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综合征一例

患者男,27岁。反复胸闷,心慌8年,不明原因夜间晕厥1次,无明显器质性心脏病。口服普罗帕酮诱发心室颤动,心电图表现:V1、V2是完全性右束支传导阻滞,ST呈穹隆形和鞍形抬高,T波倒置。经静脉滴注异丙肾上腺素后心电图恢复正常。上级医院证实为Brugagda综合征,置入埋藏式心脏转复除颤器。     

普罗帕酮诱发Brugada样心电图改变一例

Brugada综合征主要在成年时期出现症状,且主要在休息或睡眠中发生猝死,儿童时期并不常见,尤其以心房扑动为首发表现的相关报道鲜见。本文报道一例以心房扑动为首发表现的13岁患儿,无基础心脏疾病,在接受普罗帕酮治疗的过程中诱发出Brugada样心电图改变,基因检测发现可疑变异基因SCN5A,变异位点为c.2834A>G(p.D945G)。希望通过本病例的诊断、治疗及相关文献回顾,提高临床医师对Brugada综合征的诱发因素以及其可合并多种心律失常的认识,加强在应用抗心律失常药物时的心电监测,并对此类高危人群进行指导和密切随访,从而避免不良事件的发生。     

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。     

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。     

Brugada综合征一例

患者男性 ,37岁。因突然意识丧失 ,行心肺复苏后 4ｄ于 2 0 0 1年 7月 10日入院。 2 0 0 1年 7月 6日患者因头晕在医院静脉滴注丹参注射液过程中突然意识丧失、抽搐 ,心电图示心室颤动 (室颤 ,图 1)。立即给予胸外心脏按压 ,气管插管呼吸囊辅助呼吸 ,静脉注射肾上腺素 ,约 5ｍｉｎ恢复窦性心律 ,意识恢复。 38ｍｉｎ时心电图示室内阻滞伴Ｖ1～Ｖ4导联ＳＴ段抬高 (图 2 )。第 2日复查心电图仍可见室内阻滞 ,ＳＴ段恢复正常。体格检查 :血压 110 / 80ｍｍＨｇ(1ｍｍＨｇ =0 133ｋＰａ) ,神志清楚 ,两肺呼吸音清 ,心界不大 ,心率71次 /ｍｉｎ …     

Brugada综合征二例

2例Brugada综合征,心电图上Brugada波的变化呈间歇性和多变性;例1由正常向1型演变,例2由3型向1型的演变。此2例均系回顾性诊断,说明对不明原因的晕厥、心悸的病例应仔细分析心电图的变化,避免误诊。     

Brugada综合征合并先天性长QT综合征一例

目的报告1例反复发作晕厥伴胸前导联J—ST—T显著抬高患者的临床过程。方法1例15岁男孩反复于夜间卧床休息时发生晕厥，对该患者及其父母进行病史询问、体格检查、心电图及超声心动图检查，并行普罗帕酮激发试验。对患者进行冠状动脉，左、右心室造影和心内电生理检查。结果患者及其父母无器质性心脏病依据，无阳性猝死家族史。患者直立倾斜试验阴性，冠状动脉和左、右心室造影正常，心内电生理检查未发现异常，未诱发室性心律失常。患者基础心电图胸前导联J-ST—T显著抬高，晕厥后窦性心动过速时J—ST—T降低伴QTc延长。静脉注射普罗帕酮70mg后胸前导联J—ST与T波第二峰进一步抬高。患者母亲基础心电图ST—T类似于LQT3，但QTc正常。患者父母在静脉注射普罗帕酮70mg后胸前导联ST—T均进一步抬高。结论该患者心电图不同于已报道的Brugada综合征合并LQT3，可能为新的SCN5A基因突变导致的一种新的表型。     

Brugada综合征合并冠心病一例

1例56岁男性,因晕厥1次就诊于外院,行冠状动脉CT检查提示"三支病变"。入院后行心电图检查可见典型I型Brugada波,行冠状动脉造影检查可见冠状动脉前降支近段狭窄40%,考虑V1~V3导联ST段改变和冠状动脉前降支病变无关,余病变冠状动脉行介入治疗后,再次复查心电图呈Ⅱ型Brugada波,患者因拒绝植入埋藏式心脏转复除颤器,随后出院。     

