超声对致心律失常性右室发育不良的评价

致心律失常性右室发育不良（ａｒｒｈｙｔｈｍｏｇｅｎｉｃｒｉｇｈｔｖｅｎｔｒｉｃｕｌａｒｄｙｓｐｌａｓｉａ;ＡＲＶＤ）是一种少见的、原因不明的、病变主要累及右心室的心肌病。近年来,随着对该病认识的不断深入提高,其检出率呈逐渐上升的趋势。本文通过对一组临床上确诊为ＡＲＶＤ的患者进行超声心动图的研究,从而探讨超声心动图对ＡＲＶＤ的诊断价值。１　资料与方法１９９５年１０月至１９９９年５月,我院住院患者中,经心电图、心血管造影及心肌活检确诊为ＡＲＶＤ者４８例,男３５例,女１３例,平均年龄３１±５岁（１３…     

致心律失常性右室发育不良的诊治进展

致心律失常性有室发育不良（arrhythmogenicrightven－triculardysplasia,ARVD）是一种主要累及右,动室心肌组织的疾病,且多局限于右室的漏斗部前壁、心尖及右室下壁区域,Marcus等[1]称之为“发育不良三角”（triangleofdysplasia）。1978年法国学者Frank等首次描述[2]。该病原因不明,有家族群集倾向,30％有家族史;新近研究认为是由于14号常染色体长臂（14q23～q24）异常所致的显性遗传病[3]。现对该病的诊治进展综述如下。1临床表现该病好发于男性,男：女约为3：1;好发年龄16～65岁,平均30岁左右。临床特点为反复发作的左束支…     

致心律失常性右室发育不良的影象学改变

致心律失常性右室发育不良（arrhythmogenicrightventriculardisplasia,ARVD）为一少见病,1905年Osier首次报道,1988年Bari国际会议才统称之为ARVD,国内报道较少,可能与对该病认识不足有关。其病理改变主要是右心室心肌细胞稀少。缺失,代之以脂肪组织和纤维组织,受累的范围可从几乎整个右心室肌组织消失到散在分布小岛状脂肪和纤维组织不等。常由于恶性室性心律失常的发作而辞死。1ARVD影像学研究对ARVD目前诊断主要依据影象学和心电图改变。对其影象学改变目前尚缺乏系统的认识。本文结合国内外报道综述如下。1．目超声心动…     

致心律失常性右室发育不良临床观察

致心律失常性右室发育不良（ＡＲＶＤ）临床少见，常表现反复发作室性心动过速（室速）。笔者回顾近６年收治的７例患者认为：对反复发作左束支传导阻滞（ＬＢＢＢ）型室速患者，如未发作时心电图呈不完全性右支传导阻滞，Ｖ＿１～Ｖ＿４导联见Ｅｐ－ｓｉｌｏｎ波，超声心动图等检查发现右心室形态和／或功能异常，在排除其它疾病后应考虑本病。本文４例用药无效，而用射频消融术，疗效显著。     

Brugada综合征和致心律失常型右室心肌病

Brugada综合征和致心律失常型右室心肌病（ARVC）两者临床表现相似,均可导致恶性心律失常和猝死。该文就Brugada综合征和ARVC在临床表现、心电图特征等方面进行了对比分析,并对两者的诊断、鉴别诊断及治疗进行简单介绍。     

致心律失常型右室发育不全一例报告

1临床资料 患者，男，34岁，农民。反复发作心悸、胸闷2年。2年前于本院就诊时因室性心动过速（VT）发作“阿一斯综合征”1次，经抢救回复窦性心律。否认慢性支气管炎及呼吸系统疾病史。本院及南昌大学第一附属医院超声心动图（UCG）均提示右室增大（32mm，正常20～25mm），调节束增强、增粗，其中2006年南昌大学第一附属医院MRI提示右室扩大，右室心肌部分被脂肪和纤维组织取代。患者ECG示完全性右束支传导阻滞，频发多形性室性期前收缩，伴有反复室速（QRS波形均呈左束支图形）发作，综上依据诊断：致心律失常型右室发育不全。平时使用胺碘酮片隔2d服用0．2g，共6个月，仍多次发作VT；调整至0．2g／d，VT发作明显减少。经追查患者家属，患者父母ECG正常，患者兄妹3人，其妹（30岁）ECG示频发多形性室性期前收缩，UCG示右室比例增大（右室29～30mm），右室调节束增强、增粗，患者无症状。其小妹（24岁）ECG仅见偶发室性早搏，未作进一步检查。综上，临床考虑为致心律失常型右室发育不全家系。     

