小儿器质性心脏病引发的心脏性猝死

心脏性猝死（SCD）是指由于心脏原因引起的自然死亡。以急性症状出现1h内突然意识丧失为先兆，患儿发生SCD前可以有已知的心脏病史，但是死亡的时间和形式具有无法预测的特点 。多项回顾性调查显示，儿童和青少年SCD的年发病率为0．8～6．2／10万，明显低于成人的100／10万 。小儿SCD的发病率随年龄而增加，占1～13岁猝死原因的19％，14～21岁猝死原因的30％ 。     

心脏起搏器和植入式心脏转复除颤器在小儿心脏性猝死防治上的临床应用

心脏性猝死（SCD）是因心脏性原因在急性症状发生后1h内出现的意识丧失与死亡，是“自然的”、“骤然发生的”、“快速的”和“不能预期的”。SCD的原因有很多，其中严重心律失常可威胁小儿生命。心律失常性SCD中大部分（80％～90％）是由室性快速心律失常所致，较少见的是缓慢性心律失常，它们均可导致心脏停搏而发生SCD。婴儿期大多数心脏骤停与呼吸衰竭或休克进行性恶化有关。儿童中的猝死发病率较低，在1～22岁的人群中，SCD的发病率仅为其总病死率的2．5％。     

小儿心脏性猝死的预防与急救处理

心脏性猝死可突然发生在任何时候，较难预测，对心脏性猝死的预防目前尚十分有效的措施，但严密监测高危人群，及时发现并及时正确的施救，有助于提高心搏骤停儿童的存活率。     

小儿心脏性猝死的病因及发病机制

尽管心脏性猝死（SCD）在小儿很少见，但是一旦发生，对家庭和医疗机构来讲都是巨大的打击。最新的研究发现，美国SCD每年为45万例，在澳大利亚和新西兰每年有近5万患者死于各种原因的猝死 。SCD的定义是由于心脏原因从出现症状的1h内出现非预测性的自然死亡，或既往没有表现出致命的症状，但在睡眠过程中患者出现无目击者的死亡 。该病有2个发病高峰：从出生～生后6个月和年龄在45～65岁之间。     

心脏临时起搏治疗小儿心律失常

目的了解心腔心内膜临时起搏治疗在儿科的应用。方法对１８例（其中伴阿斯发作１７例）于入院后进行右心腔心内膜临时起搏治疗，起搏阈值１～２Ｖ，起搏电压３～５Ｖ，起搏频率７０～１００次／分，起搏时间１．５～１３天。起搏前静脉滴注异丙基肾上腺素，伴发心力衰竭及休克者予多巴胺、硝普钠、地戈辛。结果１６例起搏治疗后阿斯发作消失，循环改善，Ⅲ度房室传导阻滞（ＡＶＢ）、高度ＡＶＢ、扭转室速、室颤消失，均救治成活，因室速起搏死亡１例，安置预防性起搏１例。结论心脏内临时起搏是治疗小儿慢性或某些快速型心律失常伴阿斯发作的首选方法，疗效满意。对Ⅲ度ＡＶＢ或高度房室传导阻滞的患儿虽未有阿斯发作，但因手术或伴有先天性心脏病需进行心血管造影或手术时，安置预防性临时心脏起搏以防止麻醉、手术中意外。     

小儿心脏直视术后心律失常的临床分析

小儿心脏直视术后心律失常的临床分析河南新乡市中心医院心胸外科（４５３０００）朱继先，李俊杰，牛玉顺，杜德录我院自１９８６年１２月～１９９５年１２月对先天性心脏病（先心病）行心脏直视手术１１５例，术后发生心律失常４３例。现就其发生原因及治疗进行分析。临...     

加强对儿童心脏性猝死的认识与防范

为进一步提高医务工作者对儿童心脏性猝死(SCD)的认识,从而使小儿心脏性猝死得判更好的预防和控制,使高危儿童生活质量得到更大的提高,文章就小儿心脏性猝死的流行病学、危险因素及导致SCD的心脏疾病、预防、治疗等作简要概述.     

加强小儿心律失常的临床研究

近十几年来我国儿科心律失常的基础及临床研究取得了明显的进展及成绩。随着心脏电生理及药物电生理作用的深入研究及广泛应用,经导管射频消融(radio frequency catheter ablation,RFCA)治疗快速心律失常的迅速发展及普及,起搏器技术的提高和适应证的拓宽,循证医学(evidence—based medicine;EBM)在心血管疾病研究中的应用,使心律失常的诊治发生了划时代的变化,改变了许多以往心律失常诊断及治疗的传统模式。     

Surgical therapy for sudden cardiac death in children

Sudden cardiac death (SCD) in children is the result of multiple etiologies and treatment (prophylaxis) must be tailored accordingly. In children who do not have congenital heart disease, surgical therapy of SCD typically consists of implantation of an internal defibrillator, with specific attention to the small size of the patient. In children who have unrepaired congenital heart disease, therapy of SCD is primarily repair of the congenital anomaly. In children or young adults who have previously undergone surgery for congenital heart disease, SCD therapy consists of repair of any residual or acquired structural defect, often in combination with antiarrhythmia surgery or defibrillator implantation.     

Aborted sudden cardiac death in adolescent

Sudden cardiac death is rare in children and adolescents but accounts for 19% to 30% of sudden deaths until 21 years of age. Fatal ventricular arrhythmias are usual common pathways in such tragic events, and underlying etiologies include cardiac ion channelopathies in majority of cases. We present a case of aborted sudden cardiac death in field, resuscitated successfully, and a clinical event in the pediatric emergency department that led to the diagnosis of the underlying rare condition.     

Substrates for sudden cardiac death

Pre-event identification of a specific cardiac substrate abnormality in a patient is an important step in preventing sudden cardiac death (SCD) in the pediatric and adult population. Certain cardiac substrate abnormalities can render the patient "at risk" for SCD and strategies for prevention of SCD in children must involve the identification and subsequent modification of these cardiac substrates.     

Molecular genetic basis of sudden cardiac death

This article outlines the up-to-date understanding of the molecular basis of disorders that cause sudden death. Several arrhythmic disorders that cause sudden death have been well-described at the molecular level, including the long QT syndromes and Brugada syndrome; this article reviews the current scientific knowledge of these diseases. Hypertrophic cardiomyopathy, a myocardial disorder that causes sudden death also has been well-studied. Finally, a disorder in which myocardial abnormalities and rhythm abnormalities coexist, arrhythmogenic right ventricular dysplasia, is described.     

Neonatal long QT syndrome and sudden cardiac death

Neonatal sudden cardiac death most often results from cardiac electrical diseases, cardiomyopathies, or sudden infant death syndrome. In infants without a known premortem diagnosis or abnormalities identified at autopsy, sudden infant death syndrome accounts for the vast majority of sudden deaths. Potential cardiac causes of some sudden infant death syndrome cases may include malignant brady- or tachyarrhythmias and congenital long QT syndrome. The possible mechanisms include abnormal brain stem respiratory control of arousal, dysautonomia and malignant cardiac bradyarrhythmias or tachyarrhythmias. Screening for neonatal sudden cardiac death may not be feasible, but hopefully through careful review of history, physical examination, and family health history, and judicious diagnostic testing, can the risk of cardiac sudden death be reduced. Further comprehension of the genetic basis of inherited arrhythmia disorders may help elucidate the mechanisms of arrhythmogenesis and etiologies of sudden infant death. Prevention and treatment of these disorders may also be improved through more detailed understanding of the molecular basis of cardiac electrical pathophysiology.