Risk factors of early mortality and major morbidity in adult patients after surgical repair of partial atrioventricular septal defects

Objective To investigate the risk factors for prolonged postoperative mechanical ventilation patients with atrioventricular septal defect(AVSD).Methods We retrospectively analyzed the clinical data of 76 patients with atrioventricular septal defect aged more than 18 years in our hospital from January 1^st 2011 to December 31^st 2017.

MORPHOLOGICAL DIAGNOSIS OF ATRIOVENTRICULAR SEPTAL DEFECT： COMPARISON BETWEEN TWO DIMENSIONAL ECHOCARDIOGRAPHY， ANGIOCARDIOGR

ＭＯＲＰＨＯＬＯＧＩＣＡＬＤＩＡＧＮＯＳＩＳＯＦＡＴＲＩＯＶＥＮＴＲＩＣＵＬＡＲＳＥＰＴＡＬＤＥＦＥＣＴ：ＣＯＭＰＡＲＩＳＯＮＢＥＴＷＥＥＮＴＷＯＤＩＭＥＮＳＩＯＮＡＬＥＣＨＯＣＡＲＤＩＯＧＲＡＰＨＹ，ＡＮＧＩＯＣＡＲＤＩＯＧＲＡＰＨＹＡＮＤＳＵＲＧ...

Completed atrioventricular block induced by atrial septal defect occluder unfolding:A case report

BACKGROUND An atrial septal defect is a common condition and accounts for 25%of adult congenital heart diseases.Transcatheter occlusion is a widely used technique for the treatment of secondary aperture-type atrial septal defects(ASDs).CASE SUMMARY A 30-year-old female patient was diagnosed with ASD by transthoracic echocardiography(TTE)1 year ago.The electrocardiogram showed a heart rate of 88 beats per minute,normal sinus rhythm,and no change in the ST-T wave.After admission,TTE showed an atrial septal defect with a left-to-right shunt,aortic root short-axis section with an ASD diameter of 8 mm,a parasternal four-chamber section with an ASD diameter of 9 mm,and subxiphoid biatrial section with a diameter of 13 mm.Percutaneous occlusion was proposed.The intraoperative TTE scan showed that the atrial septal defect was oval in shape,was located near the root of the aorta,and had a maximum diameter of 13 mm.A 10-F sheath was placed in the right femoral vein,and a 0.035°hard guidewire was used to establish the transport track between the left pulmonary vein and the inferior vena cava.A shape-memory alloy atrial septal occluder with a waist diameter of 20 mm was placed successfully and located correctly.TTE showed that the double disk unfolded well and that the clamping of the atrial septum was smooth.Immediately after the disc was revealed,electrocardiograph monitoring showed that the ST interval of the inferior leads was prolonged,the P waves and QRS waves were separated,a junctional escape rhythm maintained the heart rate,and the blood pressure began to decrease.After removing the occluder,the elevation in the ST segment returned to normal immediately,and the sinus rhythm returned to average approximately 10 min later.After consulting the patient’s family,we finally decided to withdraw from the operation.CONCLUSION Compression of the small coronary artery,which provides an alternative blood supply to the atrioventricular nodule during the operation,leads to the emergence of a complete atrioventricular block.

Surgical Experience of a Partial Atrioventricular Septal Defect in an Elderly Patient: A Case Report

The following paper describes patch closure (bovine pericardial sheet) of an ostium primum atrial septal defect and mitral valve repair (sutured mitral valve cleft + autologous pericardial annuloplasty + Alfieri’s method) in a 50-year-old man. He had been perfectly well until he was brought to the emergency room because of acute heart failure. Chest radiography revealed right-side heart enlargement. Electrocardiography indicated atrial flutter. Echocardiography revealed a large ostium primum atrial septal defect and moderate mitral regurgitation. The pulmonary-to-systemic blood flow ratio was 3.24. First, cardiologists performed catheter ablation of the cavotricuspid isthmus for atrial flutter. We performed patch closure of an ostium primum atrial septal defect and mitral valve repair after the patient’s heart failure was under control. The patient was discharged 13 days postoperatively in a satisfactory condition without any critical complications.

