DCG监护对Brugada综合征的诊断意义

Brugada综合征是一种常染色体显性遗传性疾病，男性多见，有特征性的心电图表现、伴有多形性室速或室颤等恶性心律失常诱发的晕厥、心脏骤停或猝死。患者多有类似表现的家族史，心电图表现为正常的QT间期、右束支传导阻滞、右胸导联ST段特征性抬高(呈下斜型或马鞍型)，发作性的室速、室颤等恶性心律失常，系Brugada 1992年首次报告。回顾分析1992～2002年10年来我院8224例动态心电图(DCG)监护资料，发现3例符合或基本符合Brugada综合征的临床诊断标准，报告于后。     

植入型心律转复除颤器治疗Brugada综合征一例

Brugada综合征是一种与心脏性猝死相关的离子通道疾病,特发性多形室性心动过速（室速）、心室颤动（室颤）致猝死是Brugada综合征最严重的临床后果.目前,植入型心律转复除颤器（ICD）是惟一已证实对Brugada综合征治疗有效的方法。本院成功抢救以多次晕厥为首发表现的Brugada综合征伴室颤1例,并ICD治疗。随访14个月,患者自觉有2次发作并经程控证实为室颤发作,均ICD体内除颤成功。     

新胸导联在诊断Brugada综合征中的应用

Brugada综合征是1992年Brugada兄弟报道的一种新疾病,表现为晕厥、猝死,心脏结构正常标准心电胸前V1-V3导联ST段呈下斜型或马鞍型抬高,晕厥或猝死由室速或室颤引起。Brugada综合征标准心电表现变异大,间隙期间可完全正常,诊断困难。该研究评价自行设计的新胸导     

Brugada综合征患者心律失常及临床特点分析

目的通过动态心电图(Holter)检查、置入式心脏复律除颤器(ICD)分析并综合患者临床情况,研究Brugada综合征(Brs)患者心律失常事件发生特征及临床特点。方法对我院近5年诊断的12例Brs住院患者的临床情况进行随访观察;均行Holter检查分析心律失常事件发生特征;根据ICD的随访资料,分析室颤发作的时间特征。结果 Holter显示,其中8例患者室性期前收缩总数为0～80个/d,另外4例以夜间至清晨为主的频发室性期前收缩及短阵室性心动过速(其中1例伴有阵发性心房颤动;1例伴有频发房性期前收缩及房性心动过速,并且夜间心率缓慢29次/min伴交界性逸搏心律)。9例患者置入ICD后随访6～50个月,70次室颤发作中92.8%集中在22:00～07:00。12例患者均有晕厥病史,9例在住院期间发生室速/室颤,2例有猝死家族史,随访期间2例猝死。结论 Brs患者的室速/室颤发作多集中在夜间和凌晨;伴有房性心律失常和缓慢心律失常的Brs患者室速/室颤发生率更高。确诊的Brs患者发生心悸等不适时,应全面了解心律失常发生的情况,并酌情给予相应的治疗。     

常规心电图检查发现的Brugada波发生率与临床

目的初步调查Brugada波与Brugada综合征的发生率及与临床的关系。方法通过回顾调查近3年我院心电图室常规检查的52291份心电图，严格按诊断标准筛选有Brugada波的心电图，并对部分患者进行随访。结果共筛选出17例Brugada波患者，占总数的0．33％o，其中男性16例，女性1例，符合Brugada综合征的患者4例。结论Brugada波在临床上并非少见，但符合Brugada综合征即伴有反复晕厥、猝死的相对较少，是潜伏在体格健康的正常人群中的危险疾病。     

Brugada综合征患者猝死预警新指标(109)

正伴有严重室颤发作的Brugada综合征患者行心外膜电压标测时,可在右室流出道部位发现低电压区,而有Ⅰ型Brugada波的患者在右室流出道部位可存在异常的心电图,提示两者有内在相关性。2016年5月举行的HRS年会上,美国费城杰弗逊大学的Pavri等报告,有心电图Ⅰ型Brugada波表现的患者,心电图振幅对心脏性猝死有预警作用。该研究入组117例有自发Ⅰ型Brugada波的患者;A组为室颤组:26例有室颤发生但为幸存者,B组为晕厥组:共40例;     

Brugada综合征

Brugada综合征是一种遗传性心脏离子通道疾病，其临床特征为：①心脏结构正常；②特征性右胸导联（V1～V3）ST段呈下斜型（coved type）或马鞍型（saddleback type）抬高，伴有或不伴有右束支阻滞；③致命性室性快速性心律失常[室性心动过速（简称室速）或心室颤动（简称室颤）]发作引起反复晕厥和猝死。多数发生于青年男性，常有晕厥或猝死家族史，目前唯一被证明能有效预防Brugada综合征发生猝死的措施是埋藏式心脏转复除颤器（ICD）。     