致心律失常性右室心肌病表现Epsilon波致局限性QRS增宽1例

1病例报告患者，女，43岁，因反复发作性心悸2年，双下肢水肿1年，劳力性气促1个月余入院。患者于2年前开始出现发作性心悸、心慌不适，突发突止，持续20～30min／次左右，感头昏、乏力、出冷汗，伴晕厥1次，多次到当地医院就诊，血压测不到，心电图（ECG）示：宽QRS心动过速，心率200bpm以上，静推心律平可终止心动过速。近2个月间断口服胺碘酮治疗上诉症状有所缓解，1年前患者间断出现双下肢水肿，1个月前开始出现劳力性心累、气短不适，夜间喜高枕卧位。否认家族中类似疾病。查体：体温36．3℃，     

致心律失常型右室心肌病临床分析

致心律失常型右室心肌病，旧称致心律失常右室发育不良，其特征为右室心肌进行性被纤维脂肪组织所置换，早期呈典型的区域性，逐渐可累及整个右心室甚至部分左心室，而室间隔很少受累。     

致心律失常性右室心肌病的临床研究

目的：探讨致心律失常性右室心肌病（ARVC）的临床特点、诊断和治疗。方法：回顾分析2003～2007年入住复旦大学附属中山医院的58例ARVc患者的临床资料。分析ARVc的临床表现及心电图、超声心动图、MRI、电生理检查等,评价ARVC的检查手段及治疗方法。结果：58例患者多表现为反复胸闷、心悸。心电图、超声心动图及MRI均有特征性改变。治疗以药物为主,需长期维持治疗。结论：ARVC临床表现隐匿,以室性心律失常为主要表现,发作时常伴严重血流动力学障碍。Epsilon波和右胸导联QRS间期延长两者结合有助于ARVC的筛选和诊断。右室心尖部和流出道均为ARVC病变的好发邵位。射频消融治疗可行。     

Twenty-Seven-Year Follow-up of Arrhythmogenic Right Ventricular Dysplasia

OBATA, H., et al. : Twenty-Seven-Year Follow-up of Arrhythmogenic Right Ventricular Dysplasia. This case report describes clinical features, especially of surface ECG changes, observed for 27 years in a patient with arrhythmogenic right ventricular dysplasia (ARVD). The course of this patient was characterized by progressive deterioration of right ventricular function and progression of delayed potentials (so-called epsilon waves) following QRS complexes. However, the relation between ventricular arrhythmias and ECG changes or the degree of right ventricular abnormality was difficult to discern.     

Cardiac Sarcoidosis Masquerading as Right Ventricular Dysplasia

Patients with cardiac sarcoidosis may present with clinical and morphological features similar to arrhythmogenic right ventricular dysplasia (ARVD) or cardiomyopathy (ARVC). Three cases of cardiac sarcoidosis are presented that clinically mimicked ARVD or ARVC until a pathology diagnosis of sarcoidosis was made at biopsy or autopsy. A diagnostic distinction, while often difficult to make, is important since treatment with corticosteroids may benefit those with sarcoidosis but is not expected to be useful in cases with ARVD or ARVC. (PACE 2003; 26[Pt. I]:1498–1503)     