Ellis-Van-Creveld Syndrome and Congenital Cardiac Anomaly: Common Atrium with Atrioventricular Canal Septal Defect

Background: Children presenting with physical features of chondro-ectodermal dysplasia (Ellis-Van Creveld syndrome) such as skeletal and joint abnormalities often have concomitant congenital cardiac anomalies. Presence of cardiorespiratory symptoms in children with Ellis-Van Craved syndrome warrants a thorough cardiologic evaluation to recognize and treat underlying congenital heart anomaly. Aim: A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and accomplish successful repair of the underlying cardiac lesion to reduce the cardiac related morbidity and improve the patient survival. Case Presentation: Ten years old boy with chondroectodermal dysplasia (dental anomalies, genu valgum and other skeletal abnormalities) presented with dyspnea and cyanosis. Cardiac evaluation by 2D echo revealed an atrioventricular (AV) canal septal defect with AV valve regurgitation and a common atrium. Angiocardiography showed a goose neck deformity of the left ventricular outflow tract. The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO2 of 0.7) and O2 saturation in the common atrium was 0.7. The pulmonary venous connections to the common atrium were anomalous. Atriotomy on cardiopulmonary bypass and on a cardioplegic arrest discerned a partial AV canal septal defect with a common bridging leaflet, clefts in septal leaflets of tricuspid and mitral vlalves, an incompletely closed interventricular communication, and a common atrium with highly anomalous pulmonary venous insertions well anterior (8 cm) to vena caval orifices. Intracardiac repair was performed with two patches of Goertex to partition the common atrium into the pulmonary and systemic venous chambers after repair of the partial AV canal septal defect. Patient required only a temporary afterload reduction with enalapril;otherwise patient had an uneventful postoperative course. At a 2-year follow-up, the child was well without AV valve regurgitation and had normal biventricular function. Conclusion: A child with Ellis-Van-Creveld syndrome with skeletal abnormalities and dental anomalies had manifested with cardio-respiratory symptoms. Preoperative cardiac and intraoperative evaluation showed a common atrium with severely anomalous pulmonary venous connection and partial AV canal septal defect. Successful biventricular repair was accomplished by repairing the partial AV canal septal defect and partitioning the common atrium into left and right atrium by a complex atrial routing technique with two patches of Gore-Tex. On a follow-up at 2 years, the patient had adequate biventricular function without AV valve regurgitation. 114264

Natural History of a Transitional Atrioventricular Septal Defect in an Adult Patient with Down Syndrome

Background: Atrioventricular septal defects (AVSD) are caused by a lack of development in atrioventricular endocardial cushions. Its spectrum varies from partial to transitional, or intermediate forms with a common AV valve and 2 orifices, or even the complete form. Aim: The aim of this study is to present a case of a woman in the fifth decade of life with Down syndrome and a transitional AVSD, diagnosed with echocardiography. Case presentation: This is a patient in the fifth decade of life with Down syndrome, transitional AVSD, and severe pulmonary hypertension. Her vital signs were normal, oxygen saturation of 89% on room air. Auscultation revealed pronounced pulmonary component of the second heart sound, diastolic murmur in second left intercostal space, and moderate holosystolic heart murmur that radiated to axilla. The diagnosis was made with a transthoracic echocardiogram. The patient is receiving only medical treatment and since her last consult she has New York Heart Association (NYHA) functional class II. Conclusion: This case demonstrates the natural history of a patient in her fifth decade of life with down syndrome and complex congenital heart disease, despite having received only medical treatment, she is in functional class II. Echocardiography is the noninvasive technique of choice in the diagnosis and follow-up of patients with congenital heart diseases, as seen in this case.

