Transcatheter Closure of Multiple Perimembranous Ventricular Septal Defects With Septal Aneurysm Using Two Overlapping Amplatzer Duct Occluders II

Ventricular septal defect (VSD) is a common congenital heart defect in children. Perimembranous VSD (Pm VSD), a defect involving the membranous septum and the adjacent portion of the muscular septum, accounts for about 70 % of cases. Transcatheter closure of Pm VSDs using the Amplatzer Pm VSD device, although successful in many patients, has been associated with major adverse events. Complete heart block, thromboembolism, and new-onset valvular regurgitations in patients undergoing VSD closure using the Pm VSD occluder have been reported [3]. Recently, a new retrograde approach for transcatheter closure of Pm VSDs using the Amplatzer Duct Occluder II (ADO II) has been described with a 90 % success rate [1]. This report describes a child who had multiple Pm VSDs with septal aneurysm undergoing successful transcatheter closure using two ADO II occluders with relative ease.     

Device closure of congenital ventricular septal defects with Amplatzer devices: first experiences in Turkey

Ventricular septal defects (VSD), which cause volume overload, may be closed by interventional method. The success depends on the precise anatomic definition of the defect and its relation to other cardiac structures. We report our first experiences of transcatheter closure of perimembranous and muscular VSD. Between May 2005 and September 2006, transcatheter closure of VSD was attempted in 38 patients. Implantation was successful in 37 patients. In one patient, the procedure failed because of the long sheath kinking. We observed important complications in three patients: severe tricuspid valve regurgitation, residual VSD and tricuspid valve regurgitation and right bundle branch block in the short-term follow-up. Transcatheter device closure with Amplatzer device seems to be effective and safe in the treatment of perimembranous and muscular VSDs. Tricuspid valve incompetence may cause problems. Long-term results are required to determine the efficacy and safety.     

Ventricular septal defect with mobile tricuspid valve pouch mimicking tetralogy of Fallot

Summary A newborn with partial deletion of the long arm of chromosome six presented with cyanosis at birth. Initial studies suggested the diagnosis of tetralogy of Fallot and the infant underwent a Waterston shunt procedure with subsequent clinical improvement. The infant subsequently died of unrelated causes. Autopsy revealed a ventricular septal defect and a dysplastic pouchlike structure related to an anomalous tricuspid valve leaflet which obstructed right ventricular outflow. Classical anatomic findings of tetralogy of Fallot were absent. Clinical and echocardiographic findings that would be expected to aid early diagnosis of future cases are presented.     

膜周部室间隔缺损经导管封堵术后早期心律失常危险因素的探讨

目的心律失常是经导管封堵膜周部室间隔缺损术后的常见并发症,不仅影响术后心功能的恢复,而且直接关系到预后。分析18岁以内患者膜周部室间隔缺损经导管封堵术后早期心律失常及其危险因素,以期降低术后心律失常的发生率。方法2002年6月—2004年6月,89例膜周部室间隔缺损患者根据家长意愿接受了经导管应用Amplatzer装置及国产封堵器进行堵塞。80例应用AGA公司制造的Amlatzer膜周部室间隔缺损封堵器,9例应用国产封堵装置,术后定期行心脏超声及心电图检查。结果89例膜周部室间隔缺损封堵术均获成功。11例术后5d内发生了不同类型的传导阻滞,发生率为12%,分别为Ⅰ°房室传导阻滞1例,Ⅲ°房室传导阻滞1例,左前分支阻滞5例,不完全(部分)性右束支传导阻滞4例,完全性右束支传导阻滞3例,其中3例并发两种传导阻滞。分析本组心律失常的影响因素,发现:(1)室间隔缺损上缘距主动脉右冠瓣距离<3mm;(2)室间隔缺损直径≥8mm,封堵器直径≥10mm;(3)穿刺成功后动脉静脉(A-V)轨道建立时间≥60min及术中pH值<7·35与心律失常密切相关。结论心律失常是膜周部室间隔缺损封堵术后早期严重的并发症;严格选择手术适应证、缩短手术操作时间、防止酸中毒是降低膜周部室间隔缺损介入封堵术后心律失常发生率的有效措施。     

Simultaneous Transcatheter Closure of Ventricular Septal Defect and Pulmonary Valvuloplasty

Background

Transcatheter balloon valvuloplasty has been the accepted first line treatment for congenital pulmonic stenosis (PS) in children. Transcatheter closure of perimembranous ventricular septal defect (VSD) with Amplatzer VSD occluder is an alternative to surgical repair.

