Brugada综合征临床特征

目的总结分析中国十余年来报道的Brugada综合征患者的临床特征与治疗情况。方法通过《中国学术期刊全文数据库》和《维普全文数据库》获取资料,对1998年1月一2013年12月国内期刊报道的Bmgada综合征患者发病年龄、区域分布、临床特征与治疗进行等进行分析。结果共收集Brngada综合征患者476例,男性占94.33%,主要分布于沿海与经济较发达地区。以晕厥症状或意识丧失为主要首发临床表现者,占65.54%,30-50岁发病者占68.91%,为发病高峰。心电图特征以I型为主,占68.07%。院外死亡或人院抢救失败者占25.32%,植入ICD仅占总患者18.28%。结论中国Brngada综合征以男性患病为主,主要分布于我国沿海与经济较发达的地区,晕厥与猝死率较高,死亡率高,ICD植入率较低。     

置入埋藏式心脏转复除颤器治疗Brugada综合征并心室颤动一例

患者男,47岁。因发作性神志不清、抽搐入院,误诊为症状性癫痫,经行心电图检查示V1、V2导联ST段抬高,症状发作时心电监护示心室颤动,诊断为Brugada综合征。经置入埋藏式心脏转复除颤器,症状未再发作。     

典型Brugada综合征一例

一名33岁男性,因“间断心悸伴胸闷”入本院,外院ECG呈典型Ⅰ型Brugada样改变.一同胞兄弟28岁时猝死.本院ECG呈典型Ⅱ型Brugada样改变,电生理检查可诱发心室颤动.患者Brugada 综合征诊断明确,且属高危患者,随后植入埋藏式心脏转复除颤器.     

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）。     

埋藏式心脏复律除颤器在Brugada综合征中的应用

目的观察埋藏式心脏复律除颤器(implanted cardioverter-defibrillators,ICD)在治疗Brugada综合征中的作用。方法对2005年1月至2009年12月在广东省人民医院确诊Brugada综合征的11例患者植入ICD后的临床资料进行回顾性分析。结果植入ICD进行一级预防的患者4例(36.36%),临床表现为持续性室性心动过速或心室颤动的二级预防的患者7例(63.64%)。起搏模式:6例(54.55%)单腔ICD,5例(45.45%)双腔ICD。所有患者术中测试ICD的起搏参数并诱发心室颤动,采用20 J一次电击复律成功。随访(17.0±12.7)个月,无漏访。一级预防组和二级预防组发生恶性室性心律失常的患者分别为2例和3例;两组室性心动过速发作阵次比例[80%(4/5)vs.90.3%(28/31),P>0.05]和心室颤动发作阵次比例[20%(1/5)vs.9.6%(3/31),P>0.05]比较,差异无统计学意义;两组抗心动过速起搏成功率[75%(3/4)vs.96%(24/25),P>0.05]及除颤治疗成功率[100%(2/2)vs.100%(4/4),P>0.05]比较,差异无统计学意义。术中及术后未见植入ICD相关并发症。结论对Brugada综合征患者植入ICD预防心脏性猝死是安全有效的。     

经皮穿刺腋静脉植入埋藏式心脏转复除颤器治疗儿童Brugada综合征一例

患者男性,11岁,因近3年内晕厥、抽搐3次入院。平素体健,无猝死的家族史。心电图表现为Ⅰ型Brugada波,心内电生理检查诱发出心室颤动,诊断为Brugada综合征。经左侧腋静脉成功植入Medtronic 7230Cx埋藏式心脏转复除颤器,随访2个月,埋藏式心脏转复除颤器无放电,患者一般情况良好。     

Brugada综合征的诊治

目的提高对Brugada综合征的认识及诊断水平,探讨植入型心律转复除颤器(ICD)的治疗时机。方法通过对临床症状、特异性心电图、电生理检查及药物激发实验等综合分析,诊断3例Brugada综合征,其中2例予安装ICD治疗,1例未予治疗。结果3例随诊6～8个月,病例2曾出现一次胸闷后电击,后证实为室颤。其余2例无特殊不适。随诊时间较短,尚待进一步评价。结论对于在夜间发生的晕厥或猝死,无器质性心脏病及心电图有特异表现者,应高度怀疑Brugada综合征,尽早进行电生理及药物激发实验等检查以明确诊断。确诊者惟一有效的治疗为ICD。     

Brugada综合征一例

患者男性,40岁,因突发晕厥就诊,入院心电图V_1~V_3导联ST段抬高,T波倒置,留观期间突发心室颤动,多次复查心电图V_1~V_3导联ST段抬高,T波倒置、低平。考虑诊断为Brugada综合征,外院基因检测证实有基因突变,后该患者转上级医院植入埋藏式心脏转复除颤器。     

