猝死与特发性长Q-T综合征

心率校正后的 Q-T 间期正常最高值通常是0.44秒.Q-T 间期延长可并发室性心律失常、晕厥和猝死。各种遗传型和散发型复极延长综合征统被称为长Q-T 综合征(简称:LQTS)。特发性 LQTS 患者可能完全无症状,心电图异常只是偶然发现。通常在有先证病例的家族中,发现有人出现无法解释的晕厥,再经检查,发现是 LQTS。也有些 LQTS 病例是在调查先天性神经性耳聋病人时发现的。估计约0.3%的先天性神经性耳聋患者,心电图证明有 Q-T 间期延长。LQTS 患者晕厥反复发作是由于暂时性心室扑动或颤动。这种恶性心律失常的频繁发作造成晕厥和很高的猝死率.     

云南七个家庭不明原因猝死的临床研究

目的 探索云南不明原因猝死(SUD)的临床表现特点.方法 前瞻性的调查2005年发生在云南的不明原因猝死病例,分析了具有聚集性特征的7个家庭及相关病例的临床表现特点.结果 通过对2005年发病的7个发生聚集性猝死的家庭及其相关病例的调查资料,分析病例的临床特点.发现死者和同发病例除具有头晕、乏力、甚或反复晕厥等共同症状外,尤其采用恶心、呕吐、腹痛和腹泻的消化道症状将聚集性病例分为两大类:(1)具有消化道症状的聚集性死亡:多可以寻找到共同的可疑饮食史,或具有发病过程中类似的实验室指标(天冬氨酸转氨酶、肌酸激酶、乳酸脱氢酶等)剧烈升高到降低恢复的过程.(2)没有消化道症状的聚集死亡:这组聚集死亡和同发病例临床表现呈现多样化,首例死亡与续发死亡病例临床表现差异大,续发病例发病到死亡时间短,死亡前缺少或无症状.(3)能得到的心电图显示为显著的长Q-T间期和ST-T改变.结论 云南SUD具有时间、空间和人群高度聚集的特点,其中家庭/时间聚集性特征的猝死更是研究的主体,它很难用某一疾病来解释.其临床表现多样化,但可根据有无消化道症状粗分为两种不同特点的猝死.没有发现有遗传性疾病和(或)传染病的特征.由于其病因不明,故还需进行详细、深入的研究.     

短Q-T间期综合征3例

Q-T间期是QRS波群、ST段及T波时间的总和,代表心室肌除极和复极所需要的时间。长Q-T间期易诱发严重的室性心律失常而猝死,而短Q-T间期近年来亦认为可致心律失常性猝死和临终前的心电图改变,也是一种严重的心电现象〔1〕。本文报告3例因ST段缺失致短Q-T间期综合征。例1男,43岁,晕厥原因待查入院。入院后晕厥发作时心电图示心室颤动(图1Ⅱa),经电击除颤后恢复窦性心律,心率47～48次/min,P-R间期0.20s,QRS波群时间0.14s,QRS波群结束后立即出现T波上升肢,ST段消失,Q-T间期0.28s,为预测值的63%[Q-T间期预测值计算方法为656÷(1 心率…     

Q—T间期延长综合征

Q-T间期延长综合征(LQTS)是一种较常见的心电图异常综合征。因其易引起致命性心律失常,甚至猝死,故提高对本病的认识,及时做出诊断和处理,殊为重要。一、病因引起Q-T间期延长的原因很多。儿科常见者为原发性(特发性、先天性)LQTS。其临床特征是先天性耳聋、反复发作性晕厥,心电图Q-T间期延长,故也称心脏耳聋综合征,属常染色体隐性遗传。亦有不伴有耳聋的LQTS,属常染色体显性遗     

