Brugada综合征药物激发试验

目前认为 ,Brugada综合征 (Brugadasyndrome)是由于编码心肌钠离子通道基因突变引起功能异常而导致的临床综合征。其显著特征为 :(1)心电图V1～V3 导联ST段抬高、多变 ,多形室性心动过速 (室速 )和 /或心室颤动 (室颤 ) ;(2 )晕厥反复发作及心脏性猝死 ;(3)未发现器质性心脏病。流行病学调查表明 ,5 %的心脏性猝死者无器质性心脏病的证据 ,而其中 2 0 %是由Brugada综合征所导致的[1,2 ] 。正因为如此 ,Brugada综合征已成为当前心血管病基础和临床研究的前沿和热点之一。其临床谱很宽 ,可以从静息基因携带者(只携带突变基因 ,但无临床症…     

Brugada综合征心电图的药物激发试验

近年来 ,作为特发性心室颤动的一种特殊表现—Brugada综合征 ,正越来越引起人们的重视 ,它是一种基因决定的心电疾病 ,其特征性心电图表现为V1～V3 导联出现ST段抬高、T波倒置及右束支阻滞 ,有人称之“心电图右胸导联三联征”。但只有6 0 %的病例具备此典型心电图表现 ,其余 4 0 %患者呈隐匿性状态 ,或异常心电图自发性正常化呈间歇性过程 ,同一患者在不同时间其心电图异常程度、形态亦可不一致。影响以上心电图形态多变性、多态性的因素 ,除了自主神经调节及抗心律失常药物外 ,还与以下因素有关 :①心率 :一般情况下 ,心率快 ,ST段抬高…     

不同氟卡尼激发试验结果Brugada综合征患者心电图特点分析

背景 Brugada综合征(BS)病死率较高,近年来广泛受到学者重视.为了降低病死率,临床常选择氟卡尼治疗BS,虽可达到一定治疗效果,但部分学者发现其容易引起心电图(ECG)改变.目的 分析不同氟卡尼激发试验(FCT)结果BS患者ECG特点.方法 回顾性选取2013—2017年在内蒙古医科大学附属医院心内科行FCT的B...     

应用普罗帕酮激发试验对Brugada综合征七例患者的诊断价值

目的　评价应用普罗帕酮激发试验诊断Brugada综合征 (BS)的有效性和特异性。方法7例怀疑BS者和 10例对照者 ,经体格检查、X线胸片和超声心动图初步排除器质性心脏病 ,BS组行冠状动脉以及左、右心室造影排除冠心病。做好电复律和心肺复苏准备后按分级推注方案行普罗帕酮激发试验 ,观察标准 12导联心电图并监护心律失常发作。结果　 7例疑似BS者均达到欧洲心脏病学会建议诊断标准。 3例晕厥患者被确诊为BS ,另 4例被诊断为BS样心电图改变。 3例晕厥患者中 2例植入ICD ,其中 1例随访 10个月有 14次夜间心室颤动发作。另 1例拒绝植入ICD ,随访 4个月后夜间猝死。另 5例随访 (8± 6 )个月无心律失常事件发生。除 1例试验中出现室性早搏、心室颤动外 ,余无不良反应出现。结论　普罗帕酮激发试验可有效、特异地用于诊断Brugada综合征。     

发热诱发Brugada综合征的临床及心电图特征

目的 探究发热诱发Brugada综合征的临床及心电图特点.方法 选择2000年1月-2019年1月武汉大学人民医院收治Brugada综合征患者112例,根据诱发因素将全部病例分为发热组(34例)和非发热组(78例),比较分析两组临床特点及药物激发试验结果.结果 发热组在主要不良心血管事件发生率及家族史比例方面均显著大于...     

Brugada综合征

1992年,西班牙学者Brugada首先总结报道一组8例因严重心律失常引致猝死的新病症。其临床特点为反复间歇发作晕厥,无发作先兆,患者以中年为主,少数有家族史。经临床、实验室、X线胸片、超声心动图、CT以及心内膜心肌活检等检查可排除已知的心脏和脑部疾病。其心电图特点为:(1)完全性或不完全性右束支传导阻滞。(2)V_1—V_3导联ST段抬高,形态不同于心肌梗死时的凹面向下,也不同于急性心包炎时的凹面向上,而呈“尖峰”状,然后急剧下降,颇具特色(附图)。而心肌酶谱恒为正常。(3)Q-T间期正常。(4)常规和动态心电图可查见多源性室性期前收缩、短阵室性心动过速。患者晕厥发作时心电图记录为持续性多形性室性心动过速,但无尖端扭转现象,室速可由R-on-T引发,发作前未见心率、Q-T间期和心肌缺血改变。少数患者室性心动过速自行中止,多数患者需电击或药物才能恢复窦性心律。接受心脏电生理检查的7例中有4例H-V间期延长,7例均诱发出多源性室性心动过速,4例同时诱发出心室颤动。这一病症被命名为Bru-gada综合征。     

