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。     

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。     

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。     

永久起搏器和埋藏式心脏复律除颤器（ICD）在遗传性长QT综合征患者中的应用

目的总结合并窦性心动过缓的遗传性长QT综合征（以下简称遗传性LQTS）患者植入永久起搏器和埋藏式心脏复律除颤器（以下简称ICD）的治疗效果,对比分析这两种治疗在预防患者猝死中的差异。方法对我院从2003年6月到2013年6月出院诊断为遗传性LQTS合并窦性心动过缓、植入了永久起搏器或ICD的全部21例患者,结合门诊、电话和程控随访了解患者的生存状况、手术并发症以及晕厥、室性恶性心律失常的发作情况。结果起搏器组男性2例,女性9例,年龄39.3±14.3岁,随访时间50.6±26.3个月,1例患者猝死,2例患者再发晕厥前兆,其中1例最终更换为ICD。ICD组男性2例,女性8例,年龄34.5±11.9岁,随访时间61.4±43.5个月,3例患者接受了ICD的适当治疗,另2例患者接受了ICD的不适当治疗,1例患者术后出现囊袋感染,1例患者更换为永久起搏器。治疗有效率在起搏器组及ICD组分别为72.7%（8/11）和100.0%（10/10）,未达到统计学差异（p=0.21）。不良事件发生率在起搏器组及ICD组分别为27.3%（3/11）和30.0%（3/10）,也未达到统计学差异（p=0.63）。结论对于不能植入ICD的合并窦性心动过缓的遗传性LQTS患者,植入永久起搏器可能是一个较好的替代方法,但对于QTc≥539ms的患者,只有植入ICD才能预防猝死。植入ICD后长期无心脏事件发生的患者,根据患者意愿,可考虑更换为永久起搏器。     

Brugada综合征合并间歇性右束支传导阻滞经奎尼丁治疗成功一例

该文报道1例反复发作晕厥患者,心电图表现为Brugada综合征合并间歇性完全性右束支传导阻滞,植入式心律转复除颤器(ICD)植入后仍反复发作恶性室性心律失常电风暴,后予奎尼丁(100 mg,每日3次)口服治疗10个月无发作;停用奎尼丁后再次反复发作多形性室性心动过速电风暴触发ICD放电,再次服用相同剂量奎尼丁后随访1年余无心律失常发作。Brugada合并右束支阻滞可能与室性心动过速发生风险高有关,低剂量奎尼丁预防室性心律失常有效。     

38例症状性长QT综合征患者心脏事件复发诱因分析

目的评估症状性先天性长QT综合征(congenital long QT syndrome,cLQTS)患者心脏事件复发的主要诱因。方法本研究为回顾性病例分析。在2014年11月至2020年2月就诊于北京清华长庚医院的66例症状性cLQTS患者中,选取首次就诊后心脏事件复发的38例为研究对象。采集患者性别、发病和就诊年龄、就诊前后临床表现、家族史、治疗情况、心电图和cLQTS亚型资料。回顾性分析患者心脏事件发作时间、次数、诱因以及药物治疗情况和心电图资料。心脏事件包括:晕厥、埋藏式心脏转复除颤器(implantable cardioverter defibrillator,ICD)放电、ICD误放电、心脏骤停生还、心脏性猝死、心电图证实发生室性心动过速。结果38例心脏事件复发的cLQTS患者中,女性30例(79%),儿童14例(37%),发病年龄(15.6±14.6)岁,就诊后复发时间为(3.6±3.5)年。亚型分析表明纳入11例(29%)LQT1(含2例Jervell-Lange-Nielson综合征患儿)、19例(50%)LQT2、5例(13%)LQT3及3例(8%)其他少见亚型(1例LQT5、1例LQT7和1例LQT11)。LQT1患者中共6例(55%)患者因停药和/或漏服药物出现心脏事件发作;4例(36%)患者在特定诱因(运动、情绪激动)下发生晕厥;1例(9%)患者自行换药后出现晕厥发作。LQT2中共16例(84%)患者因漏服和/或停药后出现心脏事件,3例(16%)患者于特定诱因(惊吓、声音刺激、睡醒)下心脏事件发作;1例(5%)患者怀孕后出现反复晕厥发作;1例(20%)患者发生ICD误放电。LQT3患者中4例(80%)患者反复静息状态下出现晕厥;1例(20%)患者于心电图运动平板试验过程中出现室性心动过速。1例LQT5患者特定诱因(情绪激动、休息时)下反复出现晕厥及ICD放电。1例LQT11患者特定诱因(劳累)下有反复ICD放电。1例LQT7患者随访过程中出现ICD误放电。随访过程中2例Jervell-Lange-Nielson综合征患儿和2例LQT2患者因β受体阻滞剂效果不佳行交感神经节切除术后晕厥发作较前减少;1例服用美托洛尔后仍有晕厥发作的LQT2患者改服纳多洛尔后无晕厥发作;2例服用β受体阻滞剂效果不佳的LQT2患者加用美西律治疗后症状较前改善。结论自行停药或漏服药物是心脏事件复发的重要诱因。     

