致心律失常右心室心肌病心电图特征波新认识——附罕见粗大Epsilon波二例

致心律失常性右心室心肌病(ARVD)是主要累及右心室的非缺血性心肌病.90%的ARVD患者存在心电图异常,其中最常见的心电图改变为V1～V3导联T波倒置,大约25%～35%的患者存在特征性的Epsilon波.     

致心律失常右心室心肌病心电图特征波新认识——附罕见粗大Epsilon波二例

致心律失常性右心室心肌病(ARVD)是主要累及右心室的非缺血性心肌病.90%的ARVD患者存在心电图异常,其中最常见的心电图改变为V1～V3导联T波倒置,大约25%～35%的患者存在特征性的Epsilon波.     

巨Epsilon波:一种右心室心肌病罕见的心电图现象

近年来,我们陆续注意到一种罕见的心电现象,国内诊断为心室分离,国外早年也曾诊断为心室分离,但新近对这种心电图现象有了更深入的认识,现通过3例病例予以分析.     

致心律失常性右心室心肌病的心电图特征

目的探讨国人致心律失常性右心室心肌病患者临床心电图特征。方法分析32例致心律失常性右心室心肌病患者体表心电图各项参数。结果心电图记录到Epsilon波12例,QRS时间（V1＋V2＋V3）／（V4＋V5＋V6）≥1.2共15例,终末激动时间延长17例,出现QRS波群碎裂23例,可见异常Q波8例,V1～V3T波倒置且不存在束支传导阻滞14例,完全性右束支传导阻滞3例,不完全性右束支传导阻滞1例。28例记录到室性心动过速。结论Epsilon波、QRS时间（V1＋V2＋V3）／（V4＋V5＋V6）≥1.2、终末激动时间延长≥55ms及QRS波群碎裂是致心律失常性右心室心肌病特征性的体表心电图改变。     

巨大Epsilon波致心律失常性右室心肌病临床和心电图特点

目的总结心电图表现为巨大Epsilon波致心律失常右室心肌病(ARVC)患者的临床和心电图特点。方法分析本院于2011年1月至2014年12月收治的12导联心电图示巨大Epsilon波ARVC患者的临床和心电图特点。通过查询Medline、CNKI、VIP和万方数据库等总结该心电图特征ARVC患者的特点。结果 5例患者中男性4例。首发症状均为心悸、头昏。发作时心电图为右室起源室性心动过速。心脏超声示右室扩大;右室壁变薄,可见小凹。心电图V_1导联QRS呈"M"型;巨大Epsilon波振幅为(0.37±0.16)mV,振幅与S波比值为1.20±0.23;持续时间为(90±12.75)ms,持续时间与QRS波相比为0.60±0.06。文献报道5例患者均为男性。临床表现为心悸,晕厥发作。心电图V_1导联QRS均呈rsR′,Fontain导联、心向量和晚电位证实该R′波为Epsilon波。V_1导联Epsilon波振幅为(0.52±0.35)mV,振幅与S波比值为1.18±0.74;持续时间(96.0±11.4)ms,持续时间与QRS波相比为0.65±0.08。结论临床上巨大Epsilon波ARVC患者少见,右室激动延缓,心律失常发生率高。     

致心律失常右室发育不良心肌病表现Epsilon波一例

患者男性 ,4 2岁。因发作性心悸、胸闷 8年 ,加重 5个月 ,于 2 0 0 2年 3月 1日入院。 8年来反复发作心悸、胸闷 ,持续数分钟至数小时不等 ,可自行消失。发作时无抽搐、晕厥及大小便失禁等表现。曾在外院就诊心电图记录到室性心动过速 (室速 )。近 5个月上述症状发作频繁 ,因严重室性心律失常入院。否认家族中类似疾病史。查体 :体温 36 9℃ ,脉搏 5 6次 /min ,呼吸 2 0次 /min ,血压 14 0 /85mmHg(1mmHg =0 133kPa)。神清 ,双肺呼吸音清晰 ,心率 5 6次 /min ,律齐 ,未闻及杂音。X线胸片 :心脏扩大 ,以右室扩大为主。超声心动图 :右室扩…     

