致心律失常性右室发育不良室性心动过速的电生理检…

报告药物治疗无效、射频导管消融失败的致心律失常性右室发育不良（ＡＲＶＤ）顽固性室性心动过速（简称室速）１例患者，在电生理导引下行右室前游离壁隔离术治疗成功。术后随访７个月无室速发作，右室收缩功能正常。提示部分右室隔离术对有生命危险和药物治疗无效及射频导管消融失败的ＡＶＲＤ室速患者是安全、有效的。     

致心律失常性右室发育不良室性心动过速的电生理检查和外科治疗（附一例报告）

报告药物治疗无效、射频导管消融失败的致心律失常性右室发育不良（ＡＲＶＤ）顽固性室性心动过速（简称室速）１例患者，在电生理导引下行右室前游离壁隔离术治疗成功。术后随访７个月无室速发作，左室收缩功能正常。提示部分右室隔离术对有生命危险和药物治疗无效及射频导管消融失败的ＡＲＶＤ室速患者是安全、有效的     

致心律失常性右室发育不良症的室性心动过速的低能量电消融治疗

本文报告4例致心律失常性右室发育不良患者,因为反复出现室性心动过速药物治疗无效,而经心内膜起搏标测,在右室心尖部(3例)与右室流出道(1例)找出室性心动过速起源灶,用70～100J直流电进行经导管电消融。术中无心包填塞及肺水肿等并发症。术后不再能诱发出临床型室性心动过速。随访半年,其中2例无心律失常出现,另2例分别在1周与3个月后复发。     

致心律失常性右室心肌病的临床与电生理学特征

目的与方法 致心律失常性右室心肌病（ARVC）可引起严重心律失常，本文对6例ARVC患者进行了临床和电生理学特点观察和研究。结果 6例患者有室性心动过速（VT）的5例，多形性VT2例。所有患者均表现右心室（RV）扩大，可见到RV局部病变的2例，均没有见到左心室受累和明显的心功能受损。接受电生理学检查的4例患者中，有3例可以由电刺激诱发和终止，电生理标测2例源自RV心尖部，2例源自RV游离壁，与超声提示的RV病变部位有关。3例患者发生过阿斯综合征，其中有2例发生院外猝死，2例猝死病人均发作过多形性VT。结论 ARVC以RV进行性病变伴室性心律失常为特征。射频消融（RFCA）和ICD植入治疗、药物治疗是基本治疗手段，药物治疗以胺碘达隆加β－受体阻滞剂或索他洛尔(Sotalol)为多选，但是单纯的药物治疗并不能防止病人发生心脏性猝死。对于有条件的病人应该考虑进行RFCA或ICD治疗。     

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。     

QT离散度对致心律失常性右室发育不良的临床意义

ＡＲＶＤ患者因右心室出现异常是进行性的 ,发生室性心律失常甚至猝死的危险性很大 ,且每位患者都具有不可预测性。本研究用ＱＴｄ作为一项判断和预测ＡＲＶＤ患者发生室性心律失常和猝死可能性的一项指标来探讨其临床意义。1　资料与方法自 1990年～ 2 0 0 0年 ,11例ＡＲＶＤ患者均为住院患者 ,其中男 6例 ,女 5例 ,年龄 16～ 5 4(3 6± 6)岁 ,2例分布在同一家系 ,其余为散发患者。ＡＲＶＤ组再分为 2个亚组 :Ⅰ组 (6人 )由从未发生过持续性室性心律失常及心脏骤停的低危患者组成。Ⅱ组 (5人 )由曾发生过持续性室性心律失常及心脏骤停的高…     

刻不容缓:致心律失常性右室心肌病/发育不良的早期诊断与治疗

致心律失常性右室心肌病/发育不良是一种以纤维-脂肪组织进行性替代右室心肌细胞为特征的遗传性心肌疾病,是引起青少年心源性猝死的主要原因之一。欧洲心脏病协会颁布了最新的诊断标准,使它诊断的敏感性及特异性显著的提高。但心律失常性右室心肌病/发育不良的早期诊断与治疗对于心内科医生来说仍然是一个巨大的挑战。现综述近年来在新标准的基础上对早期诊断以及治疗心律失常性右室心肌病/发育不良的新进展。     

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/发育不良

致心律失常性右室心肌病 (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方法上有很大进展。一、概况本病 196 1年由Ｄａｌｌａｖｏｌｔａ首次报道 ,1978年由ＦｒａｎｋＦｏｎｔａｉｎｅ正式命名为致心律失常性右室发育不良 (ＡＲＶＤ) ,当时认为本病组织学上的特征是右室游离壁心肌组织部分或全部被脂肪组织替代 ,而左室正常 ,随着日益深入的病理学和临床研究发现 ,ＡＲＶＤ病人病变组织内还可见进展性间质纤维化及炎性细…     

致心律失常性右室发育不良／右室心肌病的诊断及研究进展

致心律失常性右室发育不良／或右室心肌病（ARVD／C）近20年始被认识，由Marcus于1982年首次报道。当时报道以男性为主，特征性为起源于右心室的，呈左束支传导阻滞（LBBB）图形的室性心动过速（VT）。同时有右心室扩大，右室游离壁被纤维脂肪细胞浸润，伴有明显的家族史。现综述如下。     

Ablation of Ventricular Arrhythmias in Arrhythmogenic Right Ventricular Dysplasia

VT Ablation in Right Ventricular Dysplasia. Arrhythmogenic right ventricular dysplasia (ARVD) is a genetically determined myocardial disease characterized by fibrofatty replacement