In other cases, the cardiologist may be called upon to intervene with catheter techniques to correct sequelae or residual lesions after surgical correction, avoiding redo surgery. Most often, the task consists of opening stenoses by balloon dilatation and/or stenting the main targets being pulmonary artery branch stenoses, venous obstructions after Mustard procedure, and recoarctations. Whereas simple balloon dilatation of recoarctation often brings good results, stents are often needed to obtain optimal results in pulmonary branch stenoses. Stenting of pulmonary veins has been disappointing. Closing unwanted vessels and defects is another task for the interventional cardiologist after cardiac surgery. Here, the most frequent procedure is closing aortopulmonary collaterals in pulmonary atresia and VSD after corrective surgery. Advantages and limitations of these procedures are discussed.     

Amplatzer occlusion of accessory ventriculopulmonary connections

Background: Children with complex congenital heart disease often require staged palliation to regulate systemic and pulmonary blood flow. Accessory sources of pulmonary blood flow including aortopulmonary collaterals, aortopulmonary shunts, and ventriculopulmonary connections following Glenn or Fontan palliation can be associated with elevated central venous pressures and persistent pleural drainage. Occlusion of accessory ventriculopulmonary connections in this setting has traditionally been accomplished surgically. Objective: To review the efficacy of Amplatzer devices in transcatheter occlusion of accessory ventriculopulmonary connections in children with complex congenital heart disease. Methods: Patients were identified and their records retrospectively reviewed for indication, procedural details, and clinical efficacy and outcome. Results: Between December 2004 and March 2008, seven patients underwent occlusion of accessory ventriculopulmonary connections using an Amplatzer Septal Occluder (3), an Amplatzer Duct Occluder (3), or an Amplatzer Vascular Plug (1). Underlying single ventricle physiology was present in six of these patients. The site of occlusion was the right ventricle to pulmonary artery (Sano) conduit in two patients, the native main pulmonary artery in three patients, the pulmonary valve in one patient, and a left ventricle to pulmonary artery homograft in one patient with biventricular physiology. There were no complications associated with these procedures. Conclusions: Amplatzer occlusion devices provide a safe and effective means of eliminating accessory ventriculopulmonary connections in children who have undergone surgical palliation of congenital heart disease. © 2008 Wiley‐Liss, Inc.     

Total cavopulmonary connection with extraatrial tunnel

Between April 1997 and February 2000, total cavopulmonary connection with an extraatrial tunnel was used to treat 9 cases of complicated congenital heart disease: single ventricle (4), double-outlet right ventricle (3), mitral atresia (1), and tricuspid atresia (1). There was no mortality. One patient developed bacterial endocarditis and required reoperation after 52 days to replace the tunnel. At follow-up ranging from 11 months to 3 years, 3 patients were in New York Heart Association functional class I, and 6 were in class II. One patient with single ventricle had refractory supraventricular tachycardia after a modified Fontan operation 4 years earlier, which was cured by the total cavopulmonary connection procedure. The essential factors for a good outcome include appropriate surgical indication, avoidance of aortic crossclamping and cardiac arrest, and unobstructed anastomosis between the superior and inferior venae cavae and the pulmonary artery.     

Techniques and applications of transcatheter embolization procedures in pediatric cardiology

Transcatheter embolization of congenital or acquired superfluous vascular structure has become routine procedures performed by interventional pediatric cardiologists. Embolization procedure is often part of a collaborative effort with cardiac surgeons to palliate complex congenital heart defect, such as in embolizing aortopulmonary collateral arteries in patient with single ventricle physiology. In other cases, the procedure is the definitive treatment as in embolizing coronary artery fistula. Pediatric cardiologists performing embolization procedures should be familiar with available technologies as well as understand the underlying cardiac anatomy and pathophysiology. This article provides a comprehensive review of presently available embolization agents and technologies. Some of the technologies are used only by interventional radiologists but may be useful to pediatric cardiologists. Specific clinical applications in pediatric cardiology are also discussed with summary of current literature. With continue advancement in transcatheter technology and operator expertise, all unwanted vascular communication should be amenable to transcatheter embolization.     