致心律失常性右心室心肌病的心电图进展

致心律失常性右心室心肌病（ARVC）为遗传性心肌病,1905年由Oslen首次报道;1978年由法国学者Frank Fontaine命名为致心律失常性右心室发育不良（ARVD）;1995年WHO正式归于心肌病范畴,命名为ARVC;2006年新颁布的心肌病分类将ARVC归属于遗传性原发性心肌病。     

致心律失常右心室心肌病

<正>致心律失常性右心室心肌病(ARVC)是一种主要累及右心室心肌组织的疾病,过去国内报道较少,与对该病认识不足有关。近年来,随着医疗技术水平的不断提高,对该病的研究特别在诊断手段和治疗上有很大进展。一、概况致心律失常性右心室心肌病(arrhythmogenic right ven-tricular cardiomyopathy,ARVC),也称致心律失常性右心室发育不良(arrhythmogenic right ventricular dysplasia,ARVD),最早于1961年由Dalla描述,但作为疾病由Frank等于1978年首先定名,被定义为累及右心室的原因不明心肌病。世界卫     

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

目的 探讨不同体表心电图记录方法对国人致心律失常性有室心肌病(arrhythmogenic right ventricular eardiomyopathy,ARVC)患者Epsilon波检出率的影响.方法 共人选ARVC患者32例,男性24例,女性8例,年龄18～70(42.3±13.3)岁.于安静仰卧位记录窦性心律情况下的标准12导联同步心电图(standard twelve leads electrocardiography,S-ECG)、右胸导联心电图(right precordial leads ECG,R-ECG)及Fontaine双极胸导联心电图(Fontaine bipolar precordial leads ECG,F-ECG)o Epsilon波判断标准为位于QRS波之后、sT段起始部的低波幅棘波、振荡波或平缓电位.结果 该组病例S-ECG、R-ECG及F-ECG对Epsilon波的检出率分别为37.5%、37.5%和50.0%,三种心电图记录方法之间Epsilon波检出率的比较差异无统计学意义(均为P＞0.05).其中1例Epsilon波仅见于S-ECG,3例仅见于R-ECG,5例仅见于F-ECG.S-ECG联合R-ECG的检出率为50.0%,S-ECG联合F-ECG的检出率为56.3%,三种记录方法的联合检出率为65.6%,S-ECG联合F-ECG的枪出率与S-ECG相比明显提高(P＜0.05),联合三种心电图记录方法的检出率与S-ECG相比有显著提高(P＜0.01).结论 联合使用S-ECG、R-ECG及F-ECG三种心电图记录方法,可以显著提高ARVC患者Epsilon波的检出率,且三种记录方法之间可以相互补充.     

Two autopsied cases of arrhythmogenic right ventricular dysplasia

Autopsy studies of arrhythmogenic right ventricular dysplasia (ARVD) have rarely been reported, and its etiology remains unknown. The present report describes a detailed histopathological study of two autopsied cases of ARVD. Case 1: This 21-year-old man experienced palpitation accompanied by syncope. He died after ventricular tachycardia of right ventricular origin. The heart weighed 365 g and the right ventricular cavity was markedly dilated. The distribution of fatty tissue was roughly limited to the middle layer of the free wall, replacing the myocardium with fatty degeneration. Medial hyperplasia of the small arteries within the fatty tissue was also observed. Fibroelastosis was observed in the left ventricular endocardium. In the conduction system, fatty tissue was found in the sinus node. In addition, medial hypoplasia was observed in the pulmonary arteries. Case 2: This 32-year-old man who had had an arrhythmia for 10 years died of ventricular tachycardia of right ventricular origin. His older brother also died of heart disease. His heart weighed 515 g and both the right and left ventricles were dilated. Fatty tissue, unlike that in Case 1, was shown to markedly infiltrate from the epicardium into both the right and left ventricular walls. In the right ventricular wall, muscle layers disappeared in some portions. In the conduction system, fatty tissue was observed in the sinus node. Although ARVD may be considered a syndromic entity, individual cases are different in terms of pathological morphology, with possible variations in the etiology and pathogenesis.     