云南地方性暴发性心肌炎临床特点分析

目的对发生在云南省的云南地方性暴发性心肌炎的临床特点进行归纳分析和综述。方法对1982~2003年发生在云南省的12起中最具代表性的猝死病例及存活疑似病人的症状、体征、异常心电图、X线结果进行系统分析。结果云南地方性暴发性心肌炎的临床症状不明显,多为突然发病、突然死亡,部分有头昏、头晕、胸闷、心悸等症状;病人多呈贫血貌,体检有心音低钝、心律失常等异常体征;心电图显示以ST-T改变、房室传导阻滞(AVB)、完全性右束支传导阻滞、Q-T间期延长等心肌炎的心电图改变为多,X线摄片部分有心脏扩大。结论该病符合心肌炎的诊断特点,它的临床特征、防治措施有待于进一步探讨和研究。     

Q—T离散度在有关疾病中的临床意义

Q-T间期离散度(Q-Td)是指心电图各导联间Q-T间期的变异程度[1].Q-Td及其心率校正值(Q-Tcd)与心肌细胞除极后复极不均匀有关,体表心电图上表现为Q-T间期延长和长短不一.当心室肌复极不稳定,Q-T间期不固定,则室性异位激动极易落在心肌的"易损期"而引起室性心动过速、心室颤动.Q-Td增大可见于Q-T间期延长综合征者,也可见于缺血性心脏病、高血压性心脏病、扩张性心肌病、低血钾、甲状腺功能亢进症、尿毒症等疾病.Q-Td增大是严重泵衰竭、室性心律失常、甚至猝死的先兆表现,对上述疾病和药物疗效的评价均有一定价值.     

2005—2009年新发云南不明原因猝死病区人群临床检查检测指标调查研究

目的研究2005-2009年新发云南不明原因猝死病区人群不同时期的健康状况及变化趋势，为建立和完善病例监测报告标准和诊断标准提供依据，为病因研究提供线索。方法调查新发猝死病区人群和对照人群的心电图、血液生化、血压、脉搏、呼吸、体温、身高、体重等临床检查检测指标，分析各类人群各项指标之间的差异。结果新发猝死病区人群心电图异常检出率、心肌酶异常率和均值表现为急性期：猝死病例〉同发病例〉家庭成员〉同村村民〉邻村村民〉对照人群，猝死病例和同发病例明显高于其他人群；同类人群：急性期〉恢复期，恢复期已降低至对照人群流行期前及流行期间水平；各类人群的基础生命体征检查结果相同。结论心电图和心肌酶是新发猝死病区人群临床检查检测指标中异常改变最为明显的指标，是猝死病例和同发病例区别于其他人群的高风险健康指标，具有明显的“心源性”特点；心电网和心肌酶是制定病例定义、有效干预措施和临床救治措施的重要参考内容，是病情监测指标和猝死事件发生的预警指标，病区环境中可能存在某些致心肌损伤的危险因素，表现为人群心电图发生特异性改变和心肌酶水平异常增高。     

完全性房室传导阻滞伴继发性长Q-T 间期综合征1例

患者男性,72岁.因下肢浮肿半年,活动后胸闷3月伴发作性晕厥3天入院.发作时无抽搐及大小便失禁,持续约2 min可自行缓解.既往无高血压病史.入院时体检:BP 90/60mmHg.神志清.颈静脉怒张,肝颈反流征阳性.入院后血清电解质及心肌酶谱检查均正常.超声心动描记术检查示左心室肥大,心功能减退,轻度主动脉瓣、二尖瓣关闭不全.临床诊断:缺血性心脏病,完全性房室传导阻滞伴室性逸搏心律,心功能不全2级,阿-斯综合征.心电图(图1)示:P-P间期规则,频率83次/min,QRS波群宽大畸形,R-R间期规则,频率35次/min,P-R间期长短不一,为完全性房室传导阻滞,室性逸搏心律,ST段呈水平延长约0.26s,T波巨大倒置,Q-T间期明显延长达0.76s.心电图诊断:窦性心律,完全性房室传导阻滞,室性逸搏心律,Q-T间期延长.     