植入埋藏式心脏转复除颤器的临床应用与随访

目的报道13例埋藏式心脏转复除颤器(implantable cardioverter defibrillator,ICD)的临床应用与随访结果。方法对置入10例单腔ICD及3例双腔ICD患者进行电话询问和门诊随访,通过心电图、动态心电图及ICD存储的资料,对患者病情和ICD工作情况进行分析。结果 13例患者共发作持续性室性心动过速/心室颤动(ventricular tachycardia/ventricular fibrillation,VT/VF)38次(持续性VT 36次,VF 2次),其中37次治疗成功,1次失败。非持续性VT 121次,发作均自行停止。36次持续性VT中33次经抗心动过速起搏(antitachycardia pacing,ATP)终止,2次经低能量复律(CV)终止,1次VT治疗过程中加速为VF,由高能量除颤(DF)终止。1例共2次阵发性心房颤动心室率超过设置的VT频率,ICD发生误识别,给予ATP治疗。1例术后3个月出现心律失常"电风暴"。共有3例术后因VT发作频繁而服用胺碘酮和(或)美托洛尔,并根据心律失常发作情况调整用药剂量。结论 ICD植入后应加强随访,及时调整工作参数,同时给予患者心理治疗、对提高ICD的治疗效果非常重要。     

Brugada综合征2例报告

1989年,Martini等报道6例室性颤动生还病人时,描述了心电图呈右束支阻滞图形及Vl～3ST段上抬变化[1],1992年,Brugada兄弟报道了8例心电图呈上述表现的猝死复苏生还者,认为这是一种特殊类型、属于心脏功能异常的临床心电综合征[2],以后病例逐渐增多,并于1999年定名为Brugada综合征[3].研究表明,美国每年大于30万的猝死者中有5%～12%死于多形性室速和室颤,其中半数以上与Brugada综合征有关[4,5].     

Dynamic electrocardiographic changes after aborted sudden death in a patient with Brugada syndrome and rate-dependent right bundle branch block

A 37-year-old man with Brugada syndrome and dynamic changes of the ST-segment morphology observed after an episode of aborted sudden death is described. On admission, after 3 syncopal episodes during nighttime, his electrocardiogram showed right bundle branch block (RBBB) with a J-point elevation of 0.6 mV in lead V 2 . Changes observed in the following days included a diminished J-point elevation and intermittent "saddle-back" type of morphology. During a previous 2-year follow-up, intermittent, complete, acceleration-dependent RBBB was documented. Right ventricular intracavitary tracings showed an RS pattern with a broad S wave in the unipolar electrogram; the time of onset of intrinsic deflection in this electrogram was 60 milliseconds. To our knowledge, this is the first report of an intracavitary demonstration of complete RBBB in Brugada syndrome.     

植入型心律转复除颤器治疗的适应证

致命性室性心律失常（室性心动过速／心室颤动）是心脏性猝死（SCD）的主要原因，美国每年约40万人死于此症。一系列多中心临床试验证明ICD是优于药物的有效治疗方法，明显降低SCD发生率。美国每年上万人安装ICD，其数量呈直线上升趋势。我国由于经济条件和认识水平普及的限制，采用ICD治疗的患者甚少，但近年来逐渐增多，1年约100例患者安装ICD。为提高医生和患者对应用ICD的认识及使用规范化，两个学会的ICD专家组于2001年11月在北京召开研讨会，就ICD适应证、多中心试验结果、植入技术、心动过速的识别和治疗、并发症、随诊等专题，结合我国实际情况，进入了深入和认真的讨论，并提出建议。     

Incidence, clinical implications and prognosis of atrial arrhythmias in Brugada syndrome.

AIMS: This single-centre prospective study was designed to determine the incidence, therapeutic implications and prognosis of atrial arrhythmias (AA) in patients with Brugada syndrome (BS). METHODS AND RESULTS: Fifty nine consecutive patients with BS and 31 age and gender-matched controls underwent an electrophysiological exploration and were followed-up during 34+/-13 months. The final AA incidence was 20% in BS patients vs 0% in controls (p < 0.01). Ventricular inducibility was significantly related to a history of AA (p = 0.02). The incidence of AA in patients with a spontaneous electrocardiogram of BS was 26% vs 10% in patients with a flecainide-induced electrocardiogram (p < 0.05). In patients with an indication of implantable cardioverter defibrillator (ICD), the incidence of AA reached 27% vs 13% in patients with BS but without ICD indication (p < 0.05). Inappropriate shocks due to AA episodes were observed in 14% of ICD patients vs 10.5% of appropriate shocks. Multivariate analysis identified the implantation of a single-chamber device as an independent predictive factor of inappropriate ICD discharges (p < 0.05). CONCLUSION: BS patients exhibit an abnormally high proportion of AA. Our data strongly suggest a more advanced disease process in BS patients with spontaneous AA. Careful programming of single-chamber ICD should be recommended to avoid inappropriate discharges.     

