动脉导管未闭封堵器在儿童膜部瘤室间隔缺损介入治疗中的应用研究

目的 应用动脉导管未闭(PDA)封堵器介入治疗儿童膜部瘤室间隔缺损(VSD),总结其技术要点及治疗体会.方法 20例儿童膜部瘤VSD患儿,根据左室造影结果分别植入不同型号的PDA封堵器,封堵器腰部直径(10.4±2.6)mm;术中经胸心脏超声检查、封堵后15 min重复左室造影,观察即刻封堵效果.结果 20例采用PDA封堵器治疗的膜部瘤VSD患儿中,1例Ⅳ型VSD患儿,因术后残余分流≥2 mm而放弃介入治疗;1例Ⅲ型多孔型VSD患儿术后少量残余分流(＜2mm),1个月后复查心脏超声无残余分流.术后心电图正常(或与术前一致)13例,不完全性右束支传导阻滞4例,第一度房室传导阻滞1例,阵发性交界性心动过速1例.心电图恢复正常时间≤6 d.结论 经导管采用PDA封堵器治疗儿童膜部瘤VSD疗效可靠,治疗关键是选择合适的封堵器,根据膜部瘤的形态、大小、位置确定封堵部位,是介入治疗该种类型VSD的可选方法之一.

Abstract：

Objective To study on transcatheter closure of membranous ventricular septal defect (VSD) with pseudoaneurysm by patent ductus arteriosus(PDA) occlusion devices in children and summarize the skill and clinical experience. Methods The study included 20 membranous VSD cases in children.According to the finding of the left ventricular angiography, various kinds of the PDA occlusion devices was implanted. The mean diameter of the waist of the occluder was ( 10.4 ± 2.6) mm. Examination by transthoracic echocardiography (TTE) immediately and left ventricular angiography after the occluder was implanted 15minutes later to evaluate the efficacy. Results In the 20 patients, one of the Ⅳ type VSD patient was quitted because of the significant residual shunts(≥2 mm). Slightly residual shunts ( ＜ 2 mm) was found in one Ⅲ type VSD patient with multi-outlet. And disappeared in 1 month after the procedure, which VSD patient was confirmed by TTE. Thirteen cases were normal by EKG examination (or the same before procedure).Incomplete right bundle branch block was found in 4 cases. First degree atrioventricular block was found in 1 case and paroxysmal junctional tachycardia was found in 1 case. All of them were recovered in 1 week.Conclusions Transcatheter interventional therapy with PDA occlusion devices for membranous VSD with pseudoaneurysm is safe and effective. The key of the procedure is to select suitable occluder and suitable position to plant them according to the membranous morphologic characteristics,size and position of the pseudoaneurysm. It is a facultative method for transcatheter therapy this kind of congenital heart disease.     

超声对胎儿单纯室间隔缺损在宫内和产后婴儿期闭合情况的研究

目的应用超声心动图观察胎儿期及产后婴儿期单纯室间隔缺损（IVSD）自然愈合的情况及影响因素。方法选取61例诊断有胎儿单纯性室缺但不愿终止妊娠的孕妇为研究对象,对胎儿进行二维和彩色多普勒超声心动图检查,记录胎儿室间隔缺损的位置、形态、大小、自然闭合的时间和闭合率,出生后存活的婴儿监测室间隔缺损（VSD）上述指标至1岁,计算宫内及产后1年的闭合率及总的闭合率、不同位置室缺的闭合率,并对不同大小室缺的闭合率进行组间比较。结果在61例中,22例（36.1%）宫内自然闭合,9例（14.8%）在产后1年内闭合,30例（49.2%）仍未闭合。室间隔缺损的大小以及缺损的位置与愈合率有关,室间隔缺小于3.0mm的闭合率明显高于室缺为3.1~5.0mm的闭合率（P=0.005）和5mm以上室缺的闭合率（P=0.002）,但室缺为3.1~5.0mm的闭合率与大于5mm的室缺的闭合率之间无显著性差异（P=0.39）。膜部和肌部缺损的闭合率明显高于干下型室缺的闭合率（P≤0.01）,肌部缺损的闭合率高于膜部缺损的闭合率,但两者之间无统计学差异（P=0.65）。结论单纯室间隔缺损有较高的宫内自然闭合率并且在产后婴儿期仍能自然闭合,这种自然愈合的过程与室间隔缺损的位置和大小显著相关。肌部和膜部缺损最容易愈合,干下型不易闭合;小缺损具较高的闭合率,较大的缺损不易闭合。     