云南不明原因猝死重点病区人群心电图调查研究

目的研究云南不明原因猝死重点病区人群的健康状况,探索其与云南不明原因猝死发病之间的关系,为病因学研究、制定有效的干预和临床救治措施提供科学依据。方法选择两个反复发病村、一个新发聚集性病例村和一个对照村作为调查点,连续观察测量研究对象的心电图、血液生化、血压、心率、呼吸频率、体温、身高和体重等健康指标,对比分析各类人群各项指标之间的差异。结果重点病区人群心电图以“U波异常、T波异常、传导阻滞、ST-T改变、Q-T间期延长”等心肌损伤型表现为主,其中“U波异常”检出率高达50．9％,明显区别于对照人群“窦性心律不齐、电轴左偏、低电压”等归属为“正常”的心电图改变类型;大部分相同类型的心电图检出率重点病区人群明显高于对照人群,重点病区人群的BMI超重和消度检出率低于对照人群。结论重点病区人群的心电图改变较为复杂和严重,存在较高的发生恶性心律失常的潜在风险,且反复发病村人群较新发聚集性病例村人群存在的心脏健康风险更高;“Q-T间期延长”可作为发生恶性心律失常的预警指标,心电图和心肌酶可联合作为云南不明原因猝死病区人群的特异性临床检查、检测指标,是制定病例定义和临床救治措施的重要参考指标。     

长Q-T综合征基因携带者的症状群与Q-T间期值

尽管心电图上Q-T间期延长,室性心律紊乱和猝死是家族性长Q-T综合征(LQTS)的特征,但尚不能肯定Q-T间期是诊断LQTS的敏感或特异指标。新近,作者在三个长Q-T综合征家族成员中经11p染色体的基因位点鉴别分为长Q-T基因携带者与非携带者,同时比较了两组的临床特征,     

12例心电图异常患者的传导系统病理改变观察

目的探讨心脏传导系统（CCS）病变与心律失常及心电图ST-T改变的关系。方法对12例有临床心电图异常改变的心脏标本作CCS组织学观察。结果（1）12例心电图异常者均见CCS有病变,包括有CCS脂肪浸润和（或）纤维化、淀粉样变、炎症、发育异常及出血等。（2）组织学显示急性炎症性改变者的心电图改变主要有：窦性心动过速、频发多源性室性早搏。（3）组织学显示慢性退行性改变者的心电图改变复杂多样：包括慢-快综合征、窦性心动过缓并室内分支传导阻滞、房室传导阻滞及心室内传导阻滞。（4）CCS病变可有心电图ST-T改变。结论CCS病变是心律失常及心电图ST-T改变的病理基础之一。     

Right ventricular electrocardiographic leads for detection of Brugada syndrome in sudden unexplained death syndrome survivors and their relatives

BACKGROUND: Sudden unexplained death syndrome (SUDS) is a sudden death syndrome in previously healthy Southeast Asian young adults without any structural causes of death. Many SUDS survivors show electrocardiographic (ECG) evidence of RSR' and ST elevation in leads V1 to V3, which is similar to the ECG pattern in Brugada syndrome. However, in many cases transient normalization of the ECG does not make diagnosis with standard 12-lead ECG possible. HYPOTHESIS: To overcome this problem, we utilized the new right ventricular ECG leads to detect the Brugada syndrome in SUDS survivors. METHODS: The subject was a Thai male patient who presented with a SUDS-like syncopal attack. He had cardiac arrest due to idiopathic ventricular fibrillation. RESULTS: Post-resuscitation standard 12-lead ECG showed no diagnostic features of Brugada syndrome. However, ECG patterns of RSR' and ST elevations typical for Brugada syndrome could be detected at the higher intercostal space leads V1 to V3. We observed similar findings in 2 of the other 10 SUDS survivors and 4 of 23 healthy family members. CONCLUSIONS: Our data suggest that these new right ventricular leads ECG may be helpful in detecting Brugada syndrome in SUDS survivors and their relatives.     