Case Presentation

A 12 year old boy presented with history of exertional dyspnea and atypical chest pain. Physical findings were suggestive of severe pulmonic stenosis. Transthoracic echocardiography, right and left ventricular angiography showed medium to large sized perimembranous VSD and severe valvar pulmonary stenosis. Transcatheter closure of VSD was done first so as to avoid large left to right shunt across VSD after balloon pulmonary valvuplasty.

Conclusion

We demonstrated the feasibility and success in treating combined ventricular septal defect and severe pulmonary valve stenosis with transcatheter interventional procedure in the same session.     

应用国产封堵器治疗室间隔缺损对心脏瓣膜功能的影响

目的 探讨应用国产封堵器进行室间隔缺损(VSD)封堵术对心脏瓣膜功能的影响,并与Amplatzer封堵器、外科手术治疗VSD对心脏瓣膜功能的影响进行对比.方法 将VSD患儿分为三组,国产封堵器组76例,Amplatzer封堵器组34例,外科手术组56例;国产封堵器组分别与Amplatzer封堵器组、外科手术组在术后1周及3个月对三尖瓣关闭不全、三尖瓣狭窄及主动脉瓣关闭不全的发生率进行对比研究.两组间率的比较采用卡方检验.结果 在术后1周,国产封堵器组主动脉瓣关闭不全的发生率较外科手术组高(P<0.05),与Amplatzer封堵器组比较差异无统计学意义(P>0.05).术后3个月,国产封堵器组与外科手术组、Amplatzer封堵器组比较.主动脉瓣关闭不全的发生率差异无统计学意义(P>0.05).术后1周及3个月,国产封堵器组与外科手术组、Amplatzer封堵器组分别比较,三尖瓣关闭不全、三尖瓣狭窄的发生率差异无统计学意义(P>0.05).结论 国产封堵器作为封堵VSD的新器械,在术后及近期对心脏瓣膜功能的影响轻.为避免瓣膜功能受影响,应严格掌握介入术的指征,术中应用心脏超声监测,避免操作动作粗暴,选用合适大小的封堵器.     

Perforation of the Right Aortic Valve Cusp: Complication of Ventricular Septal Defect Closure with a Modified Rashkind Umbrella

An 18-month-old boy with a perimembranous ventricular septal defect (VSD) had undergone transcatheter closure of the defect with a modified 17 mm Rashkind umbrella device at age 4 months (weight 3.8 kg). The clinical signs of a VSD persisted, and he developed aortic incompetence, first detected 5 months after the procedure, which progressed from mild to moderate. A three-dimensional echocardiographic study demonstrated that one of the four arms holding the umbrella was protruding into the aortic valve and had perforated the right aortic valve cusp. This diagnosis was confirmed at subsequent surgery. Surgical repair of the perforated right aortic valve leaflet was necessary. The umbrella was adherent to the tricuspid valve and could not be removed. Instead it was left in situ, but three of the stainless steel arms were cut off. When umbrella closure of a perimembranous VSD is undertaken, the close proximity of part of the distal umbrella to the aortic valve can lead to aortic regurgitation.     

23例小儿室间隔缺损的镶嵌治疗

目的探讨镶嵌手术（hybrid procedure）治疗室间隔缺损的方法及临床疗效。方法2006年1月至2008年7月,在经食管超声心动图（TEE）辅助下,采用镶嵌手术治疗室间隔缺损23例。其中15例经有心室表面穿刺置入封堵器,4例在体外循环（CPB）下直视置入封堵器,4例在CPB下置入封堵器后同期手术矫治其他畸形。结果23例均获成功,14例膜部室间隔缺损封堵器直径6～12mm,9例肌部室间隔缺损封堵器直径5～10mm。术后恢复顺利,心功能正常,超声随访无残余分流、主动脉瓣返流及周围组织卡压。结论将心脏外科手术与内科介入相结合的镶嵌治疗是小儿先天性心脏病的新型治疗模式,简化了,手术过程,降低了嗣手术期并发症及病死率,从而降低了手术风险。     