Brugada综合征伴发的室上性心律失常

目的：报道Brugada综合征伴发的多种室上性心律失常。方法：对符合Brugada综合征诊断标准的46例患者行动态心电图检查，记录心律失常发作类型，部分行电生理检查与射频消融治疗。结果：男性39例，女性7例。21例有家族史，23例有晕厥史，其中3例经心肺复苏。46例中5例并发阵发性室上性心动过速（阵发性室上速，4例为显性预激综合征）；3例室上速与心房扑动（房扑）并存；2例室上速与室性心动过速（室速）并存；14例房性心动过速（房速）、房扑和心房颤动（房颤）；2例房速伴三度房室阻滞；1例房速与室速并存；11例多形室速；5例单形室速；1例室颤电风暴并三度房室阻滞；1例室速与三度房室阻滞并存；1例室速与三度房室阻滞、房颤并存。39例行射频消融治疗成功32例，4例并发三度房室阻滞者植入心脏永久起搏器，4例植入心律转复除颤器。结论：除室速、室颤外，Brugada综合征可发生房颤、房扑、室上速、三度房室阻滞等多种心律失常，且两种以上心律失常可以共存。Brugada综合征心脏钠通道基因变异引起细胞膜钠通道功能异常不仅存在于希氏一浦肯野系统和心室肌，亦可存在于心房肌及房室结，引发多种类型心律失常。     

Brugada波与Brugada综合征的诊断与鉴别

1 定义 类似右束支传导阻滞(RBBB)伴持续性右胸导联ST段抬高的心电图特征,称为Brugada波。类似RBBB、持续性右胸导联ST段抬高伴发室速、室颤和猝死,称为Brugada综合征。 2 Brugada波回顾     

第43课J波综合征由复极还是除极机制所致——一个争论的问题

J波屈折发生在QRS-ST连接处(又叫Osborn 波),1953年首次报道,见于多种状态如低温,Brugada 综合征(BrS)和早复极综合征. BrS与室性心动过速或心室颤动引起的晕厥和(或)猝死相关联.当自发或Ⅰ类抗心律失常药物激发下产生穹窿型ST段抬高的Ⅰ型心电图时即可诊断. 有关从BrS获得的诊断性穹窿型心电图的产生机制,实验和临床电生理专家长期以来一直存在争论.有关Ⅰ型Brugada 心电图形成的机制出现两大学说,即"除极异常学说"和"复极异常学说".     

Brugada综合征的电生理检查和置入性心脏复律除颤器治疗

目的　探讨Brugada综合征的电生理检查和置入性心脏复律除颤器 (ICD)治疗临床研究。方法　心电图自发性或普罗帕酮药物试验表现为Brugada波的 10例患者行电生理检查 ,均为男性 ,平均年龄 (41± 10 )岁 ,经超声心动图和冠状动脉造影检查未发现器质性心脏病。电生理检查诱发心室颤动 (室颤 )并对有条件者置入ICD治疗。结果　 3例有家族性心脏猝死史 ,4例有反复晕厥史 ,其中 2例晕厥发作时心电图记录到室颤。电生理检查 ,AH和HV间期分别为 5 0～ 12 4 (86± 2 1)ms和 4 1～ 84 (5 8± 15 )ms ,4例晕厥者诱发室颤 ,1例心悸者诱发房室折返性心动过速 ,3例有自发性或诱发心房颤动。 4例诱发室颤者中 ,3例置入ICD ;另 1例因经济原因未置入ICD ,随访中发生猝死。1例诱发房室折返性心动过速者作左侧房室旁路射频导管消融治疗。结论　有晕厥症状的Brugada综合征患者 ,经电生理检查 ,室颤有很高的诱发率 ,是猝死的高危人群 ,为了防止猝死应置入ICD治疗。     

