Permanent chronotropic impairment after closure of atrial or ventricular septal defect

Objectives. Atrial and ventricular septal defects are commonly closed surgically with low rates of post-operative morbidity, and the long-term outcome has widely been described as benign. Nevertheless, there is an increasing understanding of late morbidity, and the possibility of postoperative chronotropic impairment is continuously questioned. Furthermore, potentially abnormal chronotropic responses may be associated with the recently demonstrated overrepresentation of late arrhythmias. We conducted this review to methodically describe the potential effects of surgical defect closure on the chronotropic response to exercise. Methods. We performed a search protocol based on the 'Preferred Reporting Items for Systematic Reviews and Meta- Analyses' (PRISMA) guidelines. A data collection form was specifically developed and data were extracted from the included studies by a primary reviewer and crosschecked by a secondary reviewer. Results. A search of electronic databases revealed 16 publications, which consistently reported an abnormal heart rate responses to exercise after surgical closure. In contrast, these and other studies convincingly showed normal chronotropic response in un-operated patients as well as after percutaneous closure. Potential mechanisms include disturbances in the sinoatrial or atrioventricular conduction system and denervation in the proximity of the aorta during the surgical procedure. Discussion. ASD and VSD patients have abnormal heart rate responses to exercise after surgical closure, which indicates a need of change in the preoperative information given to these patients and their parents before surgical defect closure. Although there may not be any alternatives to surgery, patients should still be informed about potential long-term consequences, including the risk of chronotropic incompetence. Moreover, this review suggests that ‘specialized’, long-term follow up may be indicated.     

30岁以上继发孔房间隔缺损患者的外科治疗

目的　总结 30岁以上继发孔房间隔缺损 (房缺 )患者的外科治疗经验。方法　从 1978年 12月至 2 0 0 2年 12月共手术治疗 30岁以上的继发孔房缺患者 4 6 9例 ,其中男 14 4例、女 32 5例 ,年龄 30～ 6 8岁 ,平均 38 6岁 ,肺动脉高压 10 5例 ,心律失常 4 5 8例。所有患者均行房缺修复术 ,补片修复缺损者 35 8例 (其中自体心包补片 30 5例 )。 2 5例术后肺动脉高压患者持续吸入低浓度 ( 6× 10 -6)一氧化氮。 5例房颤患者同期行右侧迷宫术。结果　死亡 3例 ( 0 6 % )。术后心律失常 180例 ,左心功能不全 2 7例 ,低心排出量综合征 2 8例 ,再次开胸止血 12例 ,空气栓塞 1例。 2 5例术后持续吸入低浓度一氧化氮患者平均肺动脉压下降 2 8 5 %。 5例同期行右侧迷宫术患者 ,术后房颤消失。随访35 2例 ,随访时间 3个月～ 2 0年 (平均 5 6年 ) ,心功能Ⅰ～Ⅱ级者 2 9例 ,其余患者心功能均正常。结论　成人房缺应尽早手术 ;采用补片修补时自体心包为首选 ;应用吸入一氧化氮疗法治疗术后肺动脉高压效果良好 ;合并房颤的房缺修补术应同期行迷宫手术 ;术中、术后注意防治心律失常及进行左心功能的保护和支持     

ASD and VSD Flow Dynamics and Anesthetic Management

Atrial septal defects and ventricular septal defects are often encountered in patients presenting for treatment under anesthesia. The flow mechanisms for both defects are predominantly left to right shunting prior to long-term maladaptive changes that may occur. Close examination of the shunt dynamics demonstrates a minor right to left shunt that occurs as well. The article discusses these dynamics and the impact on an anesthetic plan.Key Words: Atrial septal defect, Ventricular septal defect, Shunt, Flow dynamicsAtrial septal defects (ASDs) and ventricular septal defects (VSDs) are common congenital heart defects (CHDs) likely to be encountered by the anesthesia provider. The flow dynamics of such defects are discussed in this article with an emphasis on how they may impact anesthetic management. Epidemiological data and long-term prognosis of these defects are also discussed to assist in perioperative decision making.     