2004年云南省腾冲县云南不明原因猝死疫情的调查分析

目的 探讨云南不明原因猝死的流行特点、临床表现和病因,为制定有效的防治措施提供依据.方法 通过现场调查,收集和分析流行病学资料和临床资料,通过尸体解剖进行病理学检查和临床学研究. 结果 本起疫情发生在7月上旬,2天内发病4人,死亡4人,发病者均为农民,未发现同发病例.4个病例的临床表现不完全一致,2个10天前有上呼吸道感染症状,1个有约3天的病程,表现为乏力、头昏、胸闷、气促等,1个无任何表现; 3个死亡病例表现为心源性猝死.病毒检测结果 :1位健康家属的粪便中分离到1株新型肠道病毒;2例死亡病例的尸体病理检查结果 表现不同. 结论 本病有明显的地区性、季节性、家庭和或村庄聚集性;发病率低,病死率高;临床表现不特异,以心源性猝死为主,诊断和救治很困难.     

Familial Sudden Cardiac Death Associated with a Terminal QRS Abnormality on Surface 12-Lead Electrocardiogram in the Index Case

Familial Sudden Death and ECG Abnormalities. A case is presented of an 18-year-old male who had been resuscitated following an episode of sudden death due to ventricular fibrillation. The patient was noted to have an abnormal deflection in the terminal QRS on surface ECG and an abnormal signal-averaged KCG demonstrating a late potential coincident with the terminal QRS abnormality on the ECG. The patient had easily inducible polymorphic ventricular tachycardia during electrophysiologic study, which was suppressed by quinidine but not by procainamide or beta blockers. The surface ECG and signal-averaged ECG also were normalized by quinidine but not by procainamide or beta blockers. The patient had no further arrhythmias on quinidine for 6 years until he inexplicably discontinued his medication and died suddenly shortly thereafter. The present case may represent a unique familial sudden death syndrome or possibly a variant of the sudden death syndrome associated with right bundle branch block and ST elevation in V₁, through V₃, Currently available data suggest that, in such patients, an implantable cardioverter defibrillator may provide better protection from sudden death than does antiarrhythmic drug therapy.     

云南省地方性猝死早期发现的探讨

目的寻求云南省地方性猝死早期发现的方法。方法以现场流行病学调查及临床医学检查,调查25例死者的发病与死亡经过并确定死因;对高危人群106人及对照人群214人进行查体、心电图、X线检查,以确定各调查点的现患。结果共确诊死者及现患52人,其流行病特点、临床表现、心电图、X线检查结果均具有显著共性;运用症状监测方法,出现头晕、胸闷、气短、心悸、乏力、恶心呕吐等心功不全症候群以及抽搐、晕厥等心脑综合征者应列为监测对象。结论根据该病的流行病学及临床特点,以症状监测为核心的综合监测方法可以在该病的早期发现中加以运用。     

Brugada or not-Brugada: misdiagnosis of recorder-induced artifact

The Brugada-Brugada syndrome is a life threatening cardiac arrhythmia that features syncopal events or aborted sudden death in combination with electrocardiographic characteristics (ST-segment elevation of V(1)-V(3) and right bundle branch block). Typical ECG alterations were recorded in a young man who was admitted to our hospital after syncope and a Brugada-Brugada syndrome was suspected. The following days similar pathological ECG recordings of other patients were noticed. The electrocardiographic artifact was diagnosed as being caused by incorrect handling of the ECG recorder.     

Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report.