Fermeture percutanée des communications interventriculaires congénitales par la prothèse Amplatzer Duct OccluderII: données préliminaire d’une étude Tunisienne monocentrique

Introduction . Percutaneous closure of congenital ventricular septal defects (VSDs) represents a promising alternative to surgery with lower rate of complications and shorter hospital stay. Its main limitation is the choice of the appropriate device for each type of defect. Aim. To report the experience of the service of cardiology (Sahloul hospital, Sousse, Tunisia) in percutaneous closure of congenital VSDs with Amplatzer Duct Occluder II (ADOII). Methods. This was a retrospective, monocentric study, conducted from January 2013 to December 2017. The study included patients treated by percutaneous closure of congenital VSDs with the ADOII device. Results. Twelve patients (6 boys; 6 girls) were included. The mean±SD of patients’ age and weight were 65±41 months and 23±10 kg, respectively. VSDs were peri-membranous (n=9) and muscular (n=3), and defects were restrictive (n=11) and non-restrictive (n=1). The mean (minimum-maximum) size of VSDs was 4.72 (3-6) mm. Eleven ADOII prostheses were successfully implanted. One failure procedure was noted with migration of the device into the pulmonary artery. A second child with perimembranous defect developed transient atrioventricular block. No deaths occurred. Conclusion. The present early experience shows that percutaneous closure with ADOII device of perimembranous and trabecular VSDs is safe and effective.     

Percutaneous closure of interatrial ostium secundum defects using the Amplatzer device. Apropos of the initial 5 Tunisian cases

We report our initial experience with percutaneous closure of ostium secundum atrial septal defect using Amplatzer device. Between september 2000 and July 2001, five devices were implanted in 5 patients, 4 female and 1 male. Age ranged from 18 to 66 years. A large atrial septal defect with significant shunting was diagnosed by trans thoracic echocardiography. Procedures were performed under general anesthesia with trans esophageal echocardiography guidance. Stretched diameter of atrial septal defects was determined by balloon sizing, ranged from 21 to 32 mm. Amplatzer devices of 20 to 32 mm were respectively deployed. No complications occurred during the procedure. Total occlusion of interatrial communication, was observed in one patient, immediately after the procedure, and in 3 patients 24 hours later. First 3 patients were controlled at months follow up were free of complications. Closure of atrial septal defect with Amplatzer device appears feasible and safe. However, long term results in comparison to surgery remain to be determined before definite conclusion regarding its use can be made.     

Percutaneous closure of ostium secundum atrial septal defect using an Amplazter device. Report of the first 5 Tunisian cases

We report our initial expérience with percutaneous closure of ostium secundum atrial septal defect using Amplatzer device. Between september 2000 and july 2001, five devices were implanted in 5 patients, 4 female and 1 male. Age ranged from 18 to 66 years. A large atrial septal defect with significant shunting was diagnosed by trans thoracic echocardiography. Procedures were performed under general anesthesia with trans esophageal echocardiography guidance. Stretched diameter of atrial septal defects was determined by balloon sizing, ranged from 21 to 32 mm. Amplatzer devices of 20 to 32 mm were respectively deployed. No complications occurred during the procedure. Total occlusion of interatrial communication, was observed in one patient, immediately after the procedure, and in 3 patients 24 hours later. First 3 patients were controlled at months follow up were free of complications. Closure of atrial septal defect with Amplatzer device appears feasible and safe. However, long term results in comparison to surgery remain to be determined before definite conclusion regarding its use can be made.     