Surgical closure of an apical ventricular septal defect through a septal ventriculotomy

Here we report a 3-year and 7-month-old boy with a muscular ventricular septal defect at the apex. At age 7 months, pulmonary artery banding was performed. Surgical treatment consisted of double patch closure via septal ventriculotomy and a main pulmonary artery plasty by an end-to-end anastomosis with cardiopulmonary bypass. There was no residual leak of the ventricular septal defect or ventricular aneurysm and the postoperative course was good. This technique could be a useful surgical option for repair of apical muscular ventricular septal defects.     

Iatrogenic ventricular septal defect: A rare complication of surgical reconstruction of mitral paravalvular dehiscence

IntroductionIatrogenic ventricular septal defect is a rare complication after the surgical replacement of cardiac valves. Small defects may have no hemodynamic significance or remain unremarked at the end of the surgical procedure. Understanding of the valvular anatomy alone is not always enough to avoid such complications, especially in the hands of young surgeons.Presentation of caseWe present a case of iatrogenic ventricular septal defect that developed early after the surgical closure of a hemodynamically significant mitral paravalvular leak. Although the patient's critical state did not allow surgical intervention and he died, we think the lessons drawn from this case could be helpful to avoid such horrible complications in the future.DiscussionThis case documents a rare disastrous complication after imperfect surgical closure of a mitral paravalvular leak. Despite the unfortunate end, in reporting this case we try to direct the light to the possible mechanisms that led to the development of this injury focusing on the embryological and anatomical background.ConclusionUnderstanding the anatomical and embryological structure of the cardiac fibrotic skeleton should keep cardiac surgeons more vigilent in detecting iatrogenic ventricle septal defects before the development of a devastating hemodynamic state.     

小婴儿巨大室间隔缺损的外科治疗

目的 报告３９例出生６个月以内小婴儿巨大型空间隔缺损的外科治疗经验。方法 体外循环下除４例经肺动脉切口外其余均经右房切口,行补片缝合修补空间隔缺损。其中合并动脉导管未闭５例、房间隔水平分流１２例和右室流出道狭窄２例均同期矫治。结果 ３９例病儿均痊愈出院。结论 巨大型空间隔缺损的小婴儿出现顽固性心力衰竭、药物治疗无明显效果或生长发育停滞及肺动脉高压时即应手术。尽可能完善的围术期处理是降低病死率的重要     

Hybrid procedure with cardiopulmonary bypass for muscular ventricular septal defects in children

Objective: To summarize the technique and clinical experience of the hybrid procedure with cardiopulmonary bypass in children with muscular ventricular septal defect (mVSD). Methods: From January 2006 to June 2010, 45 cases of mVSD underwent hybrid procedures with cardiopulmonary bypass (CPB) under the guidance of transesophageal echocardiography. mVSDs were closed with devices under direct vision in the 45 cases. Fourteen patients had another lesion that required surgical repair. Large membranous VSDs were closed with a pericardial patch after the initiation of CPB in 38 cases. Results: Out of the 45 cases, 42 had only one occluder and three had two occluders. The size of the device for mVSD closure ranged from 3 to 8 mm. All cases recovered smoothly after treatment without residual shunting, aortic or mitral valve regurgitation, or restriction of surrounding structures. All the children survived the operation with no late deaths during the follow-up. Conclusion: The hybrid procedure is safe and effective for the closure of congenital heart defects in children.     

产前超声筛查中孕期胎儿心脏畸形对诊断染色体异常价值的Meta分析

目的采用Meta分析探讨中孕期胎儿超声心动图检查发现孤立性室间隔缺损、除外孤立性室间缺损的其他心脏及大血管畸形对染色体异常的产前筛查价值。方法检索Cochrane Library、PubMed、EMBASE、SCI、万方、维普、中国知网数据库中的中英文文献,按照纳入和排除标准筛选文献并进行偏倚风险评价,提取相关资料,检验异质性并选择相应的效应模型合并效应量,采用Meta-Disc 1.4软件,计算汇总敏感度、特异度及95%CI,绘制汇总受试者工作特征曲线,计算曲线下面积,对异质性文献进行敏感度分析。结果共纳入12篇文献,6篇文献分析了孤立性室间隔缺损对染色体异常的产前筛查价值,8篇文献分析了其他心脏及大血管畸形对染色体异常的产前筛查价值。采用随机效应模型合并效应量,两组汇总敏感度、特异度分别为32%、99%及29%、99%,SROC AUC分别为0.833 7及0.884 6,两组的汇总敏感度差异无统计学意义(P0.05)。结论中孕期超声心动图显示胎儿心脏畸形对染色体异常有一定的产前筛查价值,胎儿孤立性室间隔缺损是染色体异常的危险因素,存在其他心脏及大血管畸形未使染色体异常的风险增加。     