介入技术和外科手术同期"复合"治疗先天性心脏病的临床研究

目的结合介入器械和外科手术,实施“复合（Hybrid）”技术对先天性心脏病实施治疗。方法2005年3-10月,20例先天性心脏病患者接受了术中Hybrid技术治疗。球囊扩张组包括3例室间隔完整型肺动脉闭锁的新生儿和4例婴儿重度肺动脉瓣狭窄。缺损封堵组13例,包括10例房间隔缺损和3例多发室间隔缺损。正中或右侧腋下小切口进胸,在超声引导下经右室流出道置入球囊扩张管扩张肺动脉瓣或经右心房置入封堵器。2例多发室间隔缺损于体外循环下经三尖瓣置入封堵器。合并的其他心脏病变同期常规外科矫正,术式包括部分性肺静脉异位引流矫正、动脉导管结扎、冠状动脉旁路移植术等。术后心脏超声随访。结果患者全部顺利出院。1例婴儿重度肺动脉瓣狭窄术后2个月接受常规右室流出道成形术,1例多发肌部室间隔缺损因无法置入封堵器而转为常规术式。其余患者均顺利实施Hybrid术式。随访期内未发现介入器材相关的并发症。结论“复合”Hybrid技术可以避免体外循环,减少手术创伤,对于提高先天性心脏病的疗效具有重要的意受．     

Embolisation procedures in congenital heart disease

Eight therapeutic embolisation procedures were performed by the transcutaneous catheter technique in seven patients with congenital heart disease. After surgical correction of tetralogy of Fallot (four patients), catheter embolisation was used to occlude two large aortopulmonary collaterals (one patient), three small aortopulmonary collaterals (one patient), and two Blalock-Taussig shunts (two patients). In two patients congenital coronary anomalies were occluded--a coronary arteriovenous malformation and a coronary artery/bronchial artery anastomosis. In one patient a pulmonary arteriovenous malformation was embolised. Detachable balloons were used to occlude six large arteries, the three small arteries were occluded with small gelfoam fragments, and the pulmonary arteriovenous malformation was occluded with multiple steel coils and large gelfoam pieces. Successful occlusion was achieved in all cases. No complications were encountered and the procedure was well tolerated even in the two patients receiving postoperative intensive care. Therapeutic embolisation in suitable cases is a safe and effective alternative to surgery and the detachable balloon technique is effective in occluding high flow vessels.     

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.     

Anomalous origin of one pulmonary artery from the ascending aorta

We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions. The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. PATIENTS AND METHODS: Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. RESULTS: At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prolonged intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessful balloon dilation, underwent surgical reoperation. CONCLUSIONS: Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.     

Transfer of cardiac catheterisation from a paediatric to an adult cardiological centre: results at 3 years

The advances of surgical and interventional treatment of congenital heart diseases have allowed a large number of patients with congenital heart disease to reach adult age. This population involves almost 0.3/1000 of total population in West Europe and North America and can be estimated around 200000 patients in France. Patients with operated Tetralogy of Fallot, benign forms of pulmonary atresia with ventricular septal defect, simple or complex transposition of the great arteries usually survive beyond childhood. These patients can need repeated interventions to treat lesions of native or reconstructed pulmonary arteries and/or aortic arch, to occlude residual shunts, to treat pulmonary incompetence. More complex heart diseases such as single ventricle, rarely allow survival until the adult age. The majority of these patients undergo heart transplant, often made difficult by multiple cardiac surgeries, anomalies of pulmonary arteries, chronic cyanosis, aorto-pulmonary shunts. Patients with relatively simple or complex congenital heart diseases need to be followed-up in specialized units, like those created more than twenty years ago in the United States, Canada, and United Kingdom. Interventional cardiac catheterisation play a major role in the management of this population. The results of 3 years of activity in a new centre treating GUCH patients are illustrated.