先天性心脏病术后残余室间隔缺损介入治疗及中长期随访

目的 评价先天性心脏病（先心病）术后残余室间隔缺损（VSD）介入治疗的效果及中长期随访结果。 方法 回顾性分析2002年9月至2014年5月复旦大学附属儿科医院介入治疗的先心病术后残余VSD患者的临床资料,对辅助检查、介入治疗情况和随访行描述性分析。结果 21例患者进入分析,男11例,女10例,平均年龄8.8（2.7～21）岁,平均体重26.3（10.5～53.5） kg。13例为VSD修补术后残余漏,其中2例合并主动脉弓缩窄修补术后再缩窄;5例为法洛四联症（TOF）术后残余VSD,其中1例合并频发室性早搏及短阵室性心动过速;2例为右室双出口术后残余VSD;1例为完全性大动脉转位术后残余VSD。介入治疗距离外科手术时间为11～80个月。①介入治疗中左心室造影测得VSD右心室面直径为(3.73±1.56) mm,4例右室面有多处分流,1例为左室-右房瘘;肺循环与体循环血流量之比为1.53±0.23;无中度以上肺动脉高压者。②21例患者中,1例介入治疗中建立轨道时反复发生Ⅲ度房室传导阻滞而放弃堵闭行外科手术治疗;20例（95.2%）封堵成功,其中1例应用2个堵闭器。1例合并频发室性早搏及短阵室性心动过速患儿同时行射频消融治疗成功;2例合并主动脉弓再缩窄患儿经球囊扩张后缩窄解除。介入治疗中无不良事件发生。介入治疗后即刻造影和经胸超声心动图显示少量残余分流3例。ECG均无异常发现。③20例随访1～79个月,均无明显临床症状。2例在1年随访中ECG分别出现不完全性右束支传导阻滞及Ⅰ度房室传导阻滞,继续随访6~12个月无改变;2/3例即刻有残余分流患儿在随访中残余分流消失,1例介入治疗后20个月时仍有残余分流3.4 mm,心腔大小和功能正常;无新发的三尖瓣或主动脉瓣反流,3例堵闭前存在的中重度三尖瓣反流在随访中反流均为轻度;未见堵闭器移位、脱落或血栓形成。结论 经导管堵闭介入治疗先心病手术后残余VSD是一项安全、有效和易行的方法,介入治疗后即刻和中长期随访效果好,应成为此类患儿首选的治疗方法。     

Transcatheter device closure of muscular ventricular septal defect

Traditional treatment of muscular ventricular septal defect (VSD) is surgical closure, but it carries considerable morbidity and mortality. The Amplatzer muscular VSD occluder (AGA?Medical Corporation, Plymouth, MN, USA) was approved by the United States Food and Drug Administration in September 2007 and by the Taiwan Department of Health in September 2009. It is a self-expandable double-disc device made from a nitinol wire mesh. Dacron polyester patches are sewn into each disc and the connecting waist to increase the thrombogenicity of the device. Many reports have shown that transcatheter device closure of muscular VSDs is effective and safe. It has no scar, less pain, shorter hospital stay, and less cost compared to the traditional open heart surgery.     

Comparison of types of pulmonary stenosis with the state of the ventricular septum in complete transposition of the great arteries

Summary The pulmonary valve and left ventricular outflow tract of 214 hearts with d-transposition of the great arteries (d-TGA) were visually inspected (126 of 214 with intact ventricular septum and 88 of 214 with ventricular septal defect [VSD]). Pulmonary stenosis (PS), either valvular, subvalvular, or in combination, was present in 26 cases and was found to be more common in the presence of a VSD (20.5%) than intact ventricular septum (6.3%). PS occurred more commonly in the presence of a supracristal VSD than an infracristal VSD (70%, 7 of 10 vs 15%, 10 of 66). Further, infracristal or supracristal VSDs were associated with different types of pulmonary obstruction. In seven of ten cases with infracristal VSD and PS, the stenosis was caused by an anomaly of an atrioventricular valve. In six of seven cases with supracristal VSD and PS, the latter was caused by membranous encirclement of the left ventricular outflow tract. * Presented in part as an abstract at the American Heart Association Scientific Sessions, Fall 1987.     

Transcatheter device closure of congenital ventricular septal defects

Ventricular septal defects (VSD) are among the most commonly occurring congenital heart lesions. While successful surgical repair of VSDs has been possible for over 60 years, peri-operative and late complications still occur and there are inherent risks associated with sternotomy and exposure to cardiopulmonary bypass. A desire to avoid these risks, as well as the successful application of catheter-based device closure for other intracardiac and vascular shunting lesions, has lead to an interest in developing a less invasive means by which to close VSDs. The aim of this article is (a) to understand the important differences in approach to catheter-based closure of congenitally occurring VSDs, based on their location, (b) to discuss the technical aspects of device closure of VSDs, from both a percutaneous and perventricular (hybrid) approach, and (c) to review the outcomes of the experience with these devices. Acquired (post-infarction, post-trauma) VSDs will not be addressed.     

