双腔起搏器优化程控对长QT综合征的疗效影响

目的探讨双腔起搏器联合B受体阻滞剂治疗先天性长QT综合征（LQTS）的最优程控方式。方法12例正规药物治疗无效或无法耐受的LQTS患者植入DDD起搏器,分别测量不同频率起搏时QT／QTc间期的变化,然后以80次／min的频率起搏,选择性地程控开放或关闭起搏器的部分相关功能,并根据PR间期和血压情况调整B受体阻滞剂用量,随访患者植入术后心脏事件的发生情况和心功能变化情况。结果起搏频率越快,QT／QTC间期越短。80次／min频率起搏时QT／QTc间期可基本恢复正常,植入术后心脏事件发生次数明显减少（P〈0．01）,且对心功能无影响。结论双腔起搏器联合B受体阻滞剂治疗LQTS时,最优程控方式是：以80次／min的频率起搏并程控关闭滞后、睡眠、自动终止起搏器介导的心动过速和自动阈值夺获功能,开放设置室性早搏后反应、频率适应性和房室结优先功能。     

双腔起搏器时经程控治疗房性心律失常

双腔起搏器目前在临床已逐步推广使用 ,程控其某些参数以避免心律失常的发生是一个主要功能 ,本文报告两例 DDD起搏器植入患者利用程控其某些参数来治疗房性心律失常。例 1　患者男性 ,81岁 ,阵发性黑朦 ,头晕 2年入院 ,Holter检查示 :平均HR:6 1bpm,最快 HR:87bpm,最慢HR:40 bpm;频发室上速 ,短阵房颤 ,大于 2 .5 s的长 RR间期 8次 ,最长窦性停搏 4.2 s,食管电生理检查 :窦房结恢复时间 (SNRT) :16 42 m s,较正窦房结恢复时间 (CSRT) :740 m s,SNRT指数(SNRTI) :182 % ,诊断病态窦房结综合征 (慢 -快综合征 ) ,植入 CPI,VIS…     

双腔起搏器中房室间期的程控及临床应用

双心腔起搏对血流动力学的影响主要表现在两个方面：它可保持房室的顺序收缩或房室同步,但仅有房室同步尚不能提供最佳的血流动力学效应,还必须有一个最佳的房室延迟（ＡＶ ｄｅｌａｙ）或称为房室间期（ＡＶ ｉｎｔｅｒｖａｌ）,即在心室收缩之前有最佳的时间进行心房收缩,因此双腔起搏的ＡＶ间期的长短与起搏的血流动力学效应关系密切。最佳的ＡＶ间期对心室充盈,减少房室瓣返流,保持正常的心房压力及防止不良的循环及神经体液反射都是很重要的。不恰当的ＡＶ间期所带来的血流动力学负面作用甚至大于一般的心室起搏。起搏器中的不同…     

起搏器程控

程序控制(简称程控:programmability)是起搏器不可分割的一项功能,今天全世界范围内都看不到使用不带程控功能的起搏器,而且随着起搏工程技术的飞速发展,起搏器的程控功能越来越多,越来越复杂,恰到好处的运用参数,可使起搏器发挥其最大效益,病人获得最大疗效。近几年我国起搏器的临床应用发展甚快,每年超过1万台,各种新技术的开展几乎与国际同步,但对植入起搏器后的程控和随诊却是一个薄弱环节,目前尚做不到凡是开展起搏器的医院都配备有程控仪,有些单位病人装上起搏器后未程控过1次,一些可以通过程控解决的问题却轻易被放弃。希望通过这些…     

VVI起搏治疗先天性长QT综合征12例随访

<正>先天性长QT综合征(Long QT Syndrome,LQTS)是由于编码心脏离子通道的基因突变导致相应的离子通道功能异常而引起的一组综合征,主要表现为心律紊乱[1]。LQTS并非常见病,但该病发病突然、猝死率高、且以青少年发病多见。本院收治39例长QT综合征患者,其中12例由于频发尖端扭转性室性心动过速,出现心源性晕厥,我们对其进行VVI起搏治疗,本文就其随访情况报道如下:     

频率应答式双腔起搏器的临床应用及疗效分析

1资料1.1选择对象 选取2007年-2008年因缓慢性心律失常植入起搏器患者,根据起搏器模式分为(VVI/VVIR)组和(DDD/DDDR)组;同时将双腔起搏器分为DDD和DDDR组.     