房室间隔缺损胎儿的超声心动图定量参数分析

目的通过对胎儿超声心动图(FE)结构化数据的定量分析寻找产前诊断房室间隔缺损(AVSD)的关键筛查参数。方法回顾性收集首都医科大学附属北京安贞医院胎儿心脏病母胎医学会诊中心数据库2016年1月至2021年12月诊断的370例正常胎儿和96例AVSD胎儿的112个FE结构化参数。根据受试者工作特征曲线下面积(AUC),筛选出筛查效果最好的前6位参数并对6个参数的产前筛查效能进行评估。结果筛选出的6个参数均从FE的舒张末期四腔心切面获得;AUC最大的前6位参数依次为:舒张末期房室长度比值、舒张末期房室长度比值的Q值、收缩末期房室长度比值的Q值、收缩末期房室长度比值、舒张末期心室长度的Q值和舒张末期心室长度,AUC分别为0.92、0.92、0.86、0.84、0.84和0.81;在假阳性率为20%的范围内,6个参数对于AVSD产前筛查的敏感度依次为82%、82%、75%、67%、65%和71%。结论舒张末期房室长度比值及其Q值可以作为产前筛查AVSD的关键筛查参数,其中,舒张末期房室长度比值获取更容易且计算更方便。

Congenital heart“Challenges”in Down syndrome

In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old patient with Down syndrome(DS)and congenital heart disease(CHD)associated with pulmonary arterial hypertension.In this distinct population,the Authors underscore the need for early diagnosis and management as well as the need of a multidisciplinary approach for decision making.It seems that the occurrence of CHD in patients with DS adds layers of complexity to their clinical management.This editorial aims to provide a comprehensive overview of the intricate interplay between DS and congenital heart disorders,offering insights into the nuanced diagnostic and therapeutic considerations for physicians.

高原地区婴幼儿完全型房室间隔缺损的外科处理策略及随访结果

目的 评估高原地区婴幼儿合并肺动脉高压(PAH)完全型房室间隔缺损(cAVSD)患儿的外科处理策略及随访结果。方法 选择2009年12月~2021年12月期间28例2岁以下cAVSD合并PAH的患儿进行一期矫治,其中男15例,女13例,年龄(2.5~21.0)月,体质量(2.5~11.0)kg,其中合并中度及以上肺动脉高压患者15例,设为A组。合并轻度及正常肺动脉压患者13例,设为B组。对两组患者的ICU住院时间、呼吸机带管时间、主动脉阻闭时间、整体住院时间、死亡率等指标进行对比,并进行随访。术中采用改良单片法3例,双片法25例,术中直接缝合左侧房室瓣瓣裂孔17例,裂孔补片加宽及腱索移植1例,行左房室瓣后瓣瓣环成形3例。结果 两组患儿的年龄、体质量、Rastelli分型、唐氏综合征、共同瓣返流和室间隔缺损大小数据值均无统计学差异。与B组比较,A组患儿呼吸机带管时间明显变长(P<0.05),其他指标如ICU住院时间、整体住院时间、主动脉阻闭时间和病死率均无明显差异。与A组比较,B组轻度左侧房室瓣返流比例较低(P<0.05),其余项目室间隔残余分流、中重度左侧房室瓣返流、右侧房室瓣返流、完全性房室传导阻滞与术后早期死亡均无明显差异。术后随访所有患儿的随访时间为(8±3)年,心脏超声心动图随访统计结果为:二尖瓣关闭不全轻度18例、中度5例、重度2例,三尖瓣轻度关闭不全19例、中度4例、重度2例,2例重度二尖瓣关闭不全患者均接受再次瓣膜修复/置换术,全组存活病例均无左室流出道狭窄,三尖瓣重度反流1例、中度反流2例。A组的三例死亡病例根据患者在监护室最后一次超声评估结果为二尖瓣重度反流1例、中度反流1例、轻度反流1例。结论 高原地区的婴幼儿完全性房室间隔缺损患儿,其肺血管病变早、肺动脉高压进展快,应尽早进行手术治疗,手术成功的关键是避免三度房室传导阻滞及瓣膜成型的确切效果,左侧房室瓣关闭不全的手术修复效果是导致术后死亡及中远期再次手术干预的重要原因。

Late recurrent high degree atrioventricular block after percutaneous closure of a perimembranous ventricular septal defect

High degree atrioventricular block （HDAVB） is a serious complication of transcatheter closure of a perimembranous ventricular septal defect （PMVSD）. We report one patient who developed transient HDAVB seven days after transcathter closure of PMVSD and had recurrent HDAVB 42 months after the procedure.

