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.     

致心律失常性右室心肌病患者Epsilon波的检出率

Objective To investigate the prevalence of Epsilon wave in patients with arrhythmogenic right ventrieular cardiomyopathy (ARVC). Methods The Epsilon wave was detected in 32 patients [24 men, mean age (42.3±13.3) years] with ARVC using three different electrocardiography (ECG) recording methods: standard twelve leads ECG (S-ECG), right precordial leads ECG (R-ECG) and Fontaine bipolar precordiai leads ECG (F-ECG). The Epsilon wave was defined as wiggle, small spike wave and smooth potential between the end of the QRS complex and the beginning of the ST segment. Results Epsilon wave was detected in 37.5%, 37.5% and 50.0% patients with ARVC by S-ECG, R-ECG and F-ECG respectively. The detection rates derived from the three recording methods were similar(P ＞ 0.05). The Epsilon wave was only detectable by S-ECG in one case, by R-ECG in three cases, and by F-ECG in five cases. The detection rate of Epsilon wave was 50.0% by combined use of S-ECG and R-ECG (SR-ECG), 56.3% by combined use of S-ECG and F-ECG (SF-ECG), and 65.6% by combined use of the three recording methods (SRF-ECG). The detection rate was significantly higher by SF-ECG (56.3%) and SRF-ECG (65.6%) than by S-ECG alone (37.5%, all P ＜0.05). Most Epsilon waves detected by the S-ECG, R-ECG and F-ECG were small spiked waves. Conclusion Combined use of S-ECG, F-ECG and R-ECG could increase the detection rate of Epsilon wave in patients with ARVC.     

植入型心律转复除颤器治疗恶性室性心律失常的疗效评价

目的评价单中心40例植入型心律转复除颤器(ICD)治疗恶性室性心律失常的疗效及安全性。方法40例恶性室性心律失常包括室性心动过速(室速)或心室颤动(室颤)患者接受ICD治疗,男性35例,女性5例,平均年龄(49±15)岁,成功随访35例,应用体外程控仪获得ICD储存资料并结合临床随访资料进行分析。结果40例患者均成功植入ICD;35例患者平均随访25个月,其中26例患者共记录室速和室颤事件763阵,ICD成功除颤224阵(成功率99．1％),抗心动过速起搏1次成功终止室速375阵(成功率71．8％),低能量同步转复22阵(成功率100％);2例患者因窦性心动过速和心房颤动伴快速心室反应发生误放电4次。术后大多数患者联合应用抗心律失常药物。至随访期末,死亡4例,3例死于顽固性心力衰竭,1例死于肺栓塞。结论ICD联合应用抗心律失常药物能有效治疗恶性室性心律失常,预防心脏性猝死。     

导管消融终止无休止特发性左室室性心动过速伴先天性QT延长综合征心室颤动风暴一例

患女性，37岁。无家族性心脏猝死史，持续心悸6个月伴发作性晕厥4d。心电图记录为持续性呈右束支阻滞样图形伴电轴上偏室性心动过速（室速），偶尔间隙窦性心律、晕厥发作。在当地医院心电监护为心室颤动（室颤）共5次，每次用电复律转复，后转院途中及急诊室多次发生室颤并行除颤复律。多次查血钾正常，连续12天心电监护80％时间为室速，每天都有室颤，1～5次／d不等，每次发作均需电复律终止。如图1所示，电复律后立即又转变成室速，     

心房颤动时射频消融右侧房室旁路一例

心房颤动时射频消融右侧房室旁路一例曹克将单其俊杨志健李闻奇黄元铸顾翔何胜虎心房颤动（房颤）是临床上十分常见的心律失常，它不仅发生于器质性心脏病病人，也常见于预激综合征患者。在进行射频消融房室旁路时，房颤的出现导致导管标测复杂化，严重影响消融靶点的确定...     

具有良好变时作用的持续性房性异位心律:临床特征、电生理机制及射频导管消融

目的介绍具有良好变时作用的房性异位心律(C-EAR)这一特殊类型的心律失常,分析其临床特征、可能的电生理机制及导管消融方法。方法共4例患者(男性3例),临床拟诊房性心动过速(房速)收住院,基础节律均为异位房性心律。其中1例既往有房间隔缺损修补术病史,余均无器质性心脏病史。常规穿刺置入导管行心内电生理检查,术中记录基础节律、最快节律及心动过速的心内电图。是否射频消融根据电生理检查的结果。所有患者术前、术后均行动态心电图检查。结果4例患者C-EAR分别位于右后间隔、右中间隔、三尖瓣环9点钟和左下肺静脉开口处。C-EAR基础状态平均房率为(84±19)次/min;临床或术中发现的最快房率平均为(198±20)次/min。病例1和病例2术中诱发出和基础节律不同源的房速,其中病例1合并围绕右心房侧壁手术疤痕折返的房速,消融未成功;病例2合并希氏束旁房速,消融成功。病例1和病例2的基础C-EAR未作消融。病例3和病例4的房速与C-EAR同源,病例3消融失败,病例4消融成功转为稳定窦性心律。4例患者的动态心电图显示,C-EAR随昼夜节律波动,根据生理需要升降,体现出良好的变时性作用。平均最慢房率(68±20)次/min,平均最快房率为(134±17)次/min,日平均房率的平均值为(96±13)次/min。结论C-EAR是一种特殊节律,它是由异位起搏细胞的自律性增加所致。如在此基础上合并触发活动或局部微折返则可形成同源的心动过速。由于C-EAR具有良好的变时性作用,在不合并同源性心动过速时,无需消融治疗。     