Brugada综合征2例

例1患者女性。47岁。因反复晕厥、胸闷、心悸一年余就诊入院,劳累后心悸加剧,偶有失眠,作心电图检查。     

胺碘酮和普罗帕酮治疗冠心病并室性心律失常的疗效比较

比较胺碘酮和普罗帕酮治疗冠心病心肌缺血患者室性心律失常的疗效。6 9例冠心病心肌缺血合并室性心律失常患者 ,均接受冠心病正规治疗 ,其中 35例同时口服胺碘酮片 (胺碘酮组 ) ,34例口服普罗帕酮片 (普罗帕酮组 ) ,疗程 4周。疗程开始及结束时均行 2 4h动态心电图及 12导联心电图检查。结果 :两组患者用药后 2 4h室性早搏 ,短阵室性心动过速的发作次数均明显减少 (胺碘酮组用药后与用药前比较分别为 2 70 5± 14 77个vs 6 834± 45 2 8个 ,7.4 2± 3.30次vs 1.2 9± 0 .93次 ;普罗帕酮组则分别为 6 712± 3385个vs 396 2± 1983个 ,8.0 5± 3.37次vs4 .2 2± 2 .5 9次 ,P均 <0 .0 1)。胺碘酮组的疗效高于普罗帕酮组 (P <0 .0 1)。两组未见严重副作用。结论 :胺碘酮对冠心病伴室性心律失常的疗效优于普罗帕酮。     

J‐Wave Disappearance Immediately After an Episode of Ventricular Fibrillation in a Patient With Resuscitated Sudden Cardiac Death and Brugada Syndrome

J‐Wave Disaapearance After an Episode of Ventricular Fibrillation . Early repolarization (ER) abnormalities in the inferior‐lateral leads are a matter of intense scientific debate because of their demonstrated association with Brugada syndrome (BS) and idiopathic ventricular fibrillation (VF). To add fuel to the fire, we present a case in which ER abnormalities are associated with BS but in which, more importantly, they were shown to be transient and strictly correlated with an episode of VF. (J Cardiovasc Electrophysiol, Vol. 21, pp. 1413‐1415, December 2010)     

普罗帕酮治疗心房颤动的Meta分析

应用Meta分析评价普罗帕酮转复心房颤动 (简称房颤 )和 (或 )心房扑动及药物、电转复后抑制房颤复发的疗效。应用检索公开发表的相关的英、中文文献建立数据库 ,应用Meta统计分析方法按观察时间、给药途径对数据进行综合分析。结果 :普罗帕酮转复阵发性房颤的疗效随时间延长而增加 ,静脉转复率 1h为 3 7.0 %( 95 %可信区间3 2 .9%～ 4 1.2 %)、8h为 61.4 %( 4 8.2 %～ 74 .5 %) ;口服普罗帕酮转复房颤疗效在 2 ,4 ,8h分别达到 4 .0 %( 3 .8%～4 .2 %)、4 7.3 %( 4 3 .8%～ 5 0 .8%)、72 .6%( 62 .8%～ 82 .4 %) ,2 4h为 81.4 %( 5 2 .0 %～ 10 0 %)、4 8h为 76.5 %( 5 4 .1%～ 98.8%)。前 4h静脉用药比口服起效迅速。口服普罗帕酮与安慰剂对比 ,转复房颤的差异在用药后 8h最显著[3 7.7%( 2 9.4 %～ 4 5 .9%) ,P <0 .0 0 1],在 2 4h两者之差减少为 17.3 %( - 5 .0 %～ 3 9.7%) (P =0 .13 )。药物或直流电转复后普罗帕酮长期应用预防复发、维持窦性的疗效 ,在给药后 1个月的病人中为 63 .80 %( 5 8.2 %～ 69.4 %) ,6个月后为 5 4 .70 %( 4 8.0 %～ 61.4 %)。结论 :普罗帕酮能安全有效转复房颤 ,长期应用可预防转复后复发 ,耐受性好。     

Clinical predictors of atrial fibrillation in Brugada syndrome.