膜周部室间隔缺损经导管介入封堵后早期并发症危险因素的分析

目的：探讨膜周部室间隔缺损（VSD）经介入治疗后早期并发症的危险因素。 方法：回顾性纳入2015年10月1日至2017年10月1日在重庆医科大学附属儿童医院行膜周部VSD介入封堵术的患儿,从医院电子病历系统中截取临床资料,分析术后总并发症、新发心律失常、残余分流、新发中度及以上反流的危险因素。 结果：①186例患儿进入本文分析,男97例,年龄18~148（45.4±25.4）个月,体重8.5~48（15.7±6.1) kg;超声缺损（5.6±1.9）mm,压差（68.6±13.7）mmHg,距主动脉瓣距离＜3 mm 8例（4.3%）,术前有心律失常18例（9.7%）,合并膜部瘤70例（37.6%）;封堵器大小（6.3±1.2）mm,封堵器形状对称178例（95.7%）、偏心8例;麻醉方式为全麻147例（79%）、局麻39例,手术时间（38.8±15.0）min。②术后住院期间共发生并发症60例（32.3%）,新发心律失常30例（16.1%）;残余分流30例（16.1%）,其中1例为大量分流;新发中度及以上瓣膜反流9例（4.8%）,均为三尖瓣反流。③多因素Logistic回归分析显示,总并发症：超声下大缺损（OR=1.264,95％CI: 1.040～1.536,P=0.019）和偏心型封堵器（OR=6.732,95％CI: 1.018～44.509,P=0.048）为危险因素;残余分流：超声下大缺损（OR=1.577,95％CI: 1.154～2.155,P=0.004）和右心室收缩压升高（OR=1.080,95％CI: 1.001～1.165,P=0.047）为危险因素,左心室平均压升高为保护因素（OR=0.913,95％CI: 0.864～0.966,P=0.001）;新发中度及以上瓣膜反流：右心室收缩压升高（OR=1.068,95％CI: 1.006～1.133,P=0.030）和更大的封堵器（OR=1.585,95％CI: 1.003～2.506,P=0.048）为危险因素。 结论：超声下VSD大小、封堵器的大小与形状、右心室收缩压、左心室平均压与膜周部VSD介入治疗术后早期并发症发生有关,同时提示血流动力学稳定性、右心功能在术后并发症发生中的重要作用。     

小儿室间隔缺损术后残余漏

８例小儿室间隔缺损修补术后残余漏再次手术病例分析：残余漏表现为５例术后残留心前区Ⅲ级以上收缩期杂音，７例血红蛋白尿，２例右心衰，８例超声心动图（ＵＣＧ）证实心室水平残余左向右分流。均于术后２～１１２天行残余漏修补。术中见残余漏原因为不同部位缝针处组织撕裂６例，修补不全２例。８例均经带垫片褥式缝合加固，７例痊愈，１例再次发生残余漏。本文对残余漏特点、诊断、手术适应证及时机进行了讨论。     

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??Abstract??Objective??To study prevention and treatment methods for tricuspid injury in transcatheter closure procedure of perimembranous ventricular septal defects??PMVSDs??. Methods??Eight cases with tricuspid injury in transcatheter closure procedure of PMVSDs were enrolled from Oct. 2002 to Dec. 2009. Tricuspid stenosis presented in 4 cases??of whom 2 needed surgery. The right disc and screw of PMVSDs occluder got entangled into the chordae of anterior tricuspid leaflet in both operative cases. Tricuspid regurgitation occurred in 4 cases. Three cases received surgery repair and device removal. The operation finding was anterior papillary muscle rupture in 1?? anterior papillary muscle rupture combined with anterior tricuspid leaflet tear in 1 and chordae tendineae of septal leaflet stretched by device screw in 1 case. Another patient with mild to moderate tricuspid regurgitation and septal leaflet prolapse also received follow-up study. Results??Among the 5 patients with surgery?? tricuspid valve got to normal in 4 cases and valve flow velocity slightly accelerated in 1 case. In the follow-up study?? there were no clinical symptoms in the cases with mild or mild to moderate tricuspid injury with no repair. Conclusion??In procedure of transcatheter closure of PMVSDs?? passing the arteriovenous circuit wire through tricuspid chordae can lead to tricuspid injury. Sever tricuspid injury should be repaired by surgery.     

Atrioventricular Septal Defect with Cor Triatriatum: Case Report and Review of the Literature

Atrioventricular septal defect with cor triatriatum is a rare combination, with only nine cases reported so far. Cor triatriatum, a potentially correctable cause of pulmonary arterial hypertension in atrioventricular septal defect patients, was missed in a few earlier case reports, leading to death and subsequent diagnosis at autopsy. We report the case of 3-year-old female child with this combination (partial atrioventricular septal defect with cor triatriatum) wherein the final diagnosis was made at cardiac catheterization. Successful surgical correction was performed.     