Brugada综合征快速心律失常的发病机理与射频消融治疗

目的探讨Brugada综合征ST-T抬高与快速心律失常的发病机理和射频消融治疗。方法选择体表心电图V1~V3相邻2个导联ST-T下斜或马鞍型抬高≥0.2mV,且有心律失常者按常规行心脏电生理检查和射频消融。结果38例患者,男31例,女7例,年龄38.27±13.91岁。17例有晕厥或黑矇,3例经历心肺复苏。23例为Ⅰ型Brugada图形,10例为Ⅱ型,5例为Ⅰ、Ⅱ、Ⅲ型交替,均合并不同类型的心脏传导异常。10例为单形(2例)或多形(8例)室性心动过速,1例为心室颤动电风暴,5例为室性与房性心律失常并存,5例为单纯室上性心动过速(4例为显性预激综合征),3例为室上性心动过速和心房扑动,14例为房性早搏、房性心动过速和心房颤动。射频消融即刻成功32例(成功率84%),失败6例,未发生并发症。消融成功者心律失常消失后即刻,ST-T抬高未见改变。而室性早搏、房性早搏伴差传、His束传导阻滞、间歇预激旁道、心室起搏与静脉滴注异丙肾上腺素可使ST-T抬高幅度改变或逆转。随访5.72±2.03年,1例失访,5例心律失常复发,1例猝死,3例安装心脏永久起搏器,1例安装心脏转复除颤器。结论Brugada综合征常见心脏传导异常,ST-T抬高继发于心脏传导系统疾病与除极顺序改变,而非原发心内、外膜复极离散与2相折返,射频消融可有效防治Brugada综合征的多种快速心律失常。     

Brugada syndrome--an under-recognized electrical disease in patients with sudden cardiac death

In 1992, Brugada and Brugada described 8 patients with a history of aborted sudden death and a distinct ECG pattern of right bundle-branch block with ST segment elevation in leads V1-V3 and normal QT interval in the absence of any structural heart disease. It is called Brugada syndrome now and is believed to be responsible for 4-12% of all sudden deaths and around 20% of deaths in patients with structurally normal hearts. Although this syndrome is observed worldwide and the exact prevalence is unknown, it is more common in the Southeast Asian countries. Repeated syncope, ventricular fibrillation, and sudden cardiac death have been reported in patients with Brugada syndrome. The clinical presentation of Brugada syndrome is distinguished by a male predominance and the appearance of arrhythmic events at an average age of 40 years. The Brugada syndrome is inherited in an autosomal dominant manner with incomplete penetrance and an incidence ranging between 5 and 66 per 10,000. The surface ECG manifestations of the syndrome can transiently disappear, but can be unmasked by potent sodium channel blockers in some cases. Mutations of the cardiac sodium channel SCN5A have been detectable in <20% of patients with Brugada syndrome. Recent genetic studies have confirmed the genetic heterogeneity of the disorder. Antiarrhythmic drugs appear to be of little use in prolonging survival and in preventing recurrences of ventricular arrhythmias. To date, implantable cardioverter defibrillator remains the best therapy to prevent sudden death in these patients.     

Prevalence of Brugada sign in patients presenting with palpitation in southern Iran.

AIMS: Brugada syndrome is a cardiac channel abnormality that is associated with a high risk of ventricular fibrillation and sudden cardiac death and characterized by an electrocardiographic pattern of right bundle branch block and transient or persistent ST-segment elevation in leads V1-V3. No data regarding the frequency of Brugada syndrome exist in an Iranian population. The aim of this study was to determine the frequency of Brugada-type ECG pattern in southern Iran. METHODS AND RESULTS: All patients presenting with palpitation were enrolled in the study. A Brugada-type ECG pattern was determined according to the criteria recommended by European Heart Association Molecular Basis of Arrhythmias Study Group. A total of 3895 patients (mean age 38.2 +/- 11.9 years, 54% women) met all study criteria. One hundred patients (2.56%) had Brugada-type ECG pattern. Of these, 21 patients (0.54%) had definite Brugada sign (Type 1 or Types 2 and 3 with conversion to Type 1 following procainamide test). Of 21 patients with definite Brugada sign, eight had Brugada syndrome, four had history of syncope, two had coved-type ECG in the family, one had polymorphic ventricular tachycardia, and one had history of sudden cardiac death in the family. Five patients underwent ICD implantation. The incidence of a Brugada-type ECG pattern was 2.43% in subjects between 17 and 30 years and 0.13% in subjects >30 years (P = 0.01). CONCLUSION: Frequency of Brugada sign in an Iranian population presenting with palpitation is greater than some European countries and lower than a Japanese urban population.     

Diagnosis and long-term follow-up of the Brugada syndrome in patients with idiopathic ventricular fibrillation.

