先天性心脏病合并感染性心内膜炎的个体化治疗

目的：探讨先天性心脏病（CHD）合并感染性心内膜炎（IE）的个体化治疗。方法：本组病例共73例,主要表现为发热、贫血、栓塞等;血培养及赘生物培养阳性17例（23.3%）,以草绿色链球菌居多,病原体菌种呈现多样化趋势。超声心动图检查阳性率83.6%（61例）。病灶分布以主动脉瓣病变（33例）,二尖瓣病变（21）例为多。所有患者均经外科手术清除感染病灶及赘生物,并相应纠治瓣膜病变和心脏畸形。其中择期手术54例（74.0%）,急诊手术19例（26.0%）。血培养阳性病例应用大剂量敏感广谱抗生素4～8周。结果：本组无手术死亡,均经手术及抗生素治疗后临床痊愈出院。术后随访心功能改善,仅1例1年后心内膜炎复发。结论：大剂量敏感广谱抗生素治疗与积极的相应外科治疗相结合的个体化处理是治疗先天性心脏病合并感染性心内膜炎的重要手段,对提高存活率、降低病死率有着重要的意义。     

右胸前外侧与胸骨正中切口矫治先天性心脏病的临床对比研究

目的 :研究比较右胸前外侧切口和胸骨正中切口在临床上矫治先天性心脏病的优缺点。方法 :将术前超声心动图检查及手术确诊为单纯房间隔缺损或单纯室间隔缺损的患者共 15 1例 ,分成右胸前外侧切口和胸骨正中切口手术 2组 ,从术后呼吸机辅助呼吸时间、术后 2 4h引流量、体外循环时间、术后住院天数、并发症的发生率等方面进行统计分析比较。结果 :术后呼吸机辅助时间及术后住院时间 ,2组病人无明显差别 ;术后 2 4h引流量 ,右胸前外侧切口组较少 ;体外循环时间胸骨正中切口组较短 ;胸骨正中切口组切口感染发生率为 3 42 %、胸骨畸形发生率为 4 2 7% ,右胸前外侧切口组无切口感染和胸骨畸形。结论 :右胸前外侧切口可对经右房或房间沟入路完成的简单的心内直视手术 ,具有美容效果好、术后渗血少、切口感染发生率低、无胸骨畸形等突出优点。缩短住院时间不明显。是否能减少手术创伤 ,有待进一步研究     

先天性心脏病合并永存左上腔静脉的诊断及术中处理

本文报告１９０２９例先天性心脏心内直视手术中合并永存左上腔静脉（ＰＬＳＶＣ）３２６例（１．７１％）的诊断及术中处理。其中以单心房（２７．５０％）和完全性心内膜垫缺损（９．６２％）的发生率较高，而以肺动脉瓣狭窄或法乐三联症的发生率较低（０．４８％），ＰＬＳＶＣ入右房２９０例（８８．９５％），ＰＬＳＶＣ入左房３６例（１１．０４％）。术前ＰＬＳＶＣ的诊断率为３３．１２％。术后死亡５２例。作者认为，ＰＬＳＶＣ畸形在先天性心脏病中并非少见，而ＰＬＳＶＣ的许多特殊问题逐步被人们所重视。本文就ＰＬＳＶＣ的漏诊原因、术中对ＰＬＳＶＣ的不同处理方法等进行了讨论。     

先天性心脏病介入治疗与外科治疗临床比较

目的:对房间隔缺损(ASD)、室间隔缺损(VSD)的介入治疗(Amplatzer封堵器)和外科手术治疗的临床疗效进行比较。方法:2004年5月至12月住院患儿,符合单一左向右分流畸形ASD或VSD,根据治疗方法不同分为介入组和手术组;比较2组在疗效、费用、并发症、输血量及住院时间等方面的异同;通过放免法测定2组治疗前后的心钠素(ANP)水平。结果:2组手术成功率均为100%;介入组均未输血,手术组均输血治疗(P<0·01);术后住院时间介入组少于手术组(P<0·01);治疗费用介入组高于手术组(P<0·01);ANP水平、外科手术及介入治疗后均较治疗前降低。结论:单一ASD、VSD的介入治疗与手术治疗临床效果相同;介入组不需输血,术后住院时间短,但治疗费用较昂贵。     

新生儿复杂先天性心脏病外科治疗--一年经验回顾

目的回顾分析过去一年新生儿复杂先天性心脏病手术治疗结果.方法2004年5月～2005年5月我院新生儿手术临床资料.手术共完成21例,其中室间隔完整的大动脉转位(TGA/IVS)13例;合并室间隔缺损的大动脉转位(TGA/VSD)2例;右心室双出口(DORV、Taussing-Bing)1例;完全性肺静脉畸形引流(TAPVC)2例(心下型及心上型各1例);肺动脉闭锁(PAA)3例.分别行动脉调转术(ASO)16例,完全性肺静脉畸形引流矫治术2例,改良Blalock-Taussing手术3例(其中肺动脉瓣闭式扩张术2例).结果全组存活18例,死亡3例,死亡率14.3%.2例完全性大动脉转位,1例为冠状动脉壁内走行,术后死于低心排血量综合征,1例死于肺部感染.另1例为心上型完全性肺静脉畸形,术后因肺部感染导致肾功能衰竭死亡.结论准确认识新生儿复杂先天性心脏病的病理生理,是正确选择手术时机,提高围手术期治疗的关键.     