AIMS: Atrial arrhythmias have been reported in patients with Brugada syndrome. The aim of this study was to evaluate clinical predictors of atrial fibrillation (AF) in Brugada syndrome. METHODS AND RESULTS: Patients diagnosed with Brugada ECG pattern were enrolled in the study. Type 1, 2, and 3 Brugada ECG pattern was found in 28, 56, and 31 patients, respectively. A total of 85 healthy age and gender-matched subjects were selected as a control group. Mean age, maximum P-wave duration (P(max)), P-wave dispersion (P(disp)), and left atrial dimension were not significantly different between patients and controls. There were no differences between P(max), P(disp), and left atrial dimension of the type 1, 2, and 3 Brugada patients. Spontaneous paroxysmal AF was detected in 15 of 28 type 1 Brugada patients (53%) and none of the type 2 and 3 Brugada patients. All 15 patients with AF had at least one episode of paroxysmal AF and none of the patients showed persistent or chronic AF. All 15 patients who had paroxysmal AF had previous life threatening cardiac events. In contrast, paroxysmal AF did not occur in type 1 Brugada patients without previous life threatening cardiac events. In multiple regression analysis, only the occurrence of previous life threatening cardiac events was a risk factor for paroxysmal AF (P = 0.0001). CONCLUSION: It is concluded that the most important predictor of AF in Brugada syndrome is the occurrence of previous life threatening cardiac events.     

Short-term normalization of ventricular repolarization by transcatheter ablation in a patient with suspected Brugada Syndrome

We report a 30 year old male without structural heart disease who presented with recurrent nocturnal syncope and aborted sudden cardiac death. 12-lead ECG showed elevated ST in inferior leads and short coupled premature ventricular complexes (PVCs). Propafenone challenge suggested a diagnosis of an atypical Brugada syndrome. Two morphological types of PVCs and ventricular fibrillation (VF) were induced during propafenone challenge test. He underwent two ablation procedures in right ventricular inflow tract and left ventricular post-inferior septum region by pace-mapping, respectively. After ablation, VF could not be induced and the elevated ST segments normalized. Two subsequent propafenone challenge tests were also negative. Nonetheless, elevated ST segments and PVCs reappeared by 1 month follow-up. An implantable defibrillator was recommended, but the patient declined for financial reasons. Unfortunately, he suffered a sudden cardiac death at home 10 weeks post-ablation. These findings suggest that short-term normalization of ventricular repolarization possibly due to radiofrequency ablation may occur in Brugada syndrome. However, the transient nature of this finding suggests that it is not a reliable indicator of protection against sudden cardiac death.     

Radiofrequency Ablation of Ventricular Fibrillation and Multiple Right and Left Atrial Tachycardia in a Patient with Brugada Syndrome

Brugada syndrome is a well-known form of idiopathic ventricular fibrillation (VF). Few data suggest that this arrhythmia may be triggered by ventricular premature beats (VPBs), and an association with other arrhythmia such as monomorphic ventricular tachycardia (VT) or supraventricular tachycardia (SVT) has been reported. In a highly symptomatic 18-year-old-male patient with this syndrome, frequent episodes of VF, fast polymorphic VT, and fast monomorphic sustained regular tachycardia were observed. The tachycardia episodes were classified as VT or VF and as a consequence received appropriate therapies with the implanted cardioverter defibrillator (ICD). Precipitating VPBs that were stored in the ICD memory and on the electrocardiogram (ECG) exhibited the same morphology as frequent isolated VPBs. During the electrophysiological study, right and left atrial tachycardia (AT) with one-to-one atrioventricular conduction were also induced and successfully ablated. VF was ablated using the same noncontact mapping (NCM) system triggering VPBs from right ventricular outflow tract (RVOT).     