应用双腔起搏器程控特性测定心房起搏阈值

目的通过简单、易认的心室起搏心电图的变化来明确心房起搏阈值。方法和结果通过一例测试心房起搏阈值的打印报告单说明测试的过程将起搏模式程控成DDD模式,增加起搏频率,延长A-V间期。通过心室心内心电图或直接连接体表心电图,观察QRS波,直至窄QRS波演变成心室起搏的宽QRS波,未引起波变化的最小电压就是心房起搏阈值。结论通过心室起搏心电图的变化来明确心房起搏阈值是传统方法的补充。     

先天性长QT综合征的研究进展

先天性长QT综合征 (LQTS)是以QTc异常延长、反复发作致命性心律失常如尖端扭转性室性心动过速 (TDP)及心室颤动、临床上反复发生心源性晕厥、并常导致猝死为特征的常染色体显性或隐性遗传的单基因疾病〔1〕。1　病因及发病机制1 .1　病因LQTS是一种遗传性的心脏病 ,已经发现了LQTS的 2个主要临床综合征 :较常见的为只存在于心脏疾患的表现型 ,即常染色体显性遗传型(RWS) ;较罕见的为常染色体隐性遗传 (JLNS) ,心脏病变与先天性耳聋并存。现已发现了引起LQTS的 7个基因 (表 1 ) 〔1〕。表 1　LQTS致病基因一览表表现型基因型染…     

Therapeutic effect of a dualchamber pacemaker with the optimized program-control mode on long-qt syndrome

Objectives To explore the optimized program-control mode of a dual-chamber pacemaker combined withβ-blocker to treat congenital long QT syndrome(LQTS). Methods 12 LQTS patients in our hospital that still have symptoms despite use of regular drug therapies or that can not endure the therapies were implanted with DDD cardiac pacemaker.The QT/QTc intervals of those patients were measured at different pacing rates respectively.Their cardiac pacemakers were all programmed to selectively turn on and turn off some related functions at the pacing rate of 80 beats/min.The dosage ofβ-blockers was adjusted according to the patients’ PR intervals and blood pressures.The MACE and the cardiac function of the patients were recorded after operation.Results The measured QT / QTc interval decreased with the pacing rate increasing.The pacing rate of 80 beats/min can make QT/QTc interval basically normal. The MACE of the patients were statistically declined(P = 0.003) and no negative effect on cardiac function was found during the follow-up.Conclusions The optimized program-control mode of a dual-chamber pacemaker combined withβ-blocker to treat congenital LQTS are:to pace at the rate of 80 beats/min and program to turn off lag,sleep,automatic preventing PMT and automatic threshold-capture feature and turn on the PVC,rate adaptation and atrioventricular node priority function.     

先天性Q-T间期延长综合征研究进展

先天性Q- T间期延长综合征是一组家族遗传性疾病。本综述回顾了该疾病的病因、临床特点和治疗等方面的新进展 ,为进一步深刻认识该疾病提供有益的帮助。     

先天性QT延长综合征尖端扭转型室性心动过速的发作方式

目的　研究先天性QT延长的尖端扭转型室性心动过速 (室速 )发作方式及其临床意义。方法　回顾性分析 5 5例因反复晕厥而确诊为先天性QT延长综合征病人的心电图 ,其中 16例记录到尖端扭转型室速开始发作的图形。结果　共记录到尖端扭转型室速 14 9阵 ,130阵为间隙依赖性。结论　间隙依赖性尖端扭转型室速曾经被认为是后天获得性QT延长综合征标志 ,研究表明其在先天性QT延长综合征尖端扭转型室速发作中也起重要的作用     