先天性心脏病合并永存左上腔静脉及右上腔静脉缺如畸形的九例报告

本文报道了在施行先天性心脏病心内直视手术中，发现永存左上腔静脉合并右上腔静脉缺如９例。其中法乐四联症、室间隔缺损各３例；房间隔缺损、右室双出口、单心室一单心房各１例。手术死亡２例。本文对永存左上腔静脉合并右上腔静脉缺如的诊断、术中左上腔静脉的处理、心律的变化特点及其预防与诊治，进行了讨论。     

1780例先天性心脏病心内畸形的外科治疗

目的 :回顾分析 1780例先天性心脏病心内畸形的外科治疗疗效 ,并比较二种心脏停搏液灌注心脏停跳和不停跳心内直视手术的疗效。方法 :1780例患者均在全麻体外循环下进行手术 ,其中采用冷晶体心脏停搏液灌注心脏停跳下手术 (冷晶体组 ) 6 4 3例 ,冷血心脏停搏液灌注心脏停跳下手术(冷血组 ) 2 31例 ,心脏不停跳手术 (不停跳组 ) 90 6例。心脏不停跳心内直视手术仍常规建立体外循环 ,置左心房引流管 ,降温至 (32± 1)℃并维持 ,仅阻断上下腔静脉 ,不阻断主动脉 ,维持灌注压在 6 0mmHg(1mmHg =0 133kPa) ,心脏跳动下进行畸形矫正 ,方法同停跳下手术。结果 :手术早期死亡 73例 (死亡率3 8% ) ,其中冷晶体组手术死亡 4 2例 (死亡率 6 5 % ) ,残余室间隔缺损 6例 (发生率 0 9% ) ;冷血组手术死亡 7例 (死亡率 3 0 % ) ;不停跳组手术死亡 2 4例 (死亡率 2 6 % ) ,其余均痊愈出院 ,随访 6个月～ 10年 ,恢复良好。结论 :先天性心脏病外科治疗有较好疗效 ,心脏不停跳心内直视手术 ,是一种可行而有效的心肌保护方法 ,并可简化操作 ,提高手术成功率。     

永存动脉干的外科治疗

永存动脉干是一种罕见的先天性心脏畸形,占先天性心脏病的0．4％～2．8％,病死率高。目前外科手术是治疗本病的惟一方法。现综述如下。     

一站式"杂交"手术治疗婴幼儿先天性心脏病

目的 总结5年来应用一站式"杂交"技术(one-stop hybrid procedure,OHP)治疗2岁内婴幼儿和新生儿先天性心脏病(congenital heart disease,CHD)的经验.方法 自2004年3月至2009年3月,共152例低龄CHD患儿接受了OHP治疗,其中行球囊扩张72例、间隔缺损封堵43例以及体肺侧支栓塞37例.所有手术都在实时超声引导或血管造影辅助下在OHP专用手术室完成,并根据病情实施相应的常规外科手术.术后随访6个月,记录其心血管不良事件发生情况.结果 球囊扩张组72例实施肺动脉瓣或主动脉缩窄球囊扩张,扩张后2例发现右心室流出道仍显著狭窄,其中1例术中,1例术后3个月接受体外循环下流出道成形术;随访期间1例因肝功能衰竭死亡.间隔缺损组43例中2例房间隔缺损、1例室间隔缺损封堵失败转为体外循环下修补,1例室间隔缺损封堵后因术前肺部感染迁延不愈死亡,其余术后随访均末发现封堵器移位.体肺侧支栓塞组37例均无死亡.结论 OHP结合了影像学和外科技术,可以减少心脏切口并在最大程度上减少或避免体外循环对于低龄CHD患儿的损害.但OHP手术密切相关的影像学引导技术、介入器械改良等仍需进一步的研究.     