AIMS: Some patients with idiopathic ventricular fibrillation may suffer from the Brugada syndrome. The diagnostic criteria for the Brugada syndrome are uncertain and arbitrarily set. Therefore, we studied the prevalence of the Brugada syndrome using various diagnostic criteria and long-term follow-up in 37 idiopathic ventricular fibrillation patients. METHODS AND RESULTS: Idiopathic ventricular fibrillation was diagnosed after thorough clinical evaluation in 37 survivors of an out-of-hospital cardiac arrest referred to our institute (UMC Utrecht). Retrospectively, nine patients (24%, group I) were classified as potentially having the Brugada syndrome based on the presence of (in)complete right bundle branch block and ST-segment elevation in leads V(1)-V(3)of > or =1 mm. Only three patients (8%, group II) showed (in)complete right bundle branch block and > or =2 mm ST-segment elevation. With the intermittent presence of these ECG features and/or their (re)appearance with class I antiarrhythmic drugs included as criteria, the percentage of the Brugada syndrome was attenuated in group I (2/37; 5%) and group II (1/37; 3%). Sixteen (43%) of all idiopathic ventricular fibrillation patients (mean follow-up 77+/-41 months) had a recurrent episode of syncope, ventricular tachyarrhythmias or sudden death. Recurrence rate was 3/9 (33%) in Brugada patients group I, 2/3 (66%) in group II and 13/28 (46%) in patients without the Brugada syndrome (P=ns). CONCLUSIONS: Depending on the diagnostic criteria used, the Brugada syndrome was observed in 3% to 24% of patients with idiopathic ventricular fibrillation, underlining the importance of defining the precise diagnostic criteria in these patients. For all idiopathic ventricular fibrillation patients, the ventricular tachyarrhythmia recurrence rate was substantial during an average follow-up of more than 6 years.     

普罗帕酮试验引起Brugada综合征心室颤动(附一例报告)

探索普罗帕酮试验在Brugada综合征中的作用及安全性。患者有家族性夜间猝死史 ,近 1 9个月来反复发作性夜间晕厥5次 ,两次猝死未遂 ,一次记录到的心电图为心室颤动 (VF) ,其超声心动图、冠状动脉造影、左右心室造影正常 ,电生理检查心脏传导系统功能正常 ,右心室程序电刺激可诱发VF ,诊断不明。静脉用普罗帕酮 2mg/kg后 30min出现典型Brugada综合征心电图改变 ,并诱发VF。结论 :普罗帕酮可作为诊断Brugada综合征的工具 ,同时应警惕其致心律失常作用     

异常J波、Brugada综合征与特发性Brugada心电图征的临床与心电图特征

对比分析异常J波 2 1例、Brugada综合征 8例与特发性Brugada心电图征 11例的临床及心电学特点。结果 :①特发性异常J波在肢导联或 (和 )胸导联可见正向异常J波 [除aVR(部分患者aVL)外 ],其波幅较低而分布较广 ,一般V1～V2 导联不出现J波 ,若出现则JV1 ～V2R ,TV1 ～V2 (V3) 倒置或直立 ,前者常出现恶性快速性室性心律失常而发生晕厥或猝死 ,后者则无晕厥或猝死及恶性心律失常发作。结论 :异常J波和Brugada综合征及特发性Brugada心电图征是具有不同临床及心电学特点的临床实体。     

Síncope e padrão de Brugada intermitente

Brugada syndrome is a rare syndrome, with an estimated prevalence in Europe of 1-5/10 000 population, whose initial clinical presentation can be sudden death. Although it has a characteristic electrocardiographic pattern, this can be intermittent. The authors present the case of a 32-year-old man, with no family history of syncope or sudden death, who went to the emergency department for syncope without prodromes. The initial electrocardiogram (ECG) in sinus rhythm documented an isolated and non-specific ST-segment elevation in V2. During further diagnostic studies, a repeat ECG revealed type 1 Brugada pattern. This pattern was later seen in a more marked form during a respiratory infection. The patient subsequently underwent electrophysiological study, followed by implantation of an implantable cardioverter-defibrillator (ICD), with an episode of ventricular fibrillation converted via ICD shock two months after implantation.     

Brugada syndrome in pure black Africans

Aims: Although all races are concerned with the Brugada syndrome, no case has ever been reported among black Africans. We describe five different cases in this specific group of populations.
Methods and Results: In all patients, Brugada syndrome was identified after detailed noninvasive and invasive evaluations. Sex ratio was four males for one female. Convulsive syncope was noticed in 1 patient with a family history of sudden death. Diagnostic coved-type pattern was observed spontaneously in the normal position of right precordial leads in 3 patients and in a higher position of leads in 3 patients. Sixty percent had first-degree atrioventricular block. An ajmaline test was performed in 4 patients and was positive either in normal position of leads or in superior position in all of them. Sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) was inducible during programmed ventricular stimulation in 3 patients. Right ventricular cineangiography found localized apical hypokinesia with preserved systolic function in 1 patient. Automatic cardioverter defibrillator was implanted in 2 patients. SCN5A was not found in any of the patients.
Conclusion: These observations demonstrate that Brugada syndrome is also present in black African populations, and increasingly reported cases of apparent sudden death in the sub-Saharan part of the world need to rule out cardiac electrical disturbance such as Brugada syndrome.