家族史在致心律失常性右心室心肌病危险分层中的地位

目的 探讨家族史在致心律失常性右心室心肌病(ARVC)危险分层中的地位.方法 根据1994年ARVC诊断标准,纳入34例ARVC先证者,男性26例,女性8例,平均年龄(38±15)岁.对其家族成员行临床筛查,项目包括:(1)心电图V1～V3导联QRS≥110 ms、V1～V3导联S波升支≥55 ms、Epsilon波、T波倒置(V1～V3导联倒置)、(V1+V2+V3)/(V4+V5+V6)QRS≥1.2、V1～V3导联与V6导联QRS差值≥25 ms,QRS离散度≥40 ms,QT离散度≥65 ms;(2)动态心电图记录室性早搏≥2000个/24 h或室性心动过速(VT);(3)超声心动图记录双心房、双心室及右心室流出道、流人道内径大小.比较ARVC家族史和上述各项临床参数的关系.分类变量用Fisher检验,连续变量使用t检验.P≤0.05为差异有统计学意义.结果 34例ARVC先证者中55个家族成员接受评估,男性28例(6例诊断ARVC)、女性27例(3例诊断ARVC),平均年龄(35±16)岁.8例先证者有家庭成员受累,其中5例有左束支阻滞形室性心动过速(LBBB-VT,63%);26例先证者家庭成员无受累,其中20例有LBBB-VT(77%),P=0.649.家族史和室性心动过速的发生筹异无统计学意义.结论 家族史并不能反映ARVC的危险程度.     

致心律失常性右室心肌病

本文就致心律失常性右室心肌病的遗传因素、形态学特征、病因、心电图（ECG）及临床表现、诊断、治疗等方面做一综述。     

致心律失常性右室心肌病的研究进展

致心律失常性右室心肌病（ARVC）,又称致心律失常性右室发育不良／心肌病（ARVD／C）为遗传性原发性心肌疾病,呈常染色体显性遗传,为运动猝死中常见的病因,占年轻猝死病的20％,大多数病例死亡时的年龄〈40岁,有些发生于儿童。ARVC的病理特征为右心室内的心肌萎缩和纤维脂肪组织替代。以左束支阻滞图形的单形性室性心动过速为特征的、具有多种临床表型的心肌病。以右心室受累为主,晚期可累及左室。尽管心力衰竭是疾病晚期的重要并发症,但ARVD／C主要表现为室性心律失常和心脏性猝死（SCD）。     

致心律失常性右室心肌病1例

患者男，63岁，因反复心悸、室性心动过速20余年，加重1个月于2002年11月20日入院。患者既往曾因上述症状在我院及多家医院住院治疗，曾诊断为“冠心病、陈旧性正后壁心梗，阵发性室速、室上速，预激综合征”等。本次住院后曾发作一次短阵室速，治疗后缓解。家族史：其两个弟弟多年前均不明原因猝死。     

基因型在致心律失常性右心室心肌病危险分层中作用的研究进展

致心律失常性右心室心肌病（ARVC）患者可发生恶性心律失常事件,如室性心律失常、心脏猝死等,对其进行危险分层有助于识别出高危患者并即时采取恰当的干预措施。目前已发现多种基因突变与ARVC相关,而不同基因突变所致的ARVC临床特点不同,我们有望利用基因型对ARVC患者进行危险分层。该文对基因型在ARVC危险分层中作用的研...     

致心律失常性右室心肌病一例

患者女,38岁,因"反复心悸1年"入院。心悸发作时ECG示室性心动过速。否认家族猝死史。体格检查未见异常。ECG可见V2导联延迟向上的S波,心脏彩超示右室流出道增宽,心脏核磁共振示右室壁内脂肪信号影,符合致心律失常性右室心肌病。由于经济原因,未植入埋藏式心脏转复除颤器,口服胺碘酮治疗。随访半年,未再有室性心律失常发作。     

致心律失常性右室心肌病高危患者相关危险因素分析

目的探讨致心律失常性右室心肌病(ARVD/C)高危患者相关危险因素。方法根据1994年ARVD/C诊断标准,纳入43例ARVD/C先证者。分组标准:有晕厥病史并记录到室性心动过速(简称室速)为高危病人;记录到室性早搏(简称室早)、室速但无晕厥病史及其他临床情况定为低危病人。收集参数包括:①心电图V1～3QRS波时限≥110 ms、V1～3导联S波升支时限≥55 ms、Epsilon波、T波倒置、(V1+V2+V3)/(V4+V5+V6)QRS波时限≥1.2、QRS波离散度≥40 ms、QT离散度≥65 ms;②信号平均心电图记录晚电位参数;③Holter记录室早或室速;④超声记录双房、双室及右室流出道、流入道内径大小。Logistic回归分析高危患者ARVD/C病人的相关危险因素。结果心室晚电位阳性、右室射血分数<0.40与高危ARVD/C显著相关。结论晚电位阳性、右心功能不全是ARVD/C的高危因素。     

致心律失常性右室心肌病2例并文献复习

正病例1患者男,46岁,2014年6月23日因"反复胸闷心慌1月,晕厥1次"入院。患者6月初因胸闷心慌于当地医院住院治疗,期间发生晕厥1次,行心电图提示"室性心动过速"(简称室速),见图1。行电复律后意识恢复,冠状动脉造影检查未见明显异常,经药物治疗后患者仍间断胸闷,遂至我院。入院查体:HR 78次/min、BP 103/63 mmH g。心电图示交界性心律、偶发     

