Multifocal or chaotic atrial rhythm

Summary Nine infants with episodic or continuous chaotic atrial rhythm (CAR) are presented. In addition to 3 or more different P-wave contours, atrial rates greater than 100 per minute, variable PP, RR, and PR intervals, and a discrete isoelectric baseline, findings included atrial rates that varied from a low of 50 to 120 to a high of 140 to 270 per minute, ventricular rates that varied from a low of 40 to 50 to a high of 180 to 270 per minute, and periodic sinus arrest with junctional escape rhythm. Except for the arrhythmia, all had a normal cardiac examination, ECG, chest x-ray film, and echocardiogram. Six infants were otherwise normal; one had an orbital rhabdomyosarcoma; one had neonatal asphyxia; and one had respiratory distress, bronchopulmonary dysplasia, and an intraventricular cerebral hemorrhage. The CAR persisted from 3 days to 20 months; it spontaneously reverted to normal sinus rhythm in 8 infants and persists in 1 infant at age 7 months. Digoxin (4 patients), propranolol hydrochloride (3 patients), quinidine sulfate (2 patients), and lidocaine (1 patient) did not alter the CAR. No patient had heart failure secondary to the CAR, although three also had episodes of sustained atrial tachycardia, which while present caused heart failure. All patients are functioning normally at home and have normal findings on cardiac examination and have normal ECGs at ages 3 to 38 months. Seven are in normal sinus rhythm, one has rare atrial premature contractions, and one has persistent CAR. We conclude that specific treatment was not necessary in these infants with CAR, except in those with associated sustained atrial tachycardia, which itself may cause heart failure.     

Familial idiopathic atrial fibrillation with bradyarrhythmia

A 10-year-old boy is reported who presented with idiopathic atrial fibrillation and bradyarrhythmia. After history of intrauterine and postnatal bradycardia, atrial fibrillation was first documented electrocar-diographically at 16 months of age. An underlying structural heart disease was not evident. At the age of 10 years, implantation of a permanent ventricular demand pacemaker was indicated after syncope due to severe bradyarrhythmia. The family history revealed five persons of four generations with bradyarrhymias. Idiopathic atrial fibrillation known since childhood was documented in three close relatives. A high grade AV block resulting in bradyarrhythmias and the occurrence of ST-T-changes in precordial leads could be demonstrated in all affected family members suggesting a diffuse general conduction abnormality in these patients. In this family, idiopathic atrial fibrillation seems to be transmitted as an autosomal dominant trait.     

房隔穿刺和球囊房隔造口术建立房间隔缺损动物模型

为了探讨应用房隔穿刺和球囊房隔造口术建立可控大小房间隔缺损动物模型的新方法 ,选用3周～4周的乳猪共6头 ,体重5kg～10kg(平均7.5kg) ,在X线和超声引导下 ,应用房隔穿刺和Rashkind球囊房隔造口术 ,建立可控大小ASD动物模型 ;术后即刻超声测量ASD直径和ASD边缘与周围结构的距离。结果 :6例无1例死亡 ;除1例并发心包积液外 ,无其他并发症 ;术后即刻超声示ASD直径范围4mm～10mm(6.16mm±2.40mm) ;ASD边缘与二尖瓣、三尖瓣、上腔静脉、下腔静脉、右上肺静脉及冠状窦的距离均在5mm以上。提示这种新方法具有易操作性 ,可控性 ,创伤小 ,并发症少 ,成功率高等优点 ,值得推广和应用。     

食道调搏在儿科房室交界区功能检查中的应用

目的　探讨食道心房调搏测量儿童房室交界区各不应期和传导时间的应用价值 ,评价其传导功能及其特点。方法　对 5 9例正常儿童进行食道调搏 (TEAP)检查 ,测量房室交界区不应期和传导阻滞点。结果　正常儿童房室传导功能由不应期和传导阻滞点具体体现 ,为儿童正常值提供参考 ;相对不应期 (RRP)、功能不应期 (FRP)、文氏点与心率呈显著相关 (P分别为 0 .0 0 0、0 .0 1、0 .0 0 0 ) ,与性别、年龄无关 (P均 >0 .0 5 ) ;而绝对不应期 (ERP)、2∶1阻滞点与性别、年龄、心率均无关 (P均 >0 .0 5 ) ;文氏点与RRP显著相关 (P =0 .0 0 0 ) ;2 :1阻滞点与ERP显著相关 (P =0 .0 15 )。结论　TEAP可评估儿童房室交界区的传导功能 ;RRP与心率有直接关系 ,ERP是评价房室传导功能的主要指标     