Ajmaline试验诊断心电图表现正常的Brugada综合征

本工作为第一作者受国家留学基金委资助在 Ｓｔ．Ｇｅｏｒｇ Ｋｒａｎｋｈａｕｓ,Ｈａｍｂｕｒｇ,Ｇｅｒｍａｎｙ学习期间完成 近来,作为特发性心室颤动（室颤）的一种特殊原因,Ｂｒｕ－ｇａｄａ综合征已越来越引起人们的重视。它的诊断主要依据体表心电图胸前Ｖ１－Ｖ３导联ＳＴ段持续性的抬高,右束支阻滞（ＲＢＢＢ）样图形以及有晕厥、有心电图记录的室颤或室性心动过速（室速）的病史。但这类患者的ＳＴ段抬高可以是一过性的,因而对诊断造成困难。兹报道静脉注射Ａｊｍａｌｉｎｅ（１ｍｇ／ｋｇ）对２例基础心电图正常的室速患者…     

Brugada波与Brugada综合征

1991年Brugada兄弟首次报道了心电图出现右束支传导阻滞(RBBB)伴有V1～V3导联ST段抬高及猝死．引起医学界高度重视,后被称为Brugada综合征。此后,国内外文献有不少类似病例报道．有些病例既有典型心电图改变,又发生过心脏性猝死(死亡或存活),符合Brugada综合征;另有一些仅有典型的心电图改变而无症状,或伴有器质     

Brugada综合征的药物治疗

到目前为止,埋藏式心脏转复除颤器是唯一被公认能够有效预防Brugada综合征患者室性心动过速/心室颤动(VT/VF)引起的心源性猝死的治疗措施,但其存在一些问题,而药物治疗则对这些问题有一定帮助。奎尼丁是目前研究和报道最多的药物,能够相对良好地控制VT/VF的发作;静脉使用异丙肾上腺素则在控制电风暴上显示了其有效性;同时,一小部分报道显示,其他一些药物如西洛他唑、苄普地尔、丙吡胺、地诺帕明、索他洛尔、稳心颗粒等,在Brugada综合征的治疗上也可能有着一定的价值。     

Brugada波与Brugada综合征

Brugada综合征是一编码离子通道基因异常所致的家族性原发心电活动紊乱性疾病。自 1991年BrugadaP和BrugadaJ报告于临床以来 ,由于其右胸前导联特征性心电图改变和猝死病症 ,所以多年来一直是心血管病基础和临床研究的热点。 2 0 0 1年HurstJW将Brugada描述的V1～V3 导联特征性心电图改变称为“Brugada波”。本文仅对Brugada波的特点、鉴别和与Brugada综合征的关系等临床医师关注的具体问题简要讨论如下。一、Brugada波的心电图特点1、典型心电图表现Brugada波由抬高的ST段和“右束支阻滞”(RBBB)共同组成 ,典型者常伴倒置的T波 ,…     

Unusual response to the ajmaline test in a patient with Brugada syndrome.

We present a Brugada syndrome patient who suffered an aborted sudden death. The ajmaline test (1 mg/kg body weight) induced accentuated alternans ST-segment elevation in V1-V2 without ventricular arrhythmias. It could represent silent ischaemia not detected before, failure of myocardial regions to repolarize in alternate beats due to transmural dispersion of conduction and refractoriness in the right ventricular outflow tract or a rate dependent sodium channel block by ajmaline. We need more studies to know whether this electrocardiographic sign is a risk factor for life-threatening ventricular arrhythmias in Brugada syndrome patients.     

Negative flecainide test in Brugada syndrome patients with previous positive response.

Class I antiarrhythmic drug infusion has been established as the standard test to unmask Brugada syndrome. This report presents two patients with Brugada syndrome with positive flecainide response which was not reproducible in a subsequent test.     