Trans-catheter therapy of Lutembacher syndrome: a case report

Lutembacher syndrome refers to the rare combination of a congenital atrial septal defect and acquired mitral stenosis. Traditionally, Lutembacher syndrome has been corrected by surgical treatment. We describe two patients treated percutaneouly with a combined Inoue balloon valvuloplasty and septal defect closure using the Amplatzer septal occlusion device.     

法洛四联症:1岁以下外科根治术

目的回顾总结1岁以下婴儿法洛四联症(tetralogy of Fallot,TOF)根治术的经验。方法 1999年1月至2005年8月,58例1岁以下TOF患儿的根治手术。合并房间隔缺损8例、动脉导管未闭3 例、冠状动脉异常3例、肺动脉闭锁2例、21-三体综合征2例,既往行改良B-T分流术2例。术中发现室间隔缺损(ventricutar septal defect,VSD)嵴下型56例,干下型2例。VSD经右心室修补48例,经右房修补4 例,经右房、右室共同修补6例。VSD开窗2例,右心室流出道跨瓣环补片39例。结果平均体外循环时间为95．5 min,主动脉阻断时间为53．5 min。平均呼吸机辅助通气时间为30 h,重症监护室(intensive care unit, ICU)平均停留时间为4 d,术后平均住院14 d。延迟关胸3例,术后早期死亡2例(3．4％),死亡原因分别为严重心排血量低和顽固性交界性心动过速。术后早期主要并发症包括:心排血量低(5例),肾衰竭(4 例),室上性、交界性心动过速(2例),Ⅲ°房室传导阻滞(AVB)(1例)。随访3个月至5年,均恢复良好,心功能1-2级。结论婴儿期TOF根治术死亡率低,术后早中期随访并发症少,心功能良好。TOF可常规在婴儿期进行根治。     

经导管介入封堵术治疗儿童室间隔缺损36例临床分析

目的探讨经导管治疗儿童先天性心脏病室间隔缺损封堵术的可行性及安全性,进行多方面综合疗效评价。方法通过经胸超声心动图（TTE）选择适合封堵术的先天性心脏病室间隔缺损（VSD）患儿36例,术前常规检测心电图（ECG）,胸部X线等,房间隔缺损（ASD）合并VSD 1例。在TTE及X线引导下行介入封堵术,术后通过胸片、心电图和TTE进行动态的随访观察。结果35例患儿封堵成功,技术成功率为97.2%,1例（2.8%）手术失败,改为体外循环下矫正畸形。术后5例病人有少量残余分流,2例出现房室传导阻滞（AVB）,1例出现完全性右束支传导阻滞（RBBB）,1例出现不完全性左束支传导阻滞（iLBBB）,封堵器脱落1例,心包填塞（PT）1例。VSD术前TTE测量值与术中X线测量值比较,差异无统计学意义。术后75%以上病人接受了3~6个月随访,35%以上病人接受了12个月随访,X线及TTE检查：全部显示肺血流量减少,右心房、右心室缩小,心电图提示无明显心律失常发生。结论经导管先天性心脏病封堵术治疗VSD具有很高的成功率及安全性;介入封堵术疗效好,并发症少,术后恢复快;TTE和X线可以准确地应用于先天性心脏病封堵术中。     

Closure of a ventricular septum defect in a 4-year-old boy during percutaneous heart catheterisation

A 4-year-old boy in whom a muscular ventricular septal defect (VSD) had been diagnosed at birth presented with limited exercise tolerance and mild cardiomegaly. The moderately large defect was closed with an umbrella device via percutaneous heart catheterisation. Thereafter, his symptoms disappeared and the dimensions of the left heart returned to normal. A VSD occurs in 1.5-3.5 per 1,000 live births. In symptomatic patients with a medium or large-sized VSD, surgical closure is indicated to prevent the development of a fixed pulmonary resistance hypertension, ventricular dysfunction and the risk of endocarditis. Depending on the size and localisation of the defect, closure with an umbrella device may be chosen. The initial results have been promising in children. The safety and efficacy in the long term are still unknown.     