新生儿和小婴儿复杂先天性心脏病手术的发展和优化

目的:回顾新生儿(年龄≤28天)和小婴儿(28天<年龄≤90天)复杂先天性心脏病(先心病)外科治疗在上海新华医院、上海儿童医学中心的发展,对常见复杂先心病的手术方法、手术适应证和时机及围术期生命器官保护提出优化方案。方法:109例新生儿和193例小婴儿患复杂先心病而接受手术治疗。根据不同的年龄水平和手术阶段将资料按等级分组对照,A组:10例,为新生儿手术第一阶段;B组:19例,为新生儿手术第二阶段;C组:80例,为新生儿手术第三阶段;D组:10例,为小婴儿手术第一阶段;E组:31例,为小婴儿手术第二阶段;F组:152例,为小婴儿手术第三阶段。新生儿和小婴儿病种分布中大动脉转位最多,分别占66例(60.6%,66/109)和67例(34.7%,67/193)。结果:总住院死亡60例(19.9%,60/302),新生儿24例,小婴儿36例。A组、B组和C组的死亡率分别为40.0%(4/10)、57.9%(11/19)和11.2%(9/80)。D组、E组和F组的死亡率分别为70.0%(7/10)、29.0%(9/31)和13.2%(20/152)。F组患儿中大动脉转位和室间隔完整的肺动脉闭锁手术死亡率均明显高于C组。结论:对于复杂和危重先心病应该积极采取早期手术措施,并要求有急诊手术的观念。通过优化手术方法和强化围术期生命器官的保护以提高手术生存率。     

Unroofed coronary sinus and persistent left superior vena cava.

This report describes a case of unroofed coronary sinus and persistent left superior vena cava discovered during an echocardiographic investigation for dilated pulmonary artery. An unroofed coronary sinus is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava. The latter is a usual cause of a dilated coronary sinus. The detection of a dilated coronary sinus should therefore prompt the search for abnormal coronary sinus drainage and other cardiac abnormalities. The treatment of unroofed coronary sinus and persistent left superior vena cava is undertaken only after assessing the pre- and post-treatment haemodynamics of all co-existing abnormalities.     

Bilateral superior vena cava with right superior vena cava draining into left atrium

Anomalies of systemic venous return are extremely heterogeneous congenital malformations with variable ranges from completely normal physiology to severe forms of right to left shunting requiring surgical treatment. Anomalous drainage of a right-sided superior vena cava (SVC) to the left atrium (LA) is one of the rarest variants of systemic venous return anomalies, characterized by right-to-left shunt physiology and cyanosis. Here we report a 2 years old girl presented with cyanosis which was observed shortly after birth by her parents but not further investigated. She is otherwise active girl and with normal growth and development. Her clinical examination was unremarkable apart from mild clubbing of the fingers and low oxygen saturation of 88–90% in room air. Her ECG and chest X-ray were unremarkable. Echocardiography showed bilateral SVC connected by a small innominate vein. The right SVC drains directly into the LA while the left SVC drains into the right atrium (RA) via a dilated coronary sinus. There is a small superior sinus venosus type atrial septum defect (ASD) with left to right shunt. Also, there is partial anomalous pulmonary venous return with right upper and right middle pulmonary veins draining directly into the right SVC, which is connected to LA. The right lower pulmonary vein and left pulmonary veins drain directly to LA. The rest of her echocardiography demonstrated normal heart structures and function. This patient was referred for surgical correction, including baffling of the right SVC to the RA and closure of the ASD. We describe this case to highlight the importance of recognizing this rare anomalous systemic venous connection as one of the very rare causes of cyanosis in the pediatric age group as well as at older age.     

Transvenous defibrillator implantation in patients with persistent left superior vena cava and right superior vena cava atresia

In this report a transvenous cardioverter defibrillator implantationis described in two patients with a persistent left-sided superiorvena cava and right SVC atresia. In the first case, manoeuvringof the guide wire inserted through the left subclavian veininto the SVC proved impossible, revealing a left SVC originatingfrom the left brachiocephalic vein with an acute corner. Changingthe side of implantation and inserting a CPI Endotak catheterthrough the right subclavian vein, the lead was easily advancedthrough the left SVC into the coronary sinus and then into theright atrium with the tip abutting the lateral atrial wall.Subsequent manoeuvres allowed passage of the tip of the catheterinto the right ventricular apex with the proximal defibrillationcoil of the Endotak lead in the low left SVC, with its distallimit at the junction with the coronary sinus. A biphasic waveformsingle pathway RVleft SVC successfully defibrillated with astored energy of 5 J. In the second patient, implantation of a transvenous Medtronicsystem was possible from a left infraclavicular approach. Atripolar R V coil was inserted into the right ventricle viathe persistent left SVC and contiguous coronary sinus. Becauseof the acute angle required to enter the R V in this secondcase, the R V lead was looped in the right atrium in order toenter the RV in a satisfactory, albeit atypical RV location.This patient was successfully defibrillated with a 5 J monophasicwaveform delivered between the RV coil, a CSIleft SVC coil,and a subcutaneous patch. In conclusion, both of these patients illustrate the abilityto use transvenous ICDs successfully in patients with persistentleft superior vena cava although the implantation techniquedeviates substantially from traditional methods.     