Spectrum of infective endocarditis during infancy and childhood: 20-Year review

Summary The medical records of the 29 patients under 18 years of age with infective endocarditis (IE) seen over a 20-year period by our department were reviewed to provide an overview of the spectrum of IE during infancy and childhood. None of the 29 patients had had previous cardiovascular surgery. The mean age at onset of IE was 7 years 2 months; 3 patients (10%) were under 2 years of age at onset. One patient during the early years died following 4 months of treatment with various antibiotics. Three patients underwent urgent surgery, and 17 patients with healed IE had elective surgery. All of the 20 patients who were operated on survived. The remaining 8 were followed with medical treatment alone. Positive blood cultures were obtained from 24 (83%) patients, and streptococci were still commonly found (38%). Ventricular septal defect (VSD) accounted for 66% of underlying heart diseases and rheumatic heart diseases for 14%. Vegetations were detected in 12 (67%) of 18 patients observed by echocardiography. Among these 12 patients, 1 with VSD underwent urgent tricuspid valve replacement and VSD closure because of worsening congestive heart failure due to progressive tricuspid regurgitation. Echocardiography identifies patients at high risk with IE, though the presence of a vegetation on echocardiography does not necessarily of itself dictate surgical intervention.     

室间隔缺损封堵器关闭婴儿大管型动脉导管未闭的初步评价

目的 应用国产室间隔缺损封堵器关闭婴儿大管型动脉导管未闭并对其疗效进行初步评价.方法 2007年5月-2008年2月就诊的10例4～9个月婴儿经主动脉造影确诊为大管型动脉导管未闭,最窄处内径为4.1～7.4 mm,长度7.9～11.9 mm,合并中～重度的肺动脉高压.选用6～12 mnl国产肌部或膜部室间隔缺损封堵器进行介入治疗,术后主动脉造影、术后24 h及1～6个月进行超声心动图随访观察疗效.结果 全组技术成功率100%,术后主动脉造影和超声心动图显示无残余分流.随访超声心动图无明显降主动脉缩窄,1例左肺动脉轻度狭窄.无其他不良并发症.结论 室间隔缺损封堵器可用于关闭婴儿粗大管型的动脉导管未闭,成功率高,近期疗效肯定,中远期疗效需随访观察.     

Fetal cor triatriatum dexter: a report of two cases associated with nuchal edema in early second trimester.

Two early-2nd-trimester fetuses were aborted as a result of nuchal edema and suspected severe heart malformation. At autopsy one fetus demonstrated nuchal edema, mild hydronephrosis, and cor triatriatum dexter associated with ventricular septal defect and tubular hypoplasia of the aortic arch. The other fetus demonstrated severe nuchal edema, and cor triatriatum dexter was the only malformation. Cor triatriatum dexter is a rare cardiac malformation characterized by division of the right atrium into 2 compartments by a usually fenestrated membrane representing remnants of the right valve of the embryonic sinus venosus. This malformation has been diagnosed in adults and children by echocardiography, surgery, or autopsy but has not previously been published in fetuses.     

Multicenter Experience with Perventricular Device Closure of Muscular Ventricular Septal Defects

Hybrid procedures are becoming increasingly important, especially in the management of congenital heart lesions for which there are no ideal surgical or interventional options. This report describes a multicenter experience with perventricular muscular venticular septal defect (VSD) device closure. Three groups of patients (n = 12) were identified: infants with isolated muscular VSDs (n = 2), neonates with aortic coarctation and muscular VSDs (n = 3) or patients with muscular VSDs and other complex cardiac lesions (n = 2), and patients with muscular VSDs and pulmonary artery bands (n = 5). Via a sternotomy or a subxyphoid approach, the right ventricle (RV) free wall was punctured under transesophageal echocardiography guidance. A guidewire was introduced across the largest defect. A short delivery sheath was positioned in the left ventricle cavity. An Amplatzer muscular VSD occluding device was deployed across the VSD. Cardiopulmonary bypass was needed only for repair of concomitant lesions, such as double-outlet right ventricle, aortic coarctation, or pulmonary artery band removal. No complications were encountered using this technique. Discharge echocardiograms showed either mild or no significant shunting across the ventricular septum. At a median follow-up of 12 months, all patients were asymptomatic and 2 patients had mild residual ventricular level shunts. Perventricular closure of muscular VSDs is safe and effective for a variety of patients with muscular VSDs.