Cardioangiographic findings in patients with arrhythmogenic right ventricular dysplasia

The dimension, contractility, and regional wall motion of the right and left ventricles were scored on the angiograms of 13 patients with arrhythmogenic right ventricular dysplasia. In 10 patients the right ventricle was enlarged, in eight the contractility of the right ventricle was reduced, and in all but one patient there were regional wall motion abnormalities of the right ventricle. The most common abnormality of regional wall motion was mild hypokinesia. There were bulging or dyskinetic areas in seven patients. Regional wall motion abnormalities of the left ventricle were found in five patients, two of whom also had bulging or dyskinetic areas. The reproducibility of right ventricular dimension, contractility, and regional wall motion scores was generally fair but varied unexpectedly both within and between two observers (Kendall's Tau 0.38-0.92). The score values of regional wall motion for some of the segments differed considerably within and between observers. One of the observers consistently gave higher scores than the other. These data suggest that a more objective approach is needed for evaluating angiographic changes in arrhythmogenic right ventricular dysplasia.     

Two simultaneous right ventricular tachycardias in a case of arrhythmogenic right ventricular dysplasia

A 51 year old woman with arrhythmogenic right ventricular dysplasia had two types of ventricular tachycardia--(a) a regular and sustained tachycardia and with normal frontal plane axis on electrocardiography and (b) an irregular non-sustained tachycardia with a leftward frontal plane axis. Changes in the QRS complex were sometimes seen during the sustained ventricular tachycardia. The clinical, electrocardiographic, and electrophysiological data were consistent with the diagnosis of two different and sometimes simultaneous tachycardias originating in the right ventricle. This case suggests a possible new mechanism for the multiform appearances of the ventricular tachycardia.     

Cardioangiographic findings in patients with arrhythmogenic right ventricular dysplasia.

The dimension, contractility, and regional wall motion of the right and left ventricles were scored on the angiograms of 13 patients with arrhythmogenic right ventricular dysplasia. In 10 patients the right ventricle was enlarged, in eight the contractility of the right ventricle was reduced, and in all but one patient there were regional wall motion abnormalities of the right ventricle. The most common abnormality of regional wall motion was mild hypokinesia. There were bulging or dyskinetic areas in seven patients. Regional wall motion abnormalities of the left ventricle were found in five patients, two of whom also had bulging or dyskinetic areas. The reproducibility of right ventricular dimension, contractility, and regional wall motion scores was generally fair but varied unexpectedly both within and between two observers (Kendall's Tau 0.38-0.92). The score values of regional wall motion for some of the segments differed considerably within and between observers. One of the observers consistently gave higher scores than the other. These data suggest that a more objective approach is needed for evaluating angiographic changes in arrhythmogenic right ventricular dysplasia.     

Exercise-induced ST elevation in patients with arrhythmogenic right ventricular dysplasia

To test the hypothesis that local or diffuse wall motion abnormalities in the right ventricle in patients with arrhythmogenic right ventricular dysplasia (ARVD) may induce the ST-segment elevation in response to exercise, we examined exercise electrocardiograms in patients with ARVD. In 17 patients with ARVD, who demonstrated right ventricular wall motion abnormalities without organic coronary lesions, we conducted a treadmill exercise test. Significant exercise-induced ST-segment elevation (ESTE) was defined as a 0.1 mV or more ST-segment elevation at J point. ESTE was observed in 11 patients (65%). It manifested most frequently in right-sided precordial leads. Severe right ventricular asynergy was seen in all but one (91%) among 11 with ESTE, whereas it was seen only in two (33%) among six without ESTE (P<.05). The maximal magnitude of ESTE inversely correlated with right ventricular ejection fraction (r = -0.58, P<.05). ESTE was seen in two thirds of ARVD patients, helping us noninvasively diagnose ARVD. The fact that ventricular wall motion abnormalities could cause ESTE in the absence of organic coronary lesions suggested the critical role of mechanical factors in the genesis of ESTE.     

Two simultaneous right ventricular tachycardias in a case of arrhythmogenic right ventricular dysplasia.

A 51 year old woman with arrhythmogenic right ventricular dysplasia had two types of ventricular tachycardia--(a) a regular and sustained tachycardia and with normal frontal plane axis on electrocardiography and (b) an irregular non-sustained tachycardia with a leftward frontal plane axis. Changes in the QRS complex were sometimes seen during the sustained ventricular tachycardia. The clinical, electrocardiographic, and electrophysiological data were consistent with the diagnosis of two different and sometimes simultaneous tachycardias originating in the right ventricle. This case suggests a possible new mechanism for the multiform appearances of the ventricular tachycardia.