Possible bradycardic mode of death and successful pacemaker treatment in a large family with features of long QT syndrome type 3 and Brugada syndrome

INTRODUCTION: We recently identified a novel mutation of SCN5A (1795insD) in a large family with features of both long QT syndrome type 3 and the Brugada syndrome. The purpose of this study was to detail the clinical features and efficacy of pacemaker therapy in preventing sudden death in this family. METHODS AND RESULTS: The study group consisted of 116 adult family members: 60 carriers (29 males) and 56 noncarriers (28 males) of the mutant gene. Investigations included 24-hour Holter monitoring, ergometry, and electrophysiologic studies. Mean, lowest, and highest heart rate were lower in the carriers, but heart rate variability was comparable. In carriers, disproportional QT prolongation was present during bradycardia. No complex ventricular ectopy was recorded, and there were fewer isolated premature beats (both ventricular and atrial) in carriers. All patients were asymptomatic, except for two individuals who experienced syncope; in one of these patients, asystolic episodes (up to 9 sec) were repeatedly recorded. Prolonged HV intervals were present in 5 of 6 patients. Thirty carriers received a prophylactic backup pacemaker. During median follow-up of 4.5 years (range 0.0 to 22.6), their survival rate was 100%. There were five sudden deaths among the remaining 30 carriers without a pacemaker (P = 0.019). CONCLUSION: This family with a high incidence of nocturnal sudden death is characterized by bradycardia-dependent QT prolongation, intrinsic sinus node dysfunction, and generalized conduction abnormalities. There is a striking absence of complex ventricular ectopy, and pacemaker implantation was effective in preventing sudden death. These findings raise the possibility of a bradycardic rather than tachycardic mode of death.     

先天性长QT综合征七例的临床研究

目的 对1例先天性长QT综合征（long QT syndrome,LQTS)患者（先证者）所在的家族进行普查,研究该家族的发病情况及临床和心电图特点,推测其相应的表现型和基因型。方法 按常规采集26例家庭成员的临床病史,进行体格检查,并采集同步12导联心电衅,测量QT间期和校正的QT间期,采用Schwartz提出的评分标准作为LQTS的诊断标准。结果 26例中有7例（28%）长QT综合征患者,6例可疑诊断。发生晕厥的诱因均为情绪激动或体力劳动,心电图表现为QT间期延长,在发病前后延长得更加明显,T波宽大有切迹,多可见U波,病情严重的患者心电图表现更加典型,该家族中患者的首次发病年龄较在,预后好,没有1例发生猝死或未成年夭折。结论 该家族中LQTS患者的临床和心电图表现符合LQTS1或者LQTS2。基因型有可能为KVLQT1或者HERG基因的突变。     

T-wave patterns associated with the hereditary long QT syndrome

Mutations involving 6 different ion-channel genes have been identified in subjects with the hereditaryLong QT Syndrome. These gene mutations result in structural and functional changes in ion-channel proteins withresultant alterations in potassium and sodium repolarization currents that affect the morphologic features ofelectrocardiographic repolarization. This review highlights the genotype-phenotype associations related toventricular repolarization that have been reported in the LQTS literature, with particular focus on ECG T-wavepatterns in LQT1, LQT2, and LQT3 genotypes.     

Septic cardiomyopathy as a cause of long QT syndrome

A 63-year-old woman admitted with 2:1 infranodal atrioventricular block subsequently developed ventricular dysfunction incident to septic syndrome. Concomitant changes included an abnormally prolonged QTc interval (600 ms) and the occurrence of torsade de pointes. Restoration of a normal QTc interval and cessation of torsade de pointes was coincident with return of normal ventricular function and remission of sepsis. This report supports the view that sepsis-induced cardiomyopathy is another cause of the long QT syndrome.