植入型心律转复除颤器在长QT综合征患者中的应用

目的 总结阜外心血管病医院应用植入型心律转复除颤器(ICD)治疗长QT综合征(LQTS)患者的经验.方法 8例LQTS患者接受了ICD治疗,平均年龄(38.9±16.7)岁.在随访期间,根据具体情况,调整ICD的各项程控参数.结果 平均随访(27.3±25.9)个月,有3例患者出现了101次室性心动过速/心室颤动(VT/VF)事件.在出厂程控参数下,共记录到44次VF事件.重新设置VT/VF的识别及治疗参数后,共记录到57次VT事件,2次VF事件,大部分自行终止或经低能量转复成功.1例患者术后出现了电风暴,通过快速心室起搏,终止了尖端扭转性室性心动过速(Tdp)的反复发作.2例患者发生了因T波误感知导致的ICD误放电,通过延长感知灵敏度自动调整延迟,从而避免了T波误感知.结论 针对LQTS患者的发病特点,调整ICD的程控参数,可以提高疗效并减少ICD误放电.     

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。     

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。     

国人Brugada综合征的临床分析

目的探讨中国Brugada综合征的发病现状、临床特征和诊治情况。方法选择1998年1月至2013年6月在CNKI、万方数据库、维普数据库、Pubmed中以"Brugada"为自由词或关键词,检索国人发表的Brugada病例182例。采用回顾性方法,对资料进行汇总分析。结果 Brugada综合征患者男女之比14.7∶1,中位年龄41岁;25例有家族猝死史,5例有家族Ⅰ型Brugada波。72.3%有晕厥史,晕厥年龄(43.0±12.0)岁。39.0%记录到多形性室性心动过速/心室扑动/心室颤动。71例行心电生理检查,阳性率49.3%。药物激发试验阳性率为92.9%。10例误诊。埋藏式心脏转复除颤器(ICD)植入占指南推荐的19.8%。猝死率8.2%,猝死年龄(48.7±16.7)岁。结论近15年来中国报道182例Brugada综合征,其特点:累及年龄广,中青年为主,男性居多,晕厥为其最主要的临床表现,药物激发试验及心电生理检查阳性率高,治疗现状不规范,ICD植入率低,猝死率高。     

Long-term follow-up in patients with Brugada Syndrome in South China

ObjectiveTo evaluate the presence of Brugada electrocardiogram (ECG) pattern, clinical characteristics, treatment, and long‐term prognosis of Brugada syndrome in southern Chinese population.MethodsThis prospective study consisted of a consecutive series of patients with diagnostic coved type I Brugada ECG pattern at baseline between January 2007 and February 2020. Histories of symptoms including ventricular tachycardia (VT)/ventricular fibrillation (VF) episode, syncope, and family history of Brugada Syndrome (BrS) or unexplained sudden cardiac death were collected. Electrophysiological study and implantable cardioverter‐defibrillator (ICD) were performed. All patients included in this study were followed up in the outpatient department every 6 months after baseline evaluation. Occurrences of syncope, VF, and sudden death were independently analyzed by two cardiologists.Results45 (56.3%) patients were diagnosed with BrS. During a mean follow‐up of 7.9 ± 3.6 years, six patients had experienced documented VF/sudden cardiac death (SCD) or recurrent syncope. Two patients experienced episodes of syncope more than once. Two patients experienced onset of electrical storm with a total of 11 episodes of VF. There were 50% of these events occurring in fever status. One of patient with BrS died of SCD.ConclusionThere was a very low prevalence of Brugada syndrome in southern Chinese population. The risk of arrhythmic events was low in asymptomatic patients. ICD was high effective in preventing SCD without adverse device outcome in long‐term follow‐up. Fever can lead to predispose to malignant arrhythmia, and aggressive treatment of febrile state in Brugada syndrome was recommended.     

Implantable cardioverter defibrillator in high-risk long QT syndrome patients

INTRODUCTION: Implantable cardioverter defibrillators (ICDs) are increasingly being used in high-risk long QT syndrome (LQTS) patients, but there are limited data regarding clinical experience with this therapeutic modality. The aim of this study is to describe the clinical characteristics of 125 LQTS patients treated with ICDs compared with LQTS patients having similar risk indications who were not treated with ICDs. Among 125 LQTS patients with ICDs, there were 54 cardiac arrest survivors, 19 patients who had ICDs implanted due to recurrent syncope despite beta-blocker therapy, and 52 patients with ICDs implanted due to other reasons, including syncope and LQTS-related sudden death in a close family member. Patients with cardiac arrest and those with recurrent syncope despite beta-blocker therapy (n = 73) were compared to 161 LQTS patients who had similar indications (89 cardiac arrest and 72 recurrent syncope despite beta-blocker therapy) but did not receive ICDs. Total mortality was the endpoint of the analysis. There was 1 (1.3%) death in 73 ICD patients followed an average of 3 years, whereas there were 26 deaths (16%) in non-ICD patients during mean 8-year follow-up (P = 0.07 from log rank test from Kaplan-Meier curves). CONCLUSION: ICDs provide an important therapeutic option to prevent sudden arrhythmic death in high-risk LQTS patients. A long-term prospective study is needed to determine the benefit of this therapeutic modality in LQTS patients.     