房室间隔缺损的形态学诊断──二维超声心动图与心血管造影、手术的比较

为了评估二维超声心动图（２ＤＥ）对房室间隔缺损（ＡＶＳＤ）的诊断和手术方法选择价值，对８７例ＡＶＳＤ的２ＤＥ、心血管造影及手术结果进行回顾对照研究。病例年龄自３个月至１１岁（平均３．９岁）。部分型ＡＶＳＤ４６例，过渡型１２例，完全型２９例（Ａ型２１例，Ｂ型４例，Ｃ型４例）。２ＤＥ诊断与手术诊断符合率在部分型ＡＶＳＤ中为９７％，在过渡型及完全型ＡＶＳＤ中为８４％。２ＤＥ诊断与手术诊断不符者大多为过渡型与Ａ型之间的交错。２ＤＥ诊断准确率优于心血管造影检查。结果表明，２ＤＥ检查在心尖、剑突下切面中能够清楚显示ＡＶＳＤ的形态改变，获得可靠的形态学诊断。

完全性房室通道的外科治疗

目的总结94例完全性房室通道(complete atrioventricular canal defect,CAVCD)的外科治疗经验.方法一期手术矫治CAVCD 94例,房、室间隔缺损修补用双片法(涤纶片+心包片)65例,单片法29例,术中行房室瓣成形,并同期修补合并畸形.结果全组死亡10例(10.6%),其中＜6个月者4例.4例术后出现二尖瓣中至大量反流,心肺功能衰竭死亡,3例因肺动脉高压危象死亡,3例分别死于低心排血量、脑并发症和气胸.随访84例,随访时间3～6个月,超声心动图复查二尖瓣轻度反流18例,轻至中度反流12例.结论严重二尖瓣关闭不全和肺动脉高压危象是CAVCD矫治术后主要的死亡原因,早期手术矫治和确切的房室瓣成形可获得较好的手术效果,术中常规使用食管超声心动图对提高手术疗效具有重要的作用.

部分型房室间隔缺损的外科治疗

目的:总结60例部分型房室间隔缺损的外科治疗经验。方法:回顾性分析经手术治疗的60例部分型房室间隔缺损患者的临床资料。二尖瓣前瓣分裂的处理,58例缝合二尖瓣裂隙,2例行机械二尖瓣置换;原发孔房间隔缺损的修补,53例采用心包片,7例采用绦纶补片;35例采用Kirklin法将冠状静脉窦口隔入左房,25例采用McGoon法将冠状静脉窦口隔入右房;同期处理合并的畸形。结果:共死亡2例,均死于低心排出量综合征,死亡率3.3%(2/60);采用Kirklin法和McGoon法手术的患者术后完全性房室传导阻滞发生率分别为8.00%(2/35),6.06%(2/25),2组比较差异无统计学意义(P>0.05);随访1月至5年,无晚期死亡,除1例3个月后复查出现二尖瓣中度返流外,余患者未出现轻度以上二尖瓣和/或三尖瓣狭窄或关闭不全,心功能均得到不同程度改善。结论:针对部分型房室间隔缺损患者,合理的手术设计、术中细致操作、避免损伤传导组织以及加强围手术期管理均可取得的良好的近、中期疗效。

膜周部室间隔缺损经导管封堵术后并发迟发性完全性房室传导阻滞2例报告并文献复习

目的提高对膜周部室间隔缺损(PmVSD)经导管封堵术后,并发迟发性完全性房室传导阻滞(CAVB)的认识。方法回顾2例PmVSD经导管封堵术后合并迟发性CAVB的术中和术后资料,结合文献进行分析。结果例1患儿术中、术后无明确的危险因素,术后6个月出现持续性CAVB伴不完全性右束支传导阻滞,随访5年至今无症状。例2患儿术中出现一过性完全性左束支传导阻滞(CLBBB),术后5 d出现CAVB伴CLBBB,经激素等成功逆转后多次随访,ECG示Ⅰ度房室传导阻滞伴完全性右束支传导阻滞或CLBBB;术后9个月出现一过性晕厥,ECG证实间歇性CAVB伴CLBBB;目前已随访15个月,偶有晕厥发作。2例患儿家长均拒绝植入永久起搏器。结论 PmVSD经导管封堵术后并发迟发性CAVB难以预测,不论有无症状都必须长期随访。充分认识迟发性CAVB的自然史、发生率、发生机制、危险因素和防治对于尽可能减少此致命性并发症的发生是必要的。