经胸与经食管超声心动图在室间隔缺损术前对照研究中的应用价值

目的应用经胸和经食管超声心动图在室间隔缺损术前对比观察缺损类型和大小。方法 24例室间隔缺损患儿,术前分别进行经胸和经食管超声心动图检查。年龄3～12岁,平均年龄8.6±4.3岁,体重15～40kg,平均体重20.2±9.8kg。膜周型室间隔缺损18例,嵴内型室间隔缺损6例。缺损大小3～9mm,平均大小5.7±3.6mm。术前分别用两种方法观察缺损的大小,部位及缺损与周边组织的关系。结果二者对于缺损定位及大小的观察没有明显差异。对于缺损分流束的观察经食管超声明显优于经胸超声。结论经胸与经食管超声心动图在术前评价室间隔缺损中发挥着重要的作用,经食管超声评价缺损分流束的多少更具优势。     

介入治疗后的学龄期室间隔缺损患儿的认知能力及行为问题

目的研究介入治疗后的室间隔缺损(VSD)患儿的认知能力及行为问题,分析两者之间的关联性。方法选择自2010年1月-2012年12月在山东省立医院进行经导管介入封堵治疗的VSD 64例患儿。采用P300事件相关电位来评估VSD患儿的认知能力,另外采用儿童行为问题问卷来评估患儿的情绪和行为问题,分析这两者之间的关联性,并以56位正常对照儿童参与了本研究。结果 1)与对照组相比,VSD患儿在额叶和顶叶部位具有长的潜伏期,额叶和顶叶部位的波幅明显低于对照组;2)VSD的男童存在抑郁、体诉等行为问题,女童存在抑郁、强迫、社会退缩、违纪等行为问题;3)存在抑郁、体诉问题的VSD男童事件相关电位的异常分别表现为顶叶潜伏期延长、波幅降低及额叶潜伏期延长;4)存在抑郁、强迫、社会退缩、违纪问题的VSD女童事件相关电位的异常分别表现为顶叶潜伏期延长和波幅降低、额叶波幅降低、额叶潜伏期延长、顶叶波幅降低。结论与正常儿童相比,介入治疗后的VSD患儿,学龄期的认知能力存在轻度的损害,且有一定程度的情绪和行为问题,VSD患儿的行为问题与异常的事件相关电位有一定的相关性。     

降低房室间隔缺损介入治疗失败后外科手术病死率的探讨

目的 探讨降低房室间隔缺损介入治疗失败后外科手术病死率的有效措施.方法 对16例房间隔缺损(ASD)和室间隔缺损(VSD)介入治疗失败需外科手术患者的临床资料进行回顾性分析.手术均在体外循环下进行,取出封堵器,修复心内畸形.结果 本组无外科手术死亡病例.ASD部位为中央型5例,下腔型7例,与术前诊断相符率41.7%(5/12),不相符率58.3%(7/12)(P>0.05).术后ASD直径平均(31.0±1.0)mm,较术前诊断的(26.0±2.3)mm增大(P<0.05).术后VSD直径平均(5.0±0.8)mm,与术前诊断的(4.0±0.3)mm比较差异无统计学意义(P>0.05 o 3例第三度房室传导阻滞术后均恢复窦性心律,心内畸形修复完善.结论 房室间隔缺损介入治疗后发生严重并发症及时采取外科手术治疗,可提高手术成功率.     

经胸超声心动图在先天性心血管疾病介入治疗中的应用

[目的]探讨经胸超声心动图对先天性心脏病微创介入治疗的应用价值。[方法]回顾性分析某院2003年10月～2009年4月之间共38例先天性心脏病住院患者(房间隔缺损16例、室间隔缺损9例、动脉导管未闭13例)在经微创介入治疗前、治疗后即刻及治疗后3个月、1年等4个时间段经胸超声心动图检查的结果来评价微创介入治疗的疗效。[结果]38例患者全部封堵成功,术后1年复查封堵术疗效良好。[结论]经胸超声心动图对微创介入封堵先天性心脏病的术前病例选择、术中监测引导、术后疗效评价等都具有十分重要的应用价值。     