Familial idiopathic atrial fibrillation with fetal bradyarrhythmia

A woman presented at 28 wk gestation with fetal bradycardia 50 bpm, which persisted until 42 wk when an asymptomatic male baby was delivered. Electrocardiograph at 3 wk of age documented an incessant atrial fibrillation with slow ventricular response. He continued to be asymptomatic, but on follow-up at 16 y of age, 24-h Holter monitor showed a heart rate of 23 bpm and pauses of up to 6 s when a VVIR programme endocardial pacing system was employed. ECG carried out on his asymptomatic father showed intermittent atrial fibrillation, again with a slow ventricular response. CONCLUSION: Atrial fibrillation is extremely rare in children with normal cardiac structure. Most instances of fetal bradycardia are caused by congenital complete heart block. Other rare causes such as atrial fibrillation with fetal bradycardia need to be considered. This case might be a familial disorder and looks to have a good prognosis.     

Congenital aortico-left atrial tunnel

Summary A newborn baby with heart failure had ventricular septal defect and aorto-left atrial communication. Aortography demonstrated a large funnel-shaped vessel originating from a dilated left aortic sinus and opening into the base of the left atrial appendage. Open heart surgery was unsuccessful. Histological examination (750 serial sections) of the vessel including the adjoining aorta and left atrial appendage showed no evidence of coronary artery structures. To our knowledge, this represents the first case of congenital aortico-left atrial tunnel.Supported in part by grant RR-305 from the General Clinical Research Centers Program of the Division of Research Resources and by grant HE-07605-17 from the National Heart Institute, National Institutes of Health, Bethesda, MarylandDr. Lev is a Career Investigator and Educator of the Chicago Heart Association.     

食管心房调搏在小儿心动过速中的应用

目的探讨食管电生理检查对小儿心动过速的诊断、分型和终止发作的临床意义。方法采用食管心房调搏技术对３２例小儿快速心律失常进行分型诊断及转复治疗。结果心动过速３２例中体表心电图表现为室上性心动过速（室上速）２７例：其中房室折返性心动过速１５例,房室结折返...     

Hepatoma with right atrial extension

Summary The case is described of a 14-year-old boy who had a hepatoma with a right atrial extension. He presented with edema, abdominal pain, and ascites. Two-dimensional echocardiography showed a right atrial tumor that had invaded from the inferior vena cava as an extension into the right atrium of the hepatoma.     

Ballon atrial septostomy under transesophageal echocardiographic guidance

Summary Balloon atrial septostomy is an established method of palliation for several forms of congenital heart disease. Previously performed under fluoroscopic x-ray control, recent reports have demonstrated the utility of transthoracic echocardiographic monitoring. We report the first application of uniplane transesophageal echocardiography (TEE) (6.7-mm probe) as an alternative imaging modality for control of ballon atrial septostomy on neonates in the intensive care unit.     

儿童心肌炎经食道心房调搏检查36例报告

经食道心房调搏检查心肌炎患儿36例,结果:SACTc延长11例,cSNRT延长7例,SNRT延长3例;AVERP延长15例,AVFRP延长6例,文氏、2:1阻滞点降低分别为13、14例。SACT延长有5例与AVERP延长并存,AVERP延长者几乎与文氏,2:1阻滞点均降低。房室结功能异常的心电图表现主要为束支传导阻滞,其次为ST及T波改变;窦房结功能异常的心电图表现主要为窦房结传导阻滞。异常电生理患者仅有4例在1～3周内发病,大多在1月以上,最长1～2年,有心脏扩大者均有电生理异常。     