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: A Review

MARCUS, F.I., et.al .: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: A Review . Right ventricular dysplasia is being recognized with increasing frequency. It should be considered as a cause of ventricular tachycardia of left bundle branch block configuration and/or sudden unexpected death particularly during exercise in young men. The electrocardiogram (ECG) may show anterior precordial T wave inversion, particularly in lead V₂ and/or a QRS complex duration ≥ 110 ms in the right precordial leads. Echocardiographic studies focusing on the size and wall-motion abnormalities of the right ventricle are useful in confirming the diagnosis. Radionuclide angiography usually shows a moderately or markedly depressed right ventricular ejection fraction with normal or relatively well preserved left ventricular function. Cinemagnetic resonance imaging demonstrates abnormal fatty infiltration of the right ventricular myocardium and can show increased right ventricular dimensions as well as wall-motion abnormalities. Contrast ventricular angiography remains the gold standard to establish the diagnosis but must be performed with appropriate views and with care to avoid ventricular premature beats. Quantitative analysis of right ventricular dimensions can be performed in selected centers. Three-dimensional echocardiography is a promising approach to evaluate right ventricular wall-motion abnormalities as well as to demonstrate enlargement. The etiology and pathogenesis of this condition is not clear. A familial incidence has been well-documented in certain areas and an abnormal gene has been identified. Sporadic cases are the most common. In contrast to Uhl's anomaly, characterized pathologically by areas of paper thin myocardium, the right ventricular free wall is minimally decreased in thickness. Histologically there appears to be a replacement of musculature by fatty tissue. Medical therapy with sotalol or amiodarone, or combination therapy (Class Ic drugs plus beta-blocking drugs, or amiodarone plus beta-blocking drugs) is frequently effective in preventing recurrent ventricular tachycardia. Ablation using radiofrequency (RF) or direct current (DC) energy is reserved for patients who are unresponsive or intolerant of antiarrhythmic drugs. Ventricular arrhythmia recurrence of different morphology is not uncommon after apparent successful ablation. There appears to be a lower rate of successful ablation using RF energy. However, patients with this condition who have been resuscitated from sudden cardiac death or those refractory to medical treatment are candidates for ablation, implantation of an automatic cardioverter defibrillator, or cardiac transplantation. Surgery consisting of total disconnection of the right ventricle is a promising therapeutic modality.     

Demonstration of Entrainment and Presence of Slow Conduction During Ventricular Tachycardia in Arrhythmogenic Right Ventricular Dysplasia

During VT in two cases with arrhythmogenic right ventricular dysplasia, entrainment criteria, constant fusion beats except for the last entrainment beat, progressive fusion, and a localized conduction block associated with interruption of VT, were demon strated with rapid ventricular pacing performed during VT. Furthermore, a long conduction interval was present during entrainment from the pacing site to the earliest activation site during VT. indicating the presence of a slow conduction area. VT in these cases was, thus, due to reentry with an area of slow conduction within the circuit.     

Catheter Ablation of Ventricular Tachycardia in Patients with Right Ventricular Dysplasia: Identification of Target Sites by Entrainment Mapping Techniques

Objective: To identify target sites for radiofrequency ablation of ventricular tachycardia (VT) by entrainment mapping techniques in patients with arrhythmogenic right ventricular dysplasia. Methods: Entrainment mapping and radiofrequency ablation of eight VTs was performed in seven patients. Radiofrequency ablation was applied at 31 reentry circuits sites that were classified based on findings during entrainment. Results: By entrainment criteria the 31 sites were classified as: exit sites (n = 12), proximal sites (n = 6), and outer loop sites (n = 13). Radiofrequency current application terminated VT at 7 of 31 sites: 2 of 12 exit sites (17%), 4 of 6 proximal sites (67%), and 1 of 13 outer loop sites (8%). Conclusion: Radiofrequency ablation terminated VTs most often at sites proximal to the exit as opposed to outer loop sites and exit sites (P = 0.05). The critical isthmus for ablation of VT in right ventricular dysplasia often may be distant to the exit.     