婴幼儿室间隔缺损修补术后残余分流的自发愈合

目的探讨婴幼儿室间隔缺损（VSD）修补术后残余分流的预后。方法连续选取2008年l～6月上海交通大学医学院附属上海儿童医学中心407例（男255例、女152例）婴幼儿VSD患者，1岁以下273例，1～2岁88例，2～3岁46例，均采用戊二醛固定的自体心包补片连续缝合法进行修补。结果407例患者中29例出现小残余分流（≤0．4cm），残余分流率在1岁以下、1～2岁、2～3岁各年龄段之间差异无统计学意义（Y2=0．054，P=0．973）。随访29例，随访1．5个月～2年，26例患者残余分流愈合，其中23例为膜周型，残余分流位于VSD隔瓣下或VSD上、下缘，3例为肺动脉瓣下型；有3例残余分流患者随访中始终不愈合，残余分流无明显变化，均位于VSD上缘，无需再次手术。残余分流率在各类型VSD之间差异无统计学意义（2=1．035，P=0．596）。结论婴幼儿VSD修补术后小于0．4cm的残余分流大部分可以自发愈合。     

主-肺动脉间隔缺损的外科治疗

目的探讨主肺动脉间隔缺损的外科治疗。方法１９８３年１月至１９９６年１２月间,手术治疗主肺动脉间隔缺损患者５例,其中男２例,女３例;年龄６～３４岁（平均１７４岁）。按Ｍｏｒｉ分型：Ⅰ型２例,Ⅱ型２例,Ⅲ型１例。缺损直径１０～３０ｃｍ。手术均在全身麻醉、中度低温、体外循环下进行。３例经肺动脉切口,２例经主动脉切口,以绦纶毡片修补。结果５例均痊愈出院,术后随访１０个月～１２年,情况良好。结论本病确诊后应尽早手术,手术方法宜采用体外循环下修补,主动脉切口优于肺动脉切口,以补片修补可防止大血管腔狭窄及术后再通     

婴儿期室间隔缺损外科治疗

报告婴儿期室间隔缺损修补术８１例，手术死亡率３．７０％，术后并发症发生率１８．５２％，无后遗症发生。结论：室间隔缺损婴儿发生难治性心衰者应早期手术，围术期的心肌保护和呼吸管理是手术成功的关键。     

Early and late results of total correction of congenital cardiac anomalies in infancy

OBJECTIVE: We evaluated long-term results of surgical correction of congenital cardiac anomalies in infancy. METHODS: We reviewed cases of 856 patients who underwent complete correction of major cardiac anomalies in the first year of life during last 24 years at our institution, and analyzed results. Surgery involved ventricular septal defect (n = 453), tetralogy of Fallot (n = 92), atrioventricular canal defect (n = 93), and complete transposition of the great arteries (n = 218). RESULTS: Operative mortality was 2.2% in ventricular septal defect, 0% in tetralogy of Fallot, 8.6% in atrioventricular canal defect, and 4.1% in complete transposition of the great arteries. Freedom from reoperation at 20 years was 96.5 +/- 2.0% and actuarial survival was 94.2 +/- 1.3% in ventricular septal defect. Freedom from reoperation at 15 years was 90.5 +/- 3.9% in tetralogy of Fallot and 86.6 +/- 4.4% in atrioventricular canal defect. Actuarial survival at 15 years was 97.8 +/- 1.6% in tetralogy of Fallot, 85.7 +/- 3.7% in atrioventricular canal defect, and 89.9 +/- 2.2% in complete transposition of the great arteries. Actuarial survival in complete transposition of the great arteries was significantly better in arterial than in atrial switch operations. CONCLUSIONS: Total correction of ventricular septal defect, tetralogy of Fallot, atrioventricular canal defect, and complete transposition of the great arteries in infancy was conducted safely, but the incidence of reoperation in late follow-up must be reduced.     