牛心包补片修复心脏间隔缺损的应用

背景:心脏涤纶补片因其质地薄、质量轻、生物相容性好等优点常规用于心脏手术,但最近几年发现,用心脏涤纶补片对心室间隔缺损修补后,一旦发生术后残余漏,较易引起溶血、细菌或真菌感染。目的:探讨应用牛心包补片修复心脏间隔缺损的疗效。方法:采用戊二醛固定的牛心包补片修补152例心脏间隔缺损患者,其中房间隔缺损56例,室间隔缺损78例,部分房室间隔缺损18例。术后复查心电图、胸片、心脏超声,观察其术后早期病情、血流动力学和心脏功能。结果与结论:152例患者均完成修补手术,术后无早期死亡,患者随访2-6个月,牛心包补片在使用过程中及术后早期均未发现漏血、溶血、血栓、感染、排斥反应等并发症。1例患者因合并肺动脉高压,术后第1天出现高血压危象,经过抢救后好转。复查心电图、胸片、心脏超声可见心脏间隔缺损修补完全,无残余分流;肺动脉压力明显下降;活瓣已关闭无分流;心脏功能正常。证实牛心包补片可以有效修复心脏间隔缺损,改善患者的血流动力学和心功能,近期效果满意,是一种良好的心脏间隔缺损修补材料。

CRELD1基因在汉族先天性房室隔缺损患儿中的突变分析

目的探讨CRELD1基因突变在房室间隔缺损(AVSD)发病机制中的作用。方法收集67例汉族AVSD患儿(包括合并21三体综合征10例)血液标本,PCR方法扩增CRELD1基因全部11个外显子编码序列后,对扩增产物进行测序,然后与GenBank人CRELD1基因编码序列进行比对,并以100名健康汉族人群为对照。结果在67例患儿中检出1例错义突变(碱基变化为G973A,氨基酸改变为E325K),为部分型AVSD伴21三体综合征的男性患儿;7例患儿发现基因多态性(rs 2302787,碱基变化C383G,氨基酸改变P128R);24例患儿发现基因多态性(rs 3774207,碱基变化C1119T,氨基酸编码H373H)。结论合并21三体综合征的AVSD患儿中,CRELD1基因突变的检出率(1/10例,10%)高于非综合征、散发的AVSD人群(0/57例,0%),但这一结论尚有待于更多的病例数证实;AVSD患儿中,CRELD1基因突变的检出率不高,提示AVSD可能是一个多基因的遗传性疾病。

房间隔缺损经导管介入封堵术发生房室传导阻滞的分析

目的:分析房间隔缺损(ASD)经导管介入封堵术发生房室传导阻滞(AVB)的情况。方法:521(男189,女332)例,年龄1.0～65(27±19)岁住进我科行ASD封堵术的患者,对封堵术后发生AVB的患者进行分析。结果:13(男5,女8)例(2.5%)封堵术后发生AVB,年龄4～44(23±12)岁。其中2例在发生AVB后撤出封堵器,其余11例均成功植入封堵器。3例为Ⅰ°AVB,4例Ⅱ°ⅠAVB,4例Ⅱ°ⅡAVB,2例Ⅲ°AVB。7例植入的封堵器在38mm以上;3例4～8岁幼儿,植入的封堵器在22～28mm;1例为15岁青少年,所植入封堵器为34mm;另2例为成人,植入的封堵器分别为30mm和34mm。所有患者AVB均恢复正常。结论:ASD介入封堵术有发生AVB的可能,AVB多见于植入大型号封堵器的患者,多数AVB是能够恢复的。

小儿膜周部室间隔缺损介入治疗发生高度房室传导阻滞的特点及其处理(附9例报道)