Bilateral inferior venae cava combined with the persistent left superior vena cava and hemiazygos continuation of left inferior vena cava with drainage into right atrium: A case report

The persistent left superior vena cava (PLSVC) is a common venous abnormality. However, malformation of the bilateral inferior venae cava (IVC) is extremely rare, with an incidence rate of .3%. IVC malformation is associated most frequently with heart defects and isomerism and often has a poor prognosis. We presented a case of vascular malformations in the fetus of bilateral caval veins with the interruption of the left-sided venous return with hemiazygos continuation in presence of a right-sided inferior caval vein. Also noted were the PLSVC and a dilated right heart with a widened pulmonary trunk. In this case, there were no heart defects or chromosomal abnormalities, and the newborn postpartum was in a good condition.     

Absent right superior vena cava with persistent left superior vena cava: Implications and management

Seven cases of absent right superior vena cava with persistent left superior vena cava and normal situs were diagnosed at Children's Hospital of Pittsburgh. All patients had associated cardiac defects. In two cases the diagnosis was made at autopsy, the first in 1957 in a 26 day old infant with multiple congenital defects and the second in 1965 in a 22 day old infant who had pulmonary atresia with ventricular septal defect and patent ductus arteriosus. Since 1966 absent right superior vena cava has been diagnosed at cardiac catheterization in five children. Three of these children have had surgery, two for subaortic stenosis and one for an atrial septal defect. One has an insignificant atrial septal defect and the fifth has a ventricular septal defect. The electrocardiogram of four reveals a short P-R interval and a leftward frontal plane axis of the P wave, suggesting a low atrial focus. None has had any significant conduction problem. All five children are living and well; the oldest has survived 13 years postoperatively. Certain precautions are necessary should corrective cardiac surgery or transvenous pacemaker insertion be necessary.     

Persistent left superior vena cava: a blessing in disguise

Persistent left superior vena cava (SVC) is an uncommon condition,usually encountered during cannulation of the left subclavianvein. We describe a patient who required a cardiac device upgradeto cardiac resynchronization implantable cardioverter defibrillatorfor biventricular failure. The presence of a persistent leftSVC proved to be a blessing in disguise in this patient as hehad since developed total occlusion of the left subclavian-innominatesystem.     

新疆地区107例低体质量婴儿先天性心脏病的外科治疗

目的:探讨新疆地区体质量5 kg以下少数民族婴儿,先天性心脏病(先心病)的外科治疗方法及围术期处理。方法:2006年7月至2011年12月,本院手术治疗5 kg以下先心病患儿107例,年龄11 d至13个月,体质量2.6～5 kg。病种包括:动脉导管未闭(PDA)4例,室间隔缺损(VSD)和(或)房间隔缺损(ASD)54例,其中部分伴动脉导管未闭(PDA)和(或)肺动脉高压(PH),肺动脉瓣狭窄(PS)1例,法洛三联症3例,法洛四联症(TOF)9例,完全性心内膜垫缺损(ECD)9例,右心室双出口(DORV)9例,完全性大血管转位(TGA)8例(室间隔完整4例,室间隔缺损4例),完全性肺静脉异位引流(TAPVC)3例,主动脉弓缩窄(COA)并室间隔缺损、房间隔缺损3例,肺动脉闭锁(PA)2例,三尖瓣闭锁(TA)1例,房室连接不一致1例。本组中I期根治手术96例,减状手术11例(肺动脉环缩术6例,中心分流术4例,右心室流出道疏通术1例);体外循环下手术93例,其中深低温停循环3例。结果:本组术后早期死亡6例(5.6%),死于低心排出量综合征(低心排)1例,多脏器衰竭2例,呼吸衰竭2例,凝血功能障碍1例。术后主要并发症:低心排9例,多脏器损害5例,肾衰竭3例,肺部感染28例,心律失常3例,切口感染3例,心包积液2例,残余分流1例及乳糜胸1例。随访2个月～5年,81例无中期死亡,再手术3例。结论:严格评估低体质量先心病患儿手术适应证和时机,加强围手术期处理,是提高治疗效果的关键。     

Absent right and persistent left superior vena cava

A patient is described presenting with atrial fibrillation. A dilated coronary sinus was found due to the presence of a persistent left superior caval vein. Absence of the right superior caval vein was suspected with contrast injection through an i.v. line in the right arm, and was confirmed with phlebography. No associated cardiac anomalies were found. Persistent left superior vena cava is a common anomaly, although simultaneous complete absence of the right superior vena cava is rare. The incidence, embryology, diagnosis and importance of this anomaly is discussed.