定量组织速度成像和组织追踪法对扩张型心肌病左室功能的分析

目的 探讨定量组织速度成像（QTVI）和组织追踪法（TT）对扩张型心肌病（DCM）患左室收缩和舒张功能的价值。方法 获取标准心尖位左室长轴切面，两腔切面及四项切面，分别应用QTVI和TT技术分析30例正常人和20例DCM患左室长轴方向不同室壁节段即左室前壁、后壁、侧壁、下壁、前间隔和后间隔的心肌多普勒速度曲线和位移曲线；用M-型超声心动图测量收缩期二尖瓣环下移距离（Don）；用二维超声心动图测量左室射血分数（LVEF）：用多普勒超声心动图测量二尖瓣口血流快速充盈速度E峰、左房收缩充盈速度A峰，计算E/A值。结果 DCM患左室不同室壁节段Vs，Ve，Va和Ds以及LVEF均比正常人显减低。在DCM患中，用定量组织速度成像和组织追踪法测量的二尖瓣环水平Ds与用M-型超声心动图测量的Dm显相关（r=0.64，P=0.005），二尖瓣环水平的平均Vs(r=0.73，P=0.001）、平均Ds(r=0.64,P=0.005）与LVEF分别显相关。正常人与DCM患两组间E/A值无显统计学差异，而DCM患二尖瓣环平均Ve/Va较正常人显减低；正常人中二尖瓣环平均Ve/Va与E/A显相关（r=0.63,P=0.008)，而DCM患二尖瓣环平均Va与E/A无显相关。结论 定量组织速度成像和组织追踪法技术可快速、直观、元创性定量评价扩张型心肌病患左心室收缩舒张功能。     

定量组织速度成像和组织追踪对正常人左室壁运动的研究

目的应用定量组织多普勒速度成像(quantitative tissue velocity imaging,QTVI)和组织追踪法(tissue tracking,TT)分析正常人左室心肌长轴方向舒缩运动的特点,探讨QTVI和TT技术评价心肌运动的应用价值.方法获取标准心尖位左室长轴切面、两腔切面及四腔切面,分别应用QTVI和TT技术,对30例正常人左室各节段长轴方向舒缩运动的特点进行测定分析.结果正常人每个心动周期中,室壁运动速度曲线均包括收缩波(S)、快速充盈波(E)和左房收缩充盈波(A)三个主要运动波,以及等容舒张波(IR)和等容收缩波(IC).正常人心肌不同节段收缩期和舒张期运动速度呈一梯度.同一室壁从心底向心尖部,运动速度和位移逐渐降低.同一水平不同节段心肌运动速度和位移也有差别.结论正常人室壁运动存在特有的不均一性,QTVI和TT对评价局部心肌运动具有很好的应用价值,进而为诊断冠心病开辟一种新途径.     

Epsilon Wave in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Background: Epsilon wave is a major criteria for arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C). We sought to study systematically characteristics of Epsilon wave in ARVD/C patients from the southern Chinese population.
Methods: The population included 49 patients with ARVD/C meeting the diagnostic criteria. They were analyzed for the value of different electrocardiogram (ECG) criteria including Epsilon wave, the ones of 24-hour Holter recording, signal-averaged ECG, and echocardiography to learn the correlation between Epsilon wave and other variability with the use of nonparametric test. A probability value of ≤0.05 was considered significantly different.
Results: It shown that the detection rate of Epsilon wave was significantly higher in probands (65%) than involved family members (22%), P = 0.03. In the conventional as well as Fontaine leads, its detection rate were 18 (37%) and 28 (57%), respectively. The prevalence of diffuse right ventricle involvement, T-wave inversion and signal-averaged ECG are significantly different between the ARVD/C patients with Epsilon wave and without Epsilon wave.
Conclusion: It is significantly correlated between Epsilon wave and the progressive ARVD/C.     

Atrial Epicardial Pacing with Long Stimulus to P Wave Interval in a Patient with Arrhythmogenic Right Ventricular Dysplasia Complicated by Right Atrial Thrombosis

Atrial epicardial pacing with a long stimulus to P wave interval in a patient with arrhythmogenic right ventricular dysplasia complicated by right atrial thrombosis is discussed. Arrhythmogenic right ventricular dysplasia (ARVD) is associated with a high incidence of malignant ventricular arrhythmias. Most patients with ARVD need antiarrhythmic drugs, catheter ablation, or an implantable cardioverter defibrillator. We report a patient with ARVD in whom effective treatment with sotalol caused severe, symptomatic sinus bradycardia requiring permanent pacing. Due to leftward displacement of the right ventricle and the presence of two thrombi in the right atrium, an epicardial atrial lead and AAI pacemaker were implanted. A long stimulus to P wave interval caused by severe dilatation of the right atrium was recorded. During a 6 months of follow-up on sotalol treatment there were neither ventricular tachycardia (VT) attacks nor pacing problems.