目的探讨经导管介入治疗小儿膜周部室间隔缺损(VSD)发生高度房室传导阻滞(AVB)的特点及其处理。方法选择我科年龄小于12岁VSD患者,经导管介入治疗发生高度AVB的患儿,共计9(男4,女5)例,连续观察所有患儿封堵术前以及发生高度AVB后ECG的变化及其处理。结果①术中发生高度AVB5例,分别在AVB后10min～46h恢复正常窦性心律。其中3例为持续Ⅲ度AVB,经处理后2例转为持续Ⅱ度Ⅱ型,1例仍为持续Ⅲ度AVB;间歇性Ⅱ度Ⅱ型和Ⅲ度AVB各1例。②4例在术后12h～10d出现AVB,3例在AVB后1～12d恢复;1例未恢复,给予永久起搏器治疗。其中3例为间歇性高度或Ⅲ度AVB,1例为持续性Ⅲ度AVB。③术后AVB恢复的3例,在高度AVB时,ECG均表现为完全性右束支传导阻滞+左前分支传导阻滞。AVB后3～5d,ECG电轴左偏进一步加重,以后电轴左偏逐渐减轻,左前分支阻滞逐渐消失,至恢复时ECG仅遗留右束支传导阻滞。结论小儿膜周部VSD介入治疗有发生高度AVB的危险。对于高危患者,应尽早治疗。

婴幼儿完全性房室间隔缺损的外科治疗

目的：研究婴幼儿完全性房室间隔缺损（CAVSD）的外科治疗效果,探讨不同手术方法的特点.方法：2005年1月至2011年12月,CAVSD患儿142例,手术年龄2个月～6岁,平均（12.5）个月,中位年龄7个月,年龄＜6个月65例,6～12个月43例,12个月以上34例.体质量3.5 ～ 19 kg,平均（7.1±3.3）kg.采用改良单片法105例（73.9％）,37例室间隔缺损高度超过缺损宽度50％以上的患儿采用双片法（26.1％）.结果：全组手术早期病死率4.9％ （7/142）,分别死于肺动脉高压危象2例,低心排出量综合征（低心排）3例,败血症2例.不同手术年龄的三组病例手术病死率分别为4.6％、4.7％和5.9％,差异无统计学意义.按改良单片法和双片法分组手术病死率分别为3.8％ （4/105）和8.1％（3/37）,差异有统计学意义（P＜0.05）.体外循环时间和主动脉阻断时间改良单片法组明显低于双片法组,差异有统计学意义（P＜0.05）,而在不同年龄组间差异无统计学意义.而术后二尖瓣反流在各组差异均无统计学意义.结论：CAVSD应该早期诊断和早期手术治疗,3～6个月月龄是适宜的手术时机,改良单片法可适用于大部分病例,有效简化手术操作缩短手术时间,瓣膜修复效果于双片法相仿.

右腋下小切口部分型心内膜垫缺损修补术——6例报告

目的 报道经右侧腋下小切口 ,修补部分型心内膜垫缺损的手术方法及结果。 方法 1997年 11月至 2 0 0 0年 1月 ,6例部分型心内膜垫缺损患者经右侧腋下小切口 ,完成了二尖瓣成形 (心脏跳动下观察二尖瓣返流情况及成形效果 )加Ⅰ孔房缺修补术。其中 1例解剖性单心房 ,1例过渡型心内膜垫缺损。 6例均有二尖瓣前叶裂 ,其中 3例合并二尖瓣中度关闭不全 ,1例重度关闭不全 ,1例三尖瓣中度关闭不全。 结果 切开长度 (8 3± 1 3)cm ,循环阻断时间 (32 8± 8 3)分钟 ,体外循环时间 (6 6 0± 9 0 )分钟。 1例术后二尖瓣仍有少 -中量返流 ,其余患者二尖瓣关闭良好 ,无房水平残余分流。 6例均顺利康复出院。术后随访 (1 1± 0 7)年 ,患者心功能良好 ,无死亡及并发症。 结论 经右侧腋下小切口行部分型心内膜垫缺损修补术 ,是一种安全、微创的手术方法。其中心脏跳动下观察二尖瓣返流情况 ,可为瓣膜成形提供可靠依据。