致心律失常性右室心肌病患者Epsilon波的检出率

Objective To investigate the prevalence of Epsilon wave in patients with arrhythmogenic right ventrieular cardiomyopathy (ARVC). Methods The Epsilon wave was detected in 32 patients [24 men, mean age (42.3±13.3) years] with ARVC using three different electrocardiography (ECG) recording methods: standard twelve leads ECG (S-ECG), right precordial leads ECG (R-ECG) and Fontaine bipolar precordiai leads ECG (F-ECG). The Epsilon wave was defined as wiggle, small spike wave and smooth potential between the end of the QRS complex and the beginning of the ST segment. Results Epsilon wave was detected in 37.5%, 37.5% and 50.0% patients with ARVC by S-ECG, R-ECG and F-ECG respectively. The detection rates derived from the three recording methods were similar(P ＞ 0.05). The Epsilon wave was only detectable by S-ECG in one case, by R-ECG in three cases, and by F-ECG in five cases. The detection rate of Epsilon wave was 50.0% by combined use of S-ECG and R-ECG (SR-ECG), 56.3% by combined use of S-ECG and F-ECG (SF-ECG), and 65.6% by combined use of the three recording methods (SRF-ECG). The detection rate was significantly higher by SF-ECG (56.3%) and SRF-ECG (65.6%) than by S-ECG alone (37.5%, all P ＜0.05). Most Epsilon waves detected by the S-ECG, R-ECG and F-ECG were small spiked waves. Conclusion Combined use of S-ECG, F-ECG and R-ECG could increase the detection rate of Epsilon wave in patients with ARVC.     

致心律失常性右室心肌病患者Epsilon波的检出率

Objective To investigate the prevalence of Epsilon wave in patients with arrhythmogenic right ventrieular cardiomyopathy (ARVC). Methods The Epsilon wave was detected in 32 patients [24 men, mean age (42.3±13.3) years] with ARVC using three different electrocardiography (ECG) recording methods: standard twelve leads ECG (S-ECG), right precordial leads ECG (R-ECG) and Fontaine bipolar precordiai leads ECG (F-ECG). The Epsilon wave was defined as wiggle, small spike wave and smooth potential between the end of the QRS complex and the beginning of the ST segment. Results Epsilon wave was detected in 37.5%, 37.5% and 50.0% patients with ARVC by S-ECG, R-ECG and F-ECG respectively. The detection rates derived from the three recording methods were similar(P ＞ 0.05). The Epsilon wave was only detectable by S-ECG in one case, by R-ECG in three cases, and by F-ECG in five cases. The detection rate of Epsilon wave was 50.0% by combined use of S-ECG and R-ECG (SR-ECG), 56.3% by combined use of S-ECG and F-ECG (SF-ECG), and 65.6% by combined use of the three recording methods (SRF-ECG). The detection rate was significantly higher by SF-ECG (56.3%) and SRF-ECG (65.6%) than by S-ECG alone (37.5%, all P ＜0.05). Most Epsilon waves detected by the S-ECG, R-ECG and F-ECG were small spiked waves. Conclusion Combined use of S-ECG, F-ECG and R-ECG could increase the detection rate of Epsilon wave in patients with ARVC.     

获得性长QT综合征

长QT综合征包括先天性长QT综合征(cLQTS)和获得性长QT综合征(aLQTS)。aLQTS与cLQTS的临床表现相似,均表现为QT间期延长,心室复极离散度增加,尖端扭转型室性心动过速(TdP),可蜕变为心室颤动,导致反复的晕厥、癫痫样发作和猝死。目前药物性aLQTS发病机制主要是药物阻滞HERG基因编码的IKr通道,引起外向钾电流的减少,导致心肌复极延长。其他aLQTS发病机制较为复杂,尚不完全清楚,任何可引起心肌复极延长和离散度增加的因素均可导致QT间期的延长和TdP的发生。aLQTS常见的病因有药物、心律失常(快速或缓慢)、心肌肥厚、心脏扩大、心肌缺血、电解质紊乱、遗传易感性等。目前治疗aLQTS的关键在于提高认识,去除病因,降低猝死发生率。     

致心律失常性右室心肌病患者Epsilon波的检出率

Objective To investigate the prevalence of Epsilon wave in patients with arrhythmogenic right ventrieular cardiomyopathy (ARVC). Methods The Epsilon wave was detected in 32 patients [24 men, mean age (42.3±13.3) years] with ARVC using three different electrocardiography (ECG) recording methods: standard twelve leads ECG (S-ECG), right precordial leads ECG (R-ECG) and Fontaine bipolar precordiai leads ECG (F-ECG). The Epsilon wave was defined as wiggle, small spike wave and smooth potential between the end of the QRS complex and the beginning of the ST segment. Results Epsilon wave was detected in 37.5%, 37.5% and 50.0% patients with ARVC by S-ECG, R-ECG and F-ECG respectively. The detection rates derived from the three recording methods were similar(P ＞ 0.05). The Epsilon wave was only detectable by S-ECG in one case, by R-ECG in three cases, and by F-ECG in five cases. The detection rate of Epsilon wave was 50.0% by combined use of S-ECG and R-ECG (SR-ECG), 56.3% by combined use of S-ECG and F-ECG (SF-ECG), and 65.6% by combined use of the three recording methods (SRF-ECG). The detection rate was significantly higher by SF-ECG (56.3%) and SRF-ECG (65.6%) than by S-ECG alone (37.5%, all P ＜0.05). Most Epsilon waves detected by the S-ECG, R-ECG and F-ECG were small spiked waves. Conclusion Combined use of S-ECG, F-ECG and R-ECG could increase the detection rate of Epsilon wave in patients with ARVC.