普萘洛尔对21例长QT综合征的临床疗效观察

观察β 阻断剂普萘洛尔对长QT综合征 (LQTS)患者的疗效。选择适于服用β 阻断剂又能规律服药的 2 1例患者进行用药前后的对比研究。结果 :患者年龄 2 4 .4± 12 .4岁 ,首次发病年龄 11.2± 11.1岁。跟踪观察的时间为 15 .9± 32 .8个月。服用普萘洛尔的剂量为 6 3± 4 4mg/d ,按体重计算为 1.3± 0 .7mg/kg ,到达 2± 4mg/kg的患者有 3例 (14 .3% )。部分首次发病时间较早的高危患者 ,由于本身的基础心率较慢 ,普萘洛尔很难加到所需剂量 ,故服用普萘洛尔的同时 ,有 8例行左心交感神经切除术、1例安装起搏器、1例埋置埋藏式心脏复律除颤器 ,2例服用美西律。普萘洛尔使QT间期 (QTc)从用药前的 5 70± 70ms降低到 5 0 0± 6 0ms(P <0 .0 0 0 1) ;同时心率并未降低 (分别为 6 5± 11次 /分和 6 5± 10次 /分 ,P =NS)。用药前有晕厥发作的 19例用药后发作次数显著减少 ,平均晕厥发作次数由 1.90± 1.72次 /年降至 0 .17± 0 .4 0次 /年 ,P =0 .0 2。单纯普萘洛尔与联合治疗亚组之间无显著性差别。结论 :普萘洛尔能有效降低大多数LQTS患者的晕厥次数 ,缩短复极时程 ,但对那些首次发病时间较早的高危患者 ,可能需联合应用其它治疗方式。     

遗传性长 QT 综合征的药物及器械治疗

遗传性长 QT 综合征（long-QT syndrome,LQTS）以心电图上 QT 间期延长及其相关的尖端扭转型室性心动过速（torsade de pointes,TdP）为特征,易导致心脏性猝死。它的危险因素有 QTc 长度、基因型、晕厥史等。LQTS 的治疗手段包括β受体阻滞剂治疗、外科心脏左侧交感神经节切除术（LCSD）、心脏起搏治疗以及植入式心律转复除颤器（ICD）治疗。β受体阻滞剂治疗是 LQTS 治疗的基石;心脏起搏治疗适用于伴有心动过缓的 LQTS,可明显降低心脏事件的复发率,但不降低死亡率。对高危的 LQTS 应植入 ICD,皮下 ICD 并发症较少,值得推广。LCSD 技术实用性较差,更适用于 ICD 植入后频繁电击治疗的患者。     

Long QT syndromes

Opinion statement The clinical phenotype of the long QT syndrome (LQTS) is quite variable, with the frequency and type of life-threatening arrhythmias influenced by the specific genotype and a spectrum of genetic and environmental factors that are not well characterized. Patients with a history of recurrent syncope or aborted cardiac arrest are at increased risk of experiencing malignant ventricular arrhythmias, but such arrhythmias may also occur in affected individuals who previously have been asymptomatic. Beta-adrenergic drugs serve as the foundation for treatment of symptomatic patients with a history of syncope or aborted cardiac arrest and as primary prophylactic therapy in asymptomatic subjects with LQTS. Beta-blockers reduce the frequency of syncopal events, but they do not absolutely prevent the occurrence of sudden cardiac death, even in those who are compliant in taking full doses of beta-blockers. Pacemaker therapy is moderately effective in reducing the number of cardiac events in patients with inappropriate bradycardia. The implantable cardioverter-defibrillator (ICD) has functioned well as a fail-safe back-up therapy in high-risk patients, especially those with documented malignant arrhythmias or an aborted cardiac arrest. Left cervicothoracic sympathetic ganglionectomy should be reserved for patients with LQTS who are intolerant of beta-blockers or have recurrent syncope that is refractory to beta-blockers and who for one reason or another are not candidates for ICD therapy. Pharmacologically tailored gene-specific therapy for specific ion-channel disorders is in its infancy, and no specific recommendations can be made for the use of this therapy at this time.     

Implantable Cardioverter-Defibrillators in Children and Adolescents With Brugada Syndrome

Background

Young patients presenting with symptomatic Brugada syndrome have very high risks for ventricular arrhythmias and should be carefully considered for implantable cardioverter-defibrillator (ICD) placement. However, this therapy is associated with high rates of inappropriate shocks and device-related complications.