Tei指数对房缺患者修补术与封堵术前后右心功能的评价

目的比较继发孔房间隔缺损(ASD)患者经皮Amplatzer伞片封堵术和外科修补术两种术式手术前后右心室的心肌做功指数(Tei指数)的改变。方法继发孔ASD患者行外科修补术10例和经皮伞封堵术10例分别于术前、术后30天行超声心动图检查、右心室Tei指数测定。结果继发孔ASD患者均成功行经皮Amplatzer伞片封堵术以及外科修补术,两组患者的右室Tei指数在术前和术后30天相比差异无统计学意义(P>0.05)。结论修补术和封堵术对右心室Tei指数不产生明显影响。     

Anatomic correction of the congenitally corrected transposition of the great vessels by the first successful "double switch" operation in Hungary

Congenitally corrected transposition of the great arteries is a rare defect characterized by discordant atrioventricular and ventriculoarterial connections. Symptoms result from one or a combination of associated cardiovascular malformations, including ventricular septal defect, pulmonary stenosis or atresia, tricuspid valve dysfunction, dextrocardia, hypoplastic left or right ventricle. Correcting exclusively the associated defects, leaving the morphologic right ventricle in systemic position, will determine the patient's life-long prognosis. Anatomic repair by double switch technique may improve survival of patients with congenitally corrected transposition of the great arteries by establishing the morphologic left ventricle in the systemic circulation. A 3-year-old girl with congenitally corrected transposition of the great arteries, ventricular septal defect, hypoplastic right ventricle, and previous palliative procedure was corrected by double switch technique and patch closure of ventricular septal defect. The authors prefer the double switch procedure inspite of its many surgical challenges because it has very good long-term outcome. The more simple surgery namely the operation of only associated cardiac defects will involve the possibility of deterioration of right ventricular function.     

Good results after closure of a arterial septal defect during heart catheterisation instead of surgery

OBJECTIVE: Evaluation of the first results in the Netherlands of percutaneous and transvenous closure of an ASD II in children with an Amplatzer Septal Occluder (ASO). DESIGN: Prospective. METHOD: Data were collected from children with an ASD II prior to, during and up to 24 months after the insertion of an ASO during heart catheterisation in Leiden University Hospital, the Netherlands. RESULTS: Between 1 January 1998 and 29 February 2000, 28 patients (12 girls, 16 boys; mean age: 74 months (range: 15-198 months)) underwent heart catheterisation to close an ASD II with an ASO. In 26 patients an ASO could be placed without significant complications. The size of the device varied from 9-34 mm (median 16 mm). In one patient ASD closure was not attempted because of multiple ASDs. In another patient the procedure was stopped after air embolism into the coronary arteries had occurred during preparation of ASO implantation. In 23/26 patients with an implanted ASO, no residual shunt was present after 24 hours. One child, in whom the defect was found to be closed after 24 hours and after three weeks, returned abroad and was lost to follow-up. After one year all defects (n = 22) were completely closed. CONCLUSION: Percutaneous transvenous closure of an ASD II with an ASO was possible, was not associated with any significant complications and had a high success rate, even in relatively young children with large defects.     

肺动脉瓣下室间隔缺损241例临床分析

目的 总结肺动脉瓣下室间隔缺损(SPVSD)的临床特点及治疗经验.方法 回顾性分析241例SPVSD患者的临床资料.结果 术前经超声心动图确诊215例,误诊26例(10.79%,26/241).合并主动脉瓣脱垂42例,心电图多表现为左室肥大.均行室间隔缺损(VSD)心内修复术,同期行主动脉瓣置换术(AVR)8例,主动脉瓣成形术(AVP)6例,合并其他病变亦同期处理.术中证实VSD大小比术前彩超测量值偏大.无围手术期死亡,无一例出现第三度房室传导阻滞.230例术后随访3个月至5年,随访率95.44%(230/241),NYHA心功能Ⅰ级189例,Ⅱ级41例.均无VSD残余漏.行AVR或AVP患者,术后轻度关闭不全1例,余关闭良好,其他合并病变处理随访结果亦满意.结论 加强对SPVSD的全面认识,提高诊断的准确率,积极手术治疗,合理处理主动脉瓣病变及其他合并畸形或病变,可取得满意的疗效.     