口服单剂普罗帕酮转复阵发性心房颤动的疗效观察

观察口服单剂负荷量Ⅰc类抗心律失常药物普罗帕酮 (悦复隆 )转复阵发性心房颤动 (PAF)的疗效 ,以期减少患者反复住院治疗的次数。32例门诊及住院患者经心电图证实均为PAF ,持续时间 >2h ,剔除心功能Ⅱ级 (NYHA分级 )以上、病窦综合征、房室阻滞和束支阻滞。随机将患者分为两组 :治疗组 18例 ,口服悦复隆 4 5 0mg ;对照组 14例 ,口服安慰剂。以Holter或心电监护记录观察给药后 2 ,4 ,12h内心律。转复窦性心律后记录 12导联心电图。结果 :两组性别、年龄、病因、PAF病程、左房内径均无差别 (P >0 .0 5 )。治疗组转复率 2h内为 11.1% (2 / 18)、4h内为4 4 .4 % (8/ 18例 )、12h内为 6 1.1% (11/ 18例 ) ;对照组转复率则分别为 0、7.1% (1/ 14例 )、2 1.4 % (3/ 14例 )。两组比较 ,在 4和 12h内的转复率有显著性差异 (P <0 .0 5 )。治疗中两例患者转复窦律后PR间期 0 .2 2s ,2～ 2 4h后恢复至 0 .2 0s。结论 :对不伴严重心功能不全及缓慢性心律失常的PAF患者 ,口服单剂负荷量普罗帕酮能有效和迅速地使PAF转复 ,缩短PAF持续时间     

Dynamic Changes of 12-Lead Electrocardiograms in a Patient with Brugada Syndrome

Dynamic ECG Changes in Brugada Syndrome. We present a patient with Brugada syndrome in whom 12-lead ECXis were recorded just before and after an episode of ventricular fibrillation (VF). A progressive elevation of both the RS-T segment and J waves just preceding and following the VF, and a close relationship between the amplitude of the RS-T segment and the preceding R-R intervals during atrial fibrillation, were documented. These findings support the hypothesis that RS-T elevation and a subsequent VF are related to a transient outward current-mediated spike-and-dome morphology of the epicardial action potential.     

Brugada综合征室性心律失常发作的时间特征及其临床意义

目的运用Holter和置入式心脏复律除颤器(ICD)研究Brugada综合征(BrS)患者室性心律失常发作的时间特征。方法8例BrS患者和6例特发性BrS心电图征者均为男性,平均年龄(41.07±11.49)岁,根据临床表现分为心室颤动(室颤)组和无室颤组各7例,行Holter检查比较两组间室性早搏(室早)发作的时间特征。根据ICD的随访资料,分析室颤发作的时间特征。结果Holter显示,多数患者室早总数在0~74(9.61±17.23)个/24h,两组间室早的数量差异无统计学意义[(108±269)个/24h与(8±19)个/24h,P>0.05]。室颤组的98.67%的室早发作集中在夜间2200至凌晨700,而无室颤组为44.14%,室颤组明显高于无室颤组(χ2=1480,P<0.01)。5例患者ICD置入后随访9~54(23.80±17.96)个月,75次室颤发作中93.3%集中在夜间2200至凌晨700。结论高危的BrS患者的室早具有夜间和凌晨集中发作的特征,可能是新的无创性危险分层指标。BrS患者的室颤发作多集中在夜间和凌晨,可据此设计给药方案以减少副作用。     

新胸导联在诊断Brugada综合征中的应用

目的 评价自行设计的新胸导联在Brugada综合征诊断中的应用价值。方法 4例Brugada综合征先证者和9例家族成员接受新胸导联和普罗帕酮药物试验。11例无晕厥史和无猝死家族史的房室结折返性室上性心动过速患者作为新胸导联检查对照组。自行设计的心电图新胸导联包括A-G(7)列、0-5(6)行,共42个导联,记录方法同标准胸导联。普罗帕酮试验:普罗帕酮70 mg(体重>70kg者用105 mg)加入生理盐水10 ml于5 min内静脉注射,用药前、后记录标准12导联心电图;用药后标准V1-V3导联J点或ST段抬高超过2 mm(或ST段抬高由BrugadaⅡ或Ⅲ型转变成Ⅰ型),称为药物试验阳性。结果 先证者1～3均有晕厥史,先证者1和3有家族猝死史,先证者2和3晕厥发作时记录到心室颤动,先证者3猝死;除先证者4标准胸导联心电图呈Brugada典型下斜型改变外,先证者1-3标准胸导联心电图不能明确诊断为Brugada综合征。新胸导联发现先证者1-3呈典型Brugada综合征心电图改变,位于标准胸导联以外区域。新胸导联同时发现,5例家族成员在标准胸导联以外区域有典型的Brugada心电图改变。新胸导联阳性者亦被普罗帕酮试验证实。对照组11例新胸导联检查均为阴性。结论 新胸导联有助于发现Brugada综合征典型心电图改变位于标准V1-V3导联以外的病例,且应用安全,方法较简单