房间隔切面叠加彩色多普勒血流显像在房间隔缺损显像中的应用

目的探讨采用房间隔切面叠加彩色多普勒血流显像显示房间隔缺损的可行性和临床意义。方法对常规切面超声确诊为继发孔型ASD109例,采用M型超声心动图的取样线引导获得房间隔的切面后,叠加彩色多普勒血流显像显示房间隔缺损口分流束的截面彩流图,进而观察房间隔缺损口的形态及缺损口残缘与周边结构的关系,并对常规切面、房间隔切面和房间隔切面叠加CDFI三种成像方式进行对比分析。结果本组60.6%的病例采用房间隔切面可获得房间隔缺损口的二维声像图,叠加彩色多普勒血流显像后90.8%的病例可获得房间隔缺损口分流束的截面图,与常规切面对比,在显示ASD形态方面有非常显著性差异(P<0.001),在确定ASD类型和测量ASD残缘与周边结构距离所需的时间方面均有显著性差异(P<0.05)。结论房间隔切面叠加彩色多普勒血流显像可获得近似于房间隔侧面观的图像,可显示房间隔缺损口的形态,能直观地观察缺损口残缘与周边结构的关系并能准确快捷测量缺损口残缘与周边结构的距离,为房间隔缺损诊断分型和介入封堵治疗术前筛选提供新的影像信息,值得临床应用。     

A novel BMPR2 gene mutation associated with exercise-induced pulmonary hypertension in septal defects

Abstract

Objective. Our study aimed to investigate the relationship between exercise-induced pulmonary arterial hypertension and genetic changes related to the transforming growth factor-β (TGF-β) signalling pathway in patients with cardiac septal defects. Design. In a population-based group of 44 patients (age 13–25 years) with either isolated ventricular septal defect (n=27) or isolated atrial septal defect (n=17), right ventricular systolic pressure response to submaximal exercise was studied by echocardiography and classified as normal (≤45 mmHg), borderline (45–50 mmHg) or abnormal (>50 mmHg). Three genes related to TGF-β, bone morphogenetic protein receptor type 2 (BMPR2), activin receptor-like kinase 1 (ALK1) and endoglin (ENG), were analyzed by DNA sequencing (only BMPR2) and multiplex ligand-dependent probe amplification (BMPR2, ALK1 and ENG). Results. Pressure response was borderline in five and abnormal in nine patients. Five patients showed mutations in exon 12 of the bone morphogenetic protein receptor type 2 gene. The previously described polymorphism S775N (c. 2324, G > A) was found in three patients with normal pressure response. The mutation Y589C (c. 1766, A > G), which has not been described previously, was found in two of 14 patients with borderline/abnormal pressure response. Conclusion. Genetic changes in the BMPR2 gene may be overrepresented in patients with cardiac septal defects and exercise-induced pulmonary hypertension.     

完全性房室间隔缺损合并法乐四联症的手术矫正

目的　报告完全性房室间隔缺损合并法乐四联症的外科治疗。　方法　 6例患者 ,年龄 3～ 9岁 ,均施行手术根治 ,经右房 右室切口修补VSD ,房室间隔缺损采用两块补片技术修复 ,左房室瓣裂隙的修复均采用间断缝合 ,右室流出道均用带单瓣的补片跨瓣加宽。　结果　全组 6例术后出现低心排综合征、多器官衰竭、灌注肺并发症各 1例 ,早期死亡 1例为多器官衰竭者 ,5例长期存活。术后随访 6个月～ 5 5年 ,1例有轻度左房室瓣关闭不全。心功能Ⅰ级 4例 ,Ⅱ级 1例。　结论　完全性房室间隔缺损合并法乐四联症可应用 2块补片法行手术根治 ,左房室瓣瓣裂的常规修补可降低其关闭不全的发生率 ,大部分患者术后心功能可获得良好的改善。     