儿童继发孔型房间隔缺损的介入治疗

目的　探讨儿童继发孔型房间隔缺损 (atrialseptaldefect ,ASD)封堵术的指征、方法学和并发症的预防。方法　 1998年 10月～ 2 0 0 3年 1月 ,119例继发孔型ASD患儿 ,根据家属意愿接受了经导管应用Amplatzer房间隔封堵器的介入治疗。年龄 0 8～ 17 0岁 ,平均 ( 7 5± 2 8)岁 ,体重6 7～ 88.0kg ,平均 ( 2 3 7± 7 8)kg。所有病例术前检查被证实均为继发孔型ASD。按ASD球囊伸展直径或大于 1～ 2mm选择封堵器进行堵塞。其中 3例为多发ASD。 6例合并动脉导管未闭 ( patentductusarteriosus,PDA)或肺动脉瓣狭窄 ( pulmonarystenosis ,PS)者应用其他封堵装置和球囊扩张治疗合并的畸形。术后定期行心脏超声及临床检查随访。结果　 119例患儿术前经食道超声(transesophagealechocardiography ,TEE)或经胸超声 (trans thoracicechocardiography ,TTE)检测ASD平均直径 ( 12 9± 5 6 )mm ( 6 5～ 34 5mm ) ,肺动脉平均压力为 ( 2 9 0± 5 0 )mmHg( 2 5 0～ 6 2 0mmHg) ,球囊伸展直径为 ( 15 7± 4 8)mm( 8 0～ 38 0mm)。所选封堵器直径平均为 ( 15 0± 5 0 )mm( 8 0～ 38 0mm)。 112例封堵成功。 3例多发ASD也选用单一封堵器。 6例合并PDA或PS者同时完成介入治疗。 112例堵塞后即刻封堵率为 93 8% ( 1     

Right atrial myxoma with a nonembolic intestinal manifestation

Summary A 5-year-old male with right atrial myxoma without interatrial communication who presented with abdominal pain, vomiting, fever, and guaiac positive stool is reported. He was later found to have ischemia of a jejunal segment necessitating segmental resection. Although his symptoms persisted postoperatively, surgical removal, of a right atrial myxoma was followed by complete resolution of his intestinal symptoms. We demonstrated that the mesenteric vasculitis was of nonembolic origin, and we speculate autoimmune arteritis as a possible mechanism.     

经导管非开胸法关闭房间隔缺损15例报告

为探讨先天性心脏病房间隔缺损（ＡＳＤ）的非开胸疗法，采用经导管纽扣式补片对１５例ＡＳＤ患儿进行关闭治疗及随诊研究。患儿年龄２．５～１４．０岁，平均７．５岁；体重１１～３９ｋｇ，平均２０ｋｇ。采用彩色多普勒超声心动图（ＣＤＦＩ）、右上肺静脉造影及球囊三者结合测量来确定ＡＳＤ最大伸展直径。选择大于所测直径的２．０～２．５倍补片和相应长鞘。结果，１３例关闭成功（８７％），２例由于房间隔呈半弧圆形或不规则型，撤除补片改行外科手术治疗。１３例成功者，术后第２天残余分流５例（３８％），随诊１～１２个月残余分流下降为２例（１６％），１例有轻度二尖瓣关闭不全。提示，经导管纽扣式补片关闭ＡＳＤ的方法既简便、安全，又疗效佳，但有一定局限性，尚待进一步改进和完善。     

儿童快速房性心律失常的射频消融治疗

对传统方法和CARTO系统在指导导管射频消融儿童快速房性心律失常中的临床应用作初步探讨 ,前者为局灶性房速常规采用激动标测方法 ,后者则应用CARTO系统分别标测I型房扑和房缺在术后疤痕介导房内折返性房性心动过速靶点 ,并在房扑消融后重建三维电解剖图 ,判定峡部完全双向传导阻滞。结果显示3例成功消融 ,曝光时间(18±9)min。随访1月～3月无复发。由此经临床证实导管射频消融儿童快速房性心律失常安全有效 ,尤其对复杂快速房性心律失常 ,CARTO系统能在较少X线曝光下 ,易于标测和成功消融。     

介入治疗儿童多孔型房间隔缺损3例报告

近年来随着先天性心脏病介入治疗的普遍开展,应用介入治疗儿童继发孔型房间隔缺损的报道日见增多,但针对多孔型房间隔缺损的病例报道较少。我院自2001年开展介入治疗先天性心脏病以来,已成功为3例多孔型房间隔缺损患儿施行手术,现报告如下。临床资料一般资料:本组3例,均为自愿接受治疗的住院患儿,男1例,女2例;年龄3岁8个月至8岁。经胸彩色多谱勒超声心动图(TTE)诊断为ASD,2例为2个房间隔缺损孔,1例直径为16mm和4mm,两孔间距离为3mm;另1例房间隔缺,直径为10mm和3mm,两孔间距离为5mm。第3例为房间隔3个缺损孔,直径分别为18mm、3mm、2mm,两…     