高位右侧胸前导联心电图在普罗帕酮激发试验中的价值

目的 报道10例疑诊Brugada综合征（BS）患者的普罗帕酮激发试验的结果，并探讨高位右侧胸前导联心电图在普罗帕酮激发试验中的价值。方法 对心室颤动（室颤）获救、晕厥或晕厥先兆者，以及无症状但有阳性猝死家族史者，经详细的病史询问、体格检查、心电图、X线胸片和超声心动图检查排除器质性心脏病，收集10例疑诊BS患者（观察组）。将同期因阵发性室上性心动过速行射频导管消融术、无器质性心脏病依据和猝死家族史的15例患者设为对照（对照组）。观察组与对照组均进行普罗帕酮激发试验。在普罗帕酮激发试验中同时描记常规12导联心电图与高位右侧胸前导联心电图。结果 观察组10例患者中普罗帕酮试验阳性者8例。常规与高位右侧胸前导联同时呈阳性改变者1例；常规导联阳性、高位右侧胸前导联阴性者3例；常规导联阴性或可疑、高位右侧胸前导联阳性者4例。若仅凭常规胸前导联心电图，阳性例数为4例，结合常规导联与高位右侧胸前导联心电图，阳性例数增加为8例。对照组无一例达阳性诊断标准。结论 普罗帕酮激发试验用于诊断Bs的敏感性与特异性均较好，加做高位右侧胸前导联心电图可提高诊断阳性率，且极为便捷。普罗帕酮试验中可考虑加做高位右侧胸前导联心电图，以避免漏诊。     

Propranolol intoxication revealing a Brugada syndrome

This is the first report of Brugada syndrome revealed by beta-blocker intoxication. A 24-year-old healthy man ingested propranolol (2.28 g) to commit suicide. After early gastric lavage, electrolytes, cardiac enzymes, chest X-ray, and echocardiography were normal. Dosages of psychotropic drugs were negative. ECG showed a typical coved-type pattern of Brugada syndrome. Follow-up showed partial ECG normalization of the discrete saddleback-type pattern. The ajmaline- test confirmed Brugada syndrome. These ECG modifications may be explained by the stabilizing membrane effect of high concentration of propranolol and/or inhibition of ICaL. This case illustrates the possible deleterious effects of beta-blockers in patients with Brugada syndrome.     

Cycle length-dependent repolarization changes during atrial fibrillation in the Brugada syndrome

This is a case report of a patient with Brugada syndrome who developed paroxysmal atrial fibrillation. During the episode, beat-to-beat changes in ventricular repolarization were observed. These changes were a paradoxical ST-segment alteration after a short-coupled ventricular beat. These findings, not reported before, may be helpful for the diagnosis of this syndrome.     

Electrocardiographic methods for diagnosis and risk stratification in the Brugada syndrome

The Brugada syndrome (BrS) is a malignant, genetically-determined, arrhythmic syndrome manifesting as syncope or sudden cardiac death (SCD) in individuals with structurally normal hearts. The diagnosis of the BrS is mainly based on the presence of a spontaneous or Na + channel blocker induced characteristic, electrocardiographic (ECG) pattern (type 1 or coved Brugada ECG pattern) typically seen in leads V1 and V2 recorded from the 4th to 2nd intercostal (i.c.) spaces. This pattern needs to be distinguished from similar ECG changes due to other causes (Brugada ECG phenocopies). This review focuses mainly on the ECG-based methods for diagnosis and arrhythmia risk assessment in the BrS. Presently, the main unresolved clinical problem is the identification of those patients at high risk of SCD who need implantable cardioverter-defibrillator (ICD), which is the only therapy with proven efficacy. Current guidelines recommend ICD implantation only in patients with spontaneous type 1 ECG pattern, and either history of aborted cardiac arrest or documented sustained VT (class I), or syncope of arrhythmic origin (class IIa) because they are at high risk of recurrent arrhythmic events (up to 10% or more annually for those with aborted cardiac arrest). The majority of BrS patients are asymptomatic when diagnosed and considered to have low risk (around 0.5% annually) and therefore not indicated for ICD. The majority of SCD victims in the BrS, however, had no symptoms prior to the fatal event and therefore were not protected with an ICD. While some ECG markers such as QRS fragmentation, infero-lateral early repolarisation, and abnormal late potentials on signal-averaged ECG are known to be linked to increased arrhythmic risk, they are not sufficiently sensitive or specific. Potential novel ECG-based strategies for risk stratification are discussed based on computerised methods for depolarisation and repolarisation analysis, a composite approach targeting several major components of ventricular arrhythmogenesis, and the collection of large digital ECG databases in genotyped BrS patients and their relatives.Abbreviations: AP, action potential; ARI, activation-recovery intervals; BrS, Brugada syndrome; ECG, electrocardiogram; EPS, electrophysiology study; ICD, implantable cardioverter-defibrillator; IHD, ischaemic heart disease; LBBB, left bundle branch block; MAP, monophasic action potential; MI, myocardial infarction; PCA, principal component analysis; RVOT, right ventricular outflow tract; SAECG, signal-averaged electrocardiogram; SCD, sudden cardiac death; SNP, single-nucleotide polymorphism; VF, ventricular fibrillation; VT, ventricular tachycardia; WT, wavelet transform