酚妥拉明治疗室间隔缺损合并肺动脉高压的临床意义

目的　探讨酚妥拉明对治疗室间隔缺损合并重度肺动脉高压的临床意义。　方法　 33例室间隔缺损合并重度肺动脉高压患者应用彩色多普勒超声心动图检测静脉注射酚妥拉明后肺动脉收缩压 (PASP)的变化。根据静脉注射酚妥拉明后肺动脉收缩压下降程度不同分为 A组和 B组。 A组 :2 1例 ,肺动脉收缩压下降≥ 2 .6 7k Pa(2 0mm Hg) ;B组 :12例 ,肺动脉收缩压下降 <2 .6 7k Pa(2 0 mm Hg)。比较两组术后低心排血量综合征、心律失常、死亡例数等。　结果　两组肺动脉收缩压均下降 ,A组肺动脉收缩压下降明显 ,其手术疗效好于 B组。A组术后发生低心排血量综合征和心律失常较 B组低 (P<0 .0 5 )。　结论　酚妥拉明试验对室间隔缺损合并重度肺动脉高压手术治疗及预后的评估具有指导作用     

8kg以下婴儿室间隔缺损伴肺动脉高压手术治疗

为提高小婴儿室缺伴肺动脉高压的手术疗效，总结１６例８ｋｇ以下室间隔缺损（室缺）伴肺动脉高压婴儿行急诊手术修补室缺的经验。其中１４例在深低温停循环下进行，手术效果满意；术后死亡１例（６％）。作者认为，婴儿巨型室缺应尽早手术治疗为宜，深低温停循环方法尤为适合；术后强调持续保持病婴镇静及呼吸道通畅，防止肺高压危象的发生。     

Left thorax approach to repair doubly committed juxta‐arterial ventricular septal defect with Da Vinci robotic system

Robotic repair of the ventricular septal defect was performed mainly for perimembranous type via right thorax approach. Minimally invasive strategies for doubly committed juxta‐arterial ventricular septal defect were limited. Here, for the first time, we successfully repaired a doubly committed juxta‐arterial ventricular septal defect with Da Vinci robotic system via left thorax approach. The technique could provide excellent exposure of surgical field and accurate repair, with the advantage of reducing trauma and shortening the overall length of stay.     

Hypothermia During Cardiopulmonary Bypass Increases Need for Inotropic Support but Does Not Impact Inflammation in Children Undergoing Surgical Ventricular Septal Defect Closure

Minimizing the systemic inflammatory response caused by cardiopulmonary bypass is a major concern. It has been suggested that the perfusion temperature affects the inflammatory response. The aim of this prospective study was to compare the effects of moderate hypothermia (32°C) and normothermia (36°C) during cardiopulmonary bypass on markers of the inflammatory response and clinical outcomes (time on ventilator) after surgical closure of ventricular septal defects. During surgical closure of ventricular septal defects under cardiopulmonary bypass, 20 children (median age 4.9 months, range 2.3–38 months; median weight 7.2 kg, range 5.2–11.7 kg) were randomized to a perfusion temperature of either 32°C (Group 1, n = 10) or 36°C (Group 2, n = 10). The clinical data and blood samples were collected before cardiopulmonary bypass, directly after aortic cross‐clamp release, and 4 and 24 h after weaning from cardiopulmonary bypass. Time on ventilation as primary outcome did not differ between the two groups. Other clinical outcome parameters like fluid balance or length of stay in the intensive care were also similar in the two groups. Compared with Group 2, Group 1 needed significantly higher and longer inotropic support (P < 0.001). In Group 1, two infants had junctional ectopic tachycardia, and another had a pulmonary hypertensive crisis. Perfusion temperature did not influence cytokine release, organ injury, or coagulation. Cardiopulmonary bypass temperature does not influence time on ventilation or inflammatory marker release. However, in the present study, with a small patient cohort, patients operated under hypothermic bypass needed higher and longer inotropic support. The use of hypothermic cardiopulmonary bypass in infants and children should be approached with care.     

室上嵴上室间隔缺损的外科治疗

本文报道５６例室上嵴上室间隔缺损的外科治疗。男３４例，女２２例，年龄３～３９岁。１９例合并主动脉瓣叶脱垂、主动脉瓣关闭不全或佛氏窦瘤破裂。经右房切口修补室缺２例，右室漏斗切口４７例，肺动脉切９７例。直接修补缺损２６例，补片修补３０例。主动脉瓣成形术１例，主动脉瓣置换术５例。５４例全愈出院，死亡２例。作者认为室上嵴上空间隔缺损应尽早手术，以免并发主动脉瓣损害和佛氏窦瘤破裂；中度主动脉瓣关闭不全可行主动脉瓣成形术，采用肺动脉切口优于右室漏斗部切口。