致心律失常性右室心肌病的研究进展

致心律失常性右室心肌病或致心律失常性右室发育不良/心肌病是一种主要累及右室的心肌疾病,表现为室性心动过速和猝死。新近研究证明这种疾病不是一种少见疾病,患病率约为1/1000,50%~80%有家族史,是一种常染色体遗传性疾病。1994年的诊断标准导致诊断率较低。目前对诊断指标进行了修改,如胸前导联QRS时限延长。通过改进ECG记录方式可以发现更多的epsilon波。应用新的诊断标准将发现更多的有症状和无症状致心律失常性右室心肌病的患者。基因筛查特别是plakophil-in-2突变筛查将成为疾病重要的早期诊断工具。致心律失常性右室心肌病的治疗应在改变生活方式(包括限制参加竞技运动)的基础上,根据病情应用β阻滞剂、胺碘酮、索他洛尔和/或ICD治疗预防猝死。     

致心律失常性右室心肌病新观点

随着对致心律失常性右室心肌病(arrhythmogenic right ventricular cardiomyopathy,ARVC)研究的进展,目前已不再认为ARVC只是桥粒蛋白基因突变引起的一种累及右室的遗传性心肌病.ARVC可以由非桥粒蛋白的多种基因突变或非遗传因素引起,并且可以首先累及左室.因此,2019年制定ARVC诊断标准的国际专家组提出了该病的新的临床分型,并对2010年的诊断标准作出了新的评价.本文就ARVC的临床分型、致病基因、诊断及治疗研究进展等方面进行综述.     

Arrhythmogenic Right Ventricular Cardiomyopathy: Risk Stratification and Indications for Defibrillator Therapy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined disease which predisposes to life-threatening ventricular arrhythmias. The main goal of ARVC therapy is prevention of sudden cardiac death (SCD). Implantable cardioverter defibrillator (ICD) is the most effective therapy for interruption of potentially lethal ventricular tachyarrhythmias. Despite its life-saving potential, ICD implantation is associated with a high rate of complications and significant impact on quality of life. Accurate risk stratification is needed to identify individuals who most benefit from the therapy. While there is general agreement that patients with a history of cardiac arrest or hemodynamically unstable ventricular tachycardia are at high risk of SCD and needs an ICD, indications for primary prevention remain a matter of debate. The article reviews the available scientific evidence and guidelines that may help to stratify the arrhythmic risk of ARVC patients and guide ICD implantation. Other therapeutic strategies, either alternative or additional to ICD, will be also addressed.     

Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of sudden cardiac death and ventricular tachyarrhythmias in young, apparently healthy individuals and athletes. Myocardial atrophy with subsequent fibrofatty replacement predominantly affects right ventricular myocardium and results in global and regional dysfunction as well as areas of slow conduction and dispersion of refractoriness which are prerequisites for reentrant ventricular tachyarrhythmias.Patients affected with ARVC should be excluded from competitive sports and vigorous training. To provide optimal treatment, a detailed diagnostic evaluation and risk stratification are mandatory. Tailored treatment strategies aim at the suppression or effective termination of recurrent ventricular tachyarrhythmias and prevention of sudden death by antiarrhythmic drug therapy, catheter ablation, or implantation of a cardioverter defibrillator (ICD).Antiarrhythmic drugs may be used as a stand-alone treatment to suppress ventricular tachycardia (VT) recurrences in patients with ARVC and low risk of sudden death. Sotalol (preferred) or amiodarone in combination with -blockers showed the highest efficacy rates. In patients at higher risk, an ICD should be implanted and antiarrhythmic drugs be used only as an adjunct to prevent or suppress frequent VT recurrences and ICD discharges.Catheter ablation using conventional or electroanatomic mapping techniques yields good acute results for eliminating the targeted arrhythmia substrate. However, during the progressive long-term course of ARVC, VT recurrences from new arrhythmia foci are frequent and therefore limit the curative value of catheter ablation. In patients with frequent VT recurrences and ICD discharges, however, catheter ablation plays an important role as a palliative and adjunctive treatment option for arrhythmia suppression.ICD therapy has been increasingly used for secondary and also primary prevention of sudden death in patients with ARVC. In secondary prevention, the ICD has shown to improve the long-term prognosis of patients at high risk of sudden death by effective termination of life-threatening recurrences of ventricular tachyarrhythmias. However, adequate lead placement may be difficult and lead-related complications during long-term follow-up must be taken into account. The role of ICD therapy for primary prevention of sudden death in ARVC is not yet adequately defined.Ongoing international registries will provide important additional data to improve risk stratification and refine treatment algorithms in order to select the best individual treatment for arrhythmia suppression and prevention of sudden death in patients with ARVC.     

致心律不齐性右室心肌病

致心律不齐性右室心肌病 (arrhythmogenicrightventricular cardiomyopathy;ARVC)以往也叫右心室发育不良 (right ventricular dysplasia;RVD) ,特点是右室心肌进行性地为纤维脂肪所取代 ,临床常有心律不齐或猝死。 1 995年世界卫生组织 /国际心肌病学协会 (WHO/ISFC)在修订心肌病分类时 ,将 ARVC增列为与扩张性、肥厚性、限制性心肌病并列的第四类心肌病[1] 。ARVC以往长期被视为一种罕见的发育异常或先天畸形 ,80年代以前的国内外心血管病专著中很少提及。后来由于来自法国、意大利、加拿大、美国的病例报道增多 ,特别是由于它和…     

肥厚型心肌病的危险分层:新的猝死预测指标

肥厚型心肌病是一种以心室肌肥厚为特征的遗传异质性疾病,人群患病率为1/500[1]。肥厚型心肌病患者临床表现和自然病史各异,猝死为其最严重的并发症,甚至可以是一些患者的首发症状。研究表明,肥厚型心肌病患者年猝死发生率≤1%[2-3],好发于无症