应用Amplatzer双盘闭合器关闭小儿继发孔房间隔缺损

目的探讨应用Amplatzer双盘闭合器关闭小儿继发孔房间隔缺损（ASD）的临床效果。方法71例ASD患儿。男33例,女38例;年龄2～15岁,平均（7±4）岁;体重10～50kg,平均(22±11)kg。在经胸超声（TTE）的四腔心切面和剑下切面观察ASD的位置并测量ASD直径,测量房间隔（IAS）直径。在X线和超声帮助下用Amplatzer测量球囊导管测量ASD最大伸展径。根据所测得的ASD伸展径选择与之相同或比ASD大1～2mm腰部直径的Amplatzer闭合器堵闭71例患儿的ASD。术后随访疗效,追踪残余分流率和并发症。结果应用此法关闭71例ASD,全部成功。其残余分流率为2.8％。此方法并发症较少,疗效较好。本组资料分析示TTE测得的ASD径与测量球囊导管测得的ASD伸展径相关性好,从TTE测得的ASD径推算ASD伸展径的直线回归方程式6.54+0.97×TTE测得的ASD径（心尖四腔心）。结论经导管关闭继发孔ASD适应证为（1）年龄≥2岁,体重≥10kg,ASD直径≤30mm的单纯继发孔ASD或合并肺动脉瓣狭窄;（2）IAS≥ASD伸展径＋14mm;（3）ASD下缘与二尖瓣距离＞5mm,上下腔缘、后缘≥5mm,前缘≥2mm。超声心动图对病例的选择和对经导管监视关闭ASD术有不可忽略的作用,TEE还能弥补TTE的不足。Amplatzer双盘闭合器关闭ASD在儿科有较好的结果,但其疗效仍需长期随访。     

Angiographic quantification of mitral valve prolapse in children with secundum atrial septal defect

Summary We devised a simple method for quantifying mitral valve prolapse (MVP), relating the furthest point of prolapse to the measured length of the mitral orifice. By means of this index (MVPI), MVP was diagnosed angiographically in 10 of the 60 children with atrial septal defect (ASD). The mean age of the 10 children was 4.7 years. Cineangiography near the 60° right anterior oblique projection gave a profile of the mitral valve suitable for both qualitative and quantitative assessment. So far, MVP seems to be a relatively benign complication in this group of 10 children with ASD. Supported in part by Ontario Heart Foundation Grant #t-4-2 (1975–78).     

Non-surgical closure of atrial septal defect

BACKGROUND: Recently many devices for the transcatheter closure of atrial septal defect (ASD) were developed in the world. Several ASD closing devices, such as Clamshell device, buttoned device, ASD occluder system (ASDOS), Angel Wings, are not fully acceptable from a practical point of view. We reported the clinical trials of transcatheter closure of ASD for Clamshell double umbrella device and Amplatzer septal occluder (ASO) in Japan. METHODS: Clamshell devices were implanted in 11 patients. Clinical trials for ASO device were carried out in 34 patients in Japan. We reported the results of 17 patients in National Children's Hospital. RESULTS: Clamshell devices were implanted in all of the patients successfully. Minimal residual shunt was present in four patients (36%), 1 year after the procedure by Doppler color flow imaging. Nine of 11 umbrellas demonstrated fracture of the stainless steel arm. Arm fracture occurred between 1 week and 12 months after implantation. The implantations of ASO device were successful in all of the 17 patients. Sixteen out of 17 (94.1%) had complete closure in the average observation period of 5.5 months. Complications were minimal and transient. CONCLUSIONS: With respect to complete closure rate, easy manipulation, and complication rate, ASO is the best transcatheter ASD closure device among all the devices at the present time.     

Familial atrial septal defect with prolonged atrioventricular conduction

A family is described in which a mother, her two children and another two relatives all had atrial septal defect of the ostium secundum type. The index case and her mother also had prolonged atrioventricular conduction. It is considered that the defect is the familial type and inherited as a mendelian dominant trait.