Ablation of atrial fibrillation in Brugada syndrome patients with an implantable cardioverter defibrillator to prevent inappropriate shocks resulting from rapid atrial fibrillation

Background

Inappropriate shocks resulting from atrial tachyarrhythmias are highly problematic for patients with an implantable cardioverter defibrillator (ICD). We aimed to determine the effectiveness of catheter ablation of atrial fibrillation (AF) in preventing inappropriate shocks due to rapid AF in patients diagnosed with Brugada syndrome (BS) who were implanted with an ICD.

Methods

We performed AF ablation in 5 BS patients with ICDs who experienced inappropriate shocks caused by rapid paroxysmal AF and in a BS patient scheduled to determine an indication of an ICD implantation who frequently experienced rapid AF.

Results

Although 2 patients underwent a 2nd ablation procedure because of AF recurrences, 5 of the 6 patients were finally free from AF after their last procedure during a median follow-up period of 43.2 months. No further inappropriate shocks caused by rapid AF occurred after the 1st ablation session in any of the patients. A patient developed a ventricular fibrillation storm during his electrophysiological study following the ablation procedure, and then was implanted with an ICD.

Conclusions

AF ablation in BS patients may be reasonable to prevent inappropriate ICD shocks resulting from rapid AF. However, ventricular extrastimuli just after the ablation had better be avoided in them.     

努力降低中国恶性室性心律失常的死亡率

室性心律失常包括室性早搏（室早）、室性心动过速（室速）、心室扑动（室扑）和心室颤动（室颤）。室早和室速是常见的心律失常,可出现在心脏结构正常者,更易发生于器质性心脏病患者。2005年世界卫生组织公布的资料表明,全球死于心脑血管疾病的1700万人群中,心脏性猝死（sudden cardiacdeath,SCD）占40％-50％。室速、室扑和室颤都属于恶性心律失常。临床研究证实,恶性心律失常是导致SCD的最主要原因,为65％-85％。     

植入型心律转复除颤器治疗Brugada综合征五例随访

目的报道5例植入型心律转复除颤器(ICD)的Brugada综合征患者的随访结果。方法5例隐匿性Brugada综合征男性患者,平均年龄(41．60±10．14)岁,植入单腔ICD后每3个月临床随访1次,体外程控分析ICD记录的各种心律失常发作的时间、类型、治疗方式和结果。结果4例患者经钠通道阻滞剂激发试验确诊,另1例经新胸导联检查确诊(该例的诊断参考了单其俊等“新胸导联”标准作出的诊断,是否恰当有待于其他组学者的研究证实)。5例患者植入ICD前均反复发作晕厥,其中4例记录到心室颤动(室颤),3例电生理检查诱发出室颤。平均随访(22±18)个月ICD共记录到75次室颤发作,其中61次室颤触发ICD 86次放电,均成功转复窦性心律；其余14次室颤自行终止。例1出现除颤阈值增高,仍有4次短暂的晕厥发作；例4因房颤导致26次ICD误放电,经程控调整后未再发生；例5有2次晕厥发作但无ICD相关事件记录,直立-倾斜试验阳性,考虑合并血管迷走性晕厥。结论ICD是高危的Brugada综合征患者必要的和有效的治疗措施,但可能出现除颤阈值增高或误放电,应严密随访并进行合理的程控。     

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.     

埋藏式心脏转复除颤器安置的临床经验

总结非开胸经锁骨下静脉穿刺安置埋藏式心脏转复除颤器 (ICD)的手术方法、除颤阈值 (DFT)测定及ICD工作参数设置等临床经验。 10例患者 ,6例有反复晕厥病史。 2例晕厥时心电图证实为心室颤动 (简称室颤 ) ,体外电除颤成功 ,另 8例心内电生理均诱发出持续性室性心动过速或室颤。其中冠心病 8例 (1例合并Brugada综合征 ) ,扩张性心肌病 1例 ,原发性室颤 1例。 5例术前口服胺碘酮治疗。结果 :全部经锁骨下静脉置入ICD ,术中所有患者成功诱发室颤 ,并一次电击成功。手术时间 92± 2 7min。DFT≤ 2 0J,电击阻抗 4 1.2± 15 .3Ω ,R波高度 16 .3± 6 .6mV ,无手术并发症。结论 :经锁骨下静脉置入ICD方法简单 ,安全可靠 ;术前口服适量胺碘酮对术中诱发室颤无影响。