Assessing familial aggregation of congenital cardiovascular malformations in case-control studies

Recent data indicate that the familial aggregation of congenital cardiovascular malformations (CCVM) varies with the type of defect in the index case. Using a logistic regression model that allows for dependence among family members, we calculated the risk of any CCVM to case relatives compared with relatives of controls. Data from 3,908 first-degree relatives of 570 matched cases and controls identified from 1981 through 1985 by the Baltimore-Washington Infant Study were used in the analyses. Overall risk for any CCVM in case relatives was increased four-fold over that of control relatives. While relatives of cases with flow lesions (including right and left heart defects, as well as perimembranous ventricular septal defect [VSD]) had a five-fold increase in risk, the risk to relatives of nonflow lesion cases did not differ significantly from the risk to relatives of controls. Sex, maternal age, miscarriage history in the mother, and birth order had no apparent effect on risk among siblings. However, there was an indication of increased risk in relatives of nonwhite cases with VSD compared to relatives of matched controls. However, with these data it was not possible to distinguish between environmental and genetic sources of this familial aggregation.     

Aortic endocarditis complicated with a large ventricular septal defect and septic pulmonary embolism

BACKGROUND: Infectious complications of the aortic valve can lead to severe cardiac failure and widespread contiguous lesions by the involvement of subaortic structures such as aorto-left ventricular discontinuity, destruction of the aortic ring, aortic abscesses, true or false aneurysms and shunts. AIM: Report a new case of a large ventricular septal defect due to infection. CASE REPORT: Abnormal communications occurring during acute aortic valve endocarditis are rare but they are very serious complications. We report a case of a 58 year-old-man, referred to our hospital with the diagnosis of aortic endocarditis with complicating root abscesses, acquired ventricular septal defect (VSD) and pulmonary septic embolism. Surgery was indicated and the whole procedure was performed through the aortic root. The patient underwent a radical resection of the abscesses, reconstruction of the aortic ring, closure of the ventricular septal defect and prosthetic replacement of the aortic valve. The immediate postoperative course was marked by persistent sepsis. Transoesophageal echocardiography showed vegetation's in the right side of the patch without signs of dehiscence. The outcome was fatal due to hemodynamic collapse. CONCLUSION: The authors would like to select and analyze some weaknesses of their procedure like using only the transaortic access that often limits exposure of possible right-side lesions, not removing the pulmonary obstruction and using an important amount of foreign material to reconstruct subvalvular lesions.     

新的连续缝合技术与传统方法修补膜周大室间隔缺损的比较研究

目的 应用新的连续缝合技术修补膜周大室间隔缺损(VSD),并与传统方法进行对比研究.方法 手术治疗321例膜周大VSD,根据手术方式的不同分为三组:A组70例采用间断缝合法修补;B组116例采用传统连续缝合法修补;C组135例采用新的连续缝合技术修补.结果 转机时间和阻断时间c组最短,分别为(48±36)min和(26±18)min;残余分流在C组无一例发生,发生率均较其他两组低(P<0.05);各组均无第三度房室传导阻滞(AVB)发生,但都有第二度AVB,C组为0.7%(1/135),低于A组的4.3%(3/70)(P<0.05),与B组的0.9%(1/116)比较差异无统计学意义,随访1～3年均恢复为正常窦性心律;完全性右束支传导阻滞三组发生率差异无统计学意义;三尖瓣关闭不全A组为8.5%(6/70),高于其他两组(P<0.05).结论 新的连续缝合法能减少手术阻断及转机时间、减少心内异物存留、简化手术原则及操作技术,减少了残余分流发生、不增加传导阻滞的发生.