OBJECTIVES. The objectives of this study were to present data on eight patients with recurrent episodes of aborted sudden death unexplainable by currently known diseases whose common clinical and electrocardiographic (ECG) features define them as having a distinct syndrome different from idiopathic ventricular fibrillation. BACKGROUND. Among patients with ventricular arrhythmias who have no structural heart disease, several subgroups have been defined. The present patients constitute an additional subgroup with these findings. METHODS. The study group consisted of eight patients, six male and two female, with recurrent episodes of aborted sudden death. Clinical and laboratory data and results of electrocardiography, electrophysiology, echocardiography, angiography, histologic study and exercise testing were available in most cases. RESULTS. The ECG during sinus rhythm showed right bundle branch block, normal QT interval and persistent ST segment elevation in precordial leads V1 to V2-V3 not explainable by electrolyte disturbances, ischemia or structural heart disease. No histologic abnormalities were found in the four patients in whom ventricular biopsies were performed. The arrhythmia leading to (aborted) sudden death was a rapid polymorphic ventricular tachycardia initiating after a short coupled ventricular extrasystole. A similar arrhythmia was initiated by two to three ventricular extrastimuli in four of the seven patients studied by programmed electrical stimulation. Four patients had a prolonged HV interval during sinus rhythm. One patient receiving amiodarone died suddenly during implantation of a demand ventricular pacemaker. The arrhythmia of two patients was controlled with a beta-adrenergic blocking agent. Four patients received an implantable defibrillator that was subsequently used by one of them, and all four are alive. The remaining patient received a demand ventricular pacemaker and his arrhythmia is controlled with amiodarone and diphenylhydantoin. CONCLUSIONS. Common clinical and ECG features define a distinct syndrome in this group of patients. Its causes remain unknown.     

发热诱发Brugada波的遗传学及心电参数危险因素分析

目的对发热诱发Brugada波的遗传学及心电参数危险性进行分析。方法使用DNA直接测序法对临床收集的5例发热诱发Brugada波患者进行基因筛查,并回顾性调查127例住院发热患者心电图上Brugada波和不完全右束支传导阻滞(IRBBB),并与正常对照组进行心电参数的比较。结果5例发热诱发Brugada ECG患者未发现基因突变。127例发热患者Brugada波1例,发生率为0.8%,13例有IRBBB。发热组患者QTc和TpTe间期较正常对照延长(P<0.05)。0.5~3年随访126例患者无一例发生心源性猝死。结论发热致Brugada波个体与钠通道基因突变无关联。发热诱发Brugada波的健康个体心血管事件发生率低。     

New electrocardiographic leads and the procainamide test for the detection of the Brugada sign in sudden unexplained death syndrome survivors and their relatives.

AIMS: Sudden unexplained death syndrome occurs in previously healthy South-east Asian young adults without any structural cause of death. The common electrocardiographic (ECG) change in sudden unexplained death syndrome survivors is right bundle branch block and ST elevations in leads V(1) to V(3), which are similar to the ECG pattern in the Brugada syndrome (Brugada sign). It is difficult to diagnose the Brugada sign with the 12-lead ECG in sudden unexplained death syndrome survivors and their family members because the ECG could be transiently normalized. We proposed using the higher intercostal space V(1) to V(3) lead ECG, together with procainamide to detect the Brugada sign. METHODS AND RESULTS: Among 20 ventricular fibrillation cardiac arrest patients, 13 sudden unexplained death syndrome survivors and their relatives (n=88) were studied using the single standard 12-lead ECG and the new six higher intercostal space V(1) to V(3) lead ECG (-V(1) to -V(3) and -2V(1) to -2V(3)). Ten sudden unexplained death syndrome survivors and relatives (n=48) who had a normalized ECG were also infused with procainamide (10 mg x kg(-1)i.v.) to unmask the Brugada sign and both ECG methods were recorded. Forty healthy individuals and 13 spouses served as the control group. Prior to the procainamide infusion, the Brugada sign could be detected in nine sudden unexplained death syndrome survivors (69.2%) and three (3.4%) relatives with the standard ECG and in 12 (92.3%) and nine (10.2%) with the new six-lead ECG. After the procainamide infusion, the Brugada sign could be demonstrated in seven sudden unexplained death syndrome survivors (70%) and seven (14.6%) relatives with the standard ECG and in nine (90%) (P=0.26) and 23 (47.9%) (P=0.0004) with the new six-lead ECG, respectively. All the controls were negative for the Brugada sign. CONCLUSIONS: Our data suggest that the new higher intercostal space lead ECG, with or without the procainamide test is helpful in detecting the Brugada sign in sudden unexplained death syndrome survivors and their relatives.