超声心动图评价致心律失常性右心室心肌病病变程度的临床研究

目的 分析致心律失常性右心室心肌病(ARVC)患者的病变程度与超声心动图表现之间的关系.方法 分析61例已确诊的ARVC患者,根据心脏磁共振(MRI)检查结果,将其按病变侵犯部位分为右心室局部病变组(A组)、右心室弥漫病变组(B组)、累及左心室的双心室病变组(C组),分析比较3组间的超声心动图.结果 心脏MRI结果示,...     

超声心动图对致心律失常性右室心肌病的诊断评价

目的分析超声心动图诊断致心律失常右室心肌病（ARVC）的图像特点。方法对5例ARVC患者的临床资料及超声心动图检查结果进行分析。结果5例ARVC患者中4例以室性心律失常发病,1例为家系检查发现。1例猝死、1例心力衰竭、2例晕厥、1例无症状。所有患者均做了心电图、超声心动图检查,部分患者做了心室核素造影和右心室造影。超声心动图表现为：所有患者均右心室扩大,3例右室流出道扩张,1例左室稍大;3例右室壁运动弥漫性减弱,1例局限性减弱,1例右室壁瘤形成,2例室间隔运动异常。5例患者三尖瓣、肺动脉瓣均有反流,反流压差较低,前向流速减低。结论ARVC超声心动图的表现为右心室扩大、右室流出道扩张、右室壁弥漫性、局限性变薄、运动异常。患者以反复发作性室性心动过速、晕厥、猝死为首发症状,在除外其他可引起右室扩大的病变后可提示本病。     

心电图评价致心律失常性右心室心肌病病变程度的临床价值

目的 分析致心律失常性右心室心肌病(ARVC)患者的病变程度与心电图表现之间的关系.方法 分析61例已确诊的ARVC患者,根据心脏核磁共振成像(MRI)检查结果,将其按病变侵犯部位分为右心室局部病变组、右心室弥漫病变组、双心室病变组,分析比较三组的心电图特征.结果 心脏MRI结果显示右心室局部病变组19例(31%),右心室弥漫病变组28例(46%),双心室病变组14例(23%).心电图正常者3例,三组中各1例.伴有Epsilon波的患者24例(39%)、V1～V3导联的QRS波时限≥110 ms的患者21例(34%)、V1～V3导联S波升支≥55 ms的患者17例(28%)、完全右束支传导阻滞的患者10例(16%)、病理性Q波的患者9例(15%),这些指标的发生率均随病变程度的加重而增高(右心室局部病变组＜右心室弥漫病变组＜双心室病变组).Epsilon波、V1～V3导联的QRS波时限≥110 ms、完全性右束支传导阻滞(RBBB)、病理性Q波的发生率在双心室病变组中要高于右心室局部病变组,且两组间比较差异有统计学意义(P＜0.05).V1～V3导联S波升支≥55 ms的发生率在双心室病变组中要高于右心室局部病变组,且两组间比较差异有统计学意义(P＜0.05);在双心室病变组要高于右心室弥漫病变组,且两组间比较差异均有统计学意义(P均＜0.05).一度房室传导阻滞的发生率在双心室病变组中要高于右心室弥漫病变组,且两组间比较差异有统计学意义(P＜0.05).右心室局部病变组患者心电图T波倒置多局限于V1导联,右心室弥漫病变组和双心室病变组T波倒置多数表现于胸前导联V1～V3或超过V3导联的胸前导联、以及下壁导联.结论 心电图正常并不能排除ARVC.ARVC患者T波倒置在12导联心电图上具有很高的发生率,并且T波倒置在胸部导联的延伸与病变程度是相关的,T波倒置的范围可以提示ARVC病变累及的程度.

Abstract：

Objective To analyze the relationship between electrocardiographic (ECG) features and disease severity in patients with the arrhythmogenic right ventricular cardiomyopathy (ARVC). Method The study group consisted of 61 subjects with a definite diagnosis of ARVC on the basis of published guideline criteria and patients were divided into 3 subgroups according to the extent of diseased myocardium defined by cardiac magnetic resonance imaging (MRI): Group A: local involvement (n = 19, 31% ), Group B: diffuse involvement of whole right ventricle ( n = 28, 46% ) and Group C: involvement of both right and left ventricles ( n = 14, 23% ). Results Normal electrocardiogram was shown in 1 patient in each group.Epsilon wave was detected in 24 (39%) patients, QRS duration was prolonged [≥ 110 ms( V1 -V3 )] in 21 (34%) patients, S-wave upstroke was prolonged (≥55 ms) in 17 (28%) patients, complete right branch bundle block was evidenced in 10 ( 16% ) patients and pathologic Q waves was found in 9 ( 15% ) patients. The incidence of above abnormal ECG changes was increased in proportion to the degree of disease severity (group A ＜ group B ＜ group C). Incidence of Epsilon wave and prolonged QRS duration [≥ 110 ms (V1 - V3 )] were significantly higher in Group C than in Group A. Incidence of prolonged S-wave upstroke ( ≥55 ms) was significantly higher in Group C than in Group A and Group B. T-wave inversion in V1 leads was often found in Group A. T-wave inversion in inferior leads ( V1 - V3 leads or beyond V3 ) was often presented in Group B and Group C. Conclusions Normal ECG does not exclude the possibility of diagnosis of ARVC. The extent of T-wave inversion in the precordial leads and incidence of Epsilon wave, prolonged QRS duration [≥ 110 ms (Vt -V3 )] and prolonged S-wave upstroke ( ≥55 ms) were related to degree of disease severity in patients with ARVC.     

致心律失常性右心室发育不良的超声心动图特点分析

目的:分析致心律失常性右心室发育不良(ARVC)的超声心动图表现,结合多排螺旋CT、心血管造影等影像学手段,以及心电图等特异性表现,提高超声工作者对ARVC的认识,减少误诊率。方法:分析ARVC的超声心动图特征,并利用二维斑点追踪技术测量右心室壁各节段应变率,与正常组对比分析。结果:超声心动图可表现为右心室增大、右心室流出道增宽、右心室壁运动弥散性减弱以及局部减弱,部分病例可见室壁瘤形成,三尖瓣反流多见等。二维斑点追踪显示ARVC组整体右心室壁应变率减低,以右心室心尖段为著。结论:超声心动图结合患者临床、心电图及其他影像学检查将大大提高ARVC的诊断率,超声心动图可较早发现患者的心脏改变,是评估患者预后及随访观察的重要手段。     

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。     

致心律失常性右室心肌病超声心动图的诊断进展

超声心动图诊断致心律失常性右室心肌病(ARVC)技术有三维超声、组织多普勒、斑点追踪;三维超声能更好的评价右室容积及射血分数,组织多普勒能更准确反映心肌收缩舒张功能变化,而斑点追踪则能定量描绘室壁节段运动异常。这些技术的运用为ARVC的早期诊断提供了更多有用信息。     

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。     

右心室壁传导阻滞在致心律失常性右心室心肌病诊断中的地位

目的 探讨右心室壁传导阻滞(parietal block)在致心律失常性右心室心肌病(arrhythmogenic right ventricular dysplasia,ARVC)诊断标准中的适用性.方法 回顾性分析可疑ARVC先证者62例的临床评估资料,项目包括性别、心电图、24 h动态心电图、超声心动图.对比分析...     

致心律失常性右心室心肌病

致心律失常性右心室心肌病(ARVC)亦称致心律失常性右心室发育不良,是一种心肌细胞逐渐被脂肪和纤维组织取代,病变以右心室为主,有一定家族倾向的原发性心肌病〔1〕。本病最重要的临床意义在于心脏猝死,在30岁以下的猝死患者中,ARVC可占20%〔2〕。1病因探讨遗传因素至少30%的ARV     

致心律失常性右心室心肌病

致心律失常性右心室心肌病是一种少见的心肌疾病 ,易发生心律失常、心力衰竭和猝死。本文将对其发病机制、临床表现、病理特征、诊断及治疗方面的研究进展进行综述     

37例致心律失常性右心室心肌病患者PKP2基因检测

目的 调查单中心致心律失常性右心室心肌病(ARVC)患者PKP2突变发生率.方法 对50例考虑诊断为ARVC的患者采用2010年新诊断标准进行重新评估.采用聚合酶链式反应(PCR)扩增PKP2基因各外显子片段并测序,结果与200例正常对照组进行比对分析.结果 37例被确诊ARVC,9例为临界诊断,另4例为疑似诊断.确诊患者中有10例(27％)携带7个新突变和3个已报道突变,包括7个无义突变和3个错义突变,临界诊断及疑似诊断患者均未检测出PKP2基因突变.携带PKP2突变的患者与未携带突变的患者临床特征差异无统计学意义.结论 本组ARVC患者PKP2基因突变发生率与欧美国家相似,但突变谱存在差异.     

Prognostic value of cardiovascular magnetic resonance in patients with suspected arrhythmogenic right ventricular cardiomyopathy

Background

Early recognition and accurate risk stratification are important in the management of arrhythmogenic right ventricular cardiomyopathy (ARVC). Identification of predictors of outcome by cardiovascular magnetic resonance (CMR) in patients undergoing evaluation for ARVC is limited. We investigated the predictive value of morphological abnormalities detected by CMR for major clinical events in patients with suspected ARVC.

Methods

We performed a longitudinal study on 369 consecutive patients with at least one criterion for ARVC. Abnormal CMR was defined by the presence of one of the following: increased right ventricular (RV) volumes, reduced RV ejection fraction, RV regional wall motion abnormalities, myocardial fatty infiltration, and myocardial fibrosis. The end-point was a composite of cardiac death, sustained ventricular tachycardia, ventricular fibrillation, and appropriate ICD discharge.

Results

Twenty patients met the composite end-point over a mean follow-up of 4.3 ± 1.5 years. An abnormal CMR was an independent predictor of outcomes (p < 0.001). The presence of multiple abnormalities heralded a particular high risk of events (HR 23.0, 95% CI 5.7–93.2, p < 0.001 for 2 abnormalities; HR 35.8, 95% CI 9.7–132.6, p < 0.001 for 3 or more abnormalities). The positive predictive value of an abnormal CMR study was 21.0% for an adverse event, whilst the negative predictive value of a normal CMR study was 98.8% over the follow-up period.

Conclusions

CMR provides important prognostic information in patients under evaluation for ARVC. A normal study portends a good prognosis. Conversely, the presence of multiple abnormalities identifies a high risk group of patients who may benefit from ICD implantation.     

碎裂QRS波在致心律失常性右心室心肌病诊断中的价值

目的评价碎裂QRS波（fQRs波）在致心律失常性右心室心肌病（ARVC）诊断中的价值。方法43例符合ARVC诊断标准的患者,男性33例,平均年龄（40．4±13．9）岁。采集临床资料,记录静息状态下标准12导联心电图,描述fQRs波的心电图特征,判断是否存在fQRs波、epsilon波和右胸前导联T波倒置（TWI）并比较其阳性率之间的差异。结果本组患者fQRs波以下壁导联（44．3％）和右胸前导联（24．2％）最为多见。QRS波中,fQRs波见于R波者多于S波者（58．4％对32．9％,Z=4．30,P〈0．01）。共31例患者判断为fQRS波阳性,2～9（4．6±1．7）个／例。fQRs波阳性率较epsilon波（73．8％VS30．2％,Z=3．67,P〈0．01）和TWI（73．8％对41．9％,Z=2．61,P〈0．01）显著增高。结论fQRs波阳性是ARVC患者常见的心电图异常,常见于下壁和右胸前导联,可作为提示诊断的无创心电指标。     

碎裂QRS波在致心律失常性右心室心肌病诊断中的价值

Objective To evaluate diagnostic value of fragmented QRS complex (fQRS)in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods Forty-three patients [33 men, aged (40. 4 ± 13.9)years]meet the ISFC/ESC diagnostic criteria for ARVC were enrolled in this study. A standard twelve-lead electrocardiogram was obtained during the resting status. Characteristics of fQRS were detailedly studied by three doctors independently. A comparison of the prevalence among fQRS, epsilon wave and T wave inversion( TWI )in the right precordial leads exceeding V3 was done. Results Most fQRS could be found in the inferior leads (44. 3% ) and the right precordial leads (24. 2% ). Within the QRS complex, the prevalence of fQRS in the R wave was significantly higher than it in the S wave(58. 4% vs 32. 9% ,Z =4. 30,P ＜0. 01 ).fQRS could be found in a total of 31 of 43 cases( mean 4. 6 ± 1.7 ( range 2 to 9) per patient). The prevalence of fQRS was significantly higher than that of epsilon wave ( 73.8% vs 30. 2%, Z = 3.67, P ＜ 0. 01 ) and TWI (73.8% vs41.9% ,Z =2. 61 ,P＜0. 01 ). Conclusion fQRS was a common electrocardiographic abnormality,and most was found in the inferior and right precardial leads in patients with ARVC. It may be used as an important noninvasive preliminary screening electrocardiographic criteria.     

致心律失常性右心室心肌病的QRS及QT离散度研究

目的　探讨致心律失常性右心室心肌病 (arrhythmogenicrightventricularcardiomyopathy ,ARVC)危重患者、家系中无症状患者及家系中可疑患者的QRS及QT离散度的存在规律。方法　按欧洲心脏病学会 (ESC)的诊断标准 ,选择有室性心动过速、晕厥或心脏骤停 (cardiacarrest)史的ARVC患者 2 5例为危重组 ,家系调查发现的家系中无明显症状的ARVC患者 16例为无症状组 ,家系中高度怀疑为ARVC但尚未达到诊断标准的 18例为可疑组 ,正常对照组为 14例。同步记录标准 12导联心电图。测量每个导联QRS时限 ,记录 12导联中的QRS时限最大值 (QRSm ,最长的 1个QRS值 )、QRS离散度 (QRSd ,12导联中最长的 1个QRS值减最短的 1个QRS值 )及QRS时限平均值 (QRSa)。测量每个导联QT间期 ,记录 12导联中的QT最大值 (QTm ,最长的 1个QT间期 )、QT平均值 (QTa)、QT离散度 (QTd ,导联中最长的 1个QT值减最短的 1个QT值 )、校正QTd(QTcd)及校正QTm(QTcm)。比较各组各指标的差异。结果　可疑组的年龄低于对照组、危重组与无症状组。 4组之间QRSd差异无显著性。QTd在危重组为 (6 9 6± 4 1)ms,在无症状组为 (77 3± 5 7)ms均显著高于对照组 (5 5 4±6 3)ms(P均为 0 0 0 3) ;危重组与无症状组差异无显著性。QRSm、QRSa、QTa、QTcd、QT     

评价致心律失常性右室心肌病右室功能超声指标与磁共振的相关性

目的用目前公认的5个超声指标评价致心律失常性右室心肌病（ARVC）患者的右室功能,研究其与磁共振（MRI）结果的相关性。方法对11例ARVC患者行超声及MRI检查,测量右室功能指标并行相关分析。本研究除了运用传统的心尖四腔心法测量右室面积改变分数（RVFAC 4C）外,增加了胸骨旁右室三腔心切面法测量右室面积改变分数（RVFAC RV 3C）。结果 5个指标中胸骨旁短轴RVFAC RV 3C、三尖瓣环收缩峰值速度、三尖瓣环收缩位移与MRI结果相关,r值分别为0.72、0.65、0.67。结论胸骨旁短轴RVFAC RV3C是评价ARVC患者右室功能的重要指标并且其与MRI测量的结果具有高度的相关性。     

植入型心律转复除颤器在致心律失常性右心室心肌病患者中的应用

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)治疗致心律失常性右心室心肌病(ARVC)的经验.方法 入选我院2004年3月至2012年3月确诊并植入ICD的ARVC患者,收集临床资料,进行常规电话及门诊随诊.结果 共18例患者[男12例,女6例,平均年龄(46.4±13.8)岁]入选,平均随访(46.6±30.5)个月,有6例患者出现了111次室性心动过速(VT)/心室颤动(VF)事件,其中仅1次VF事件,其余均为VT事件,其中3例患者术后共出现了7次电风暴.ICD治疗组中的C反应蛋白(CRP)明显高于ICD未治疗组.111次VT/VF事件中,12次经电除颤终止心动过速,其余均通过抗心动过速起搏(ATP)终止了心动过速.结论 ARVC患者ICD植入后的VT/VF事件以VT为主,多数可经ATP终止;出现电风暴的ARVC患者易反复发作恶性心律失常;CRP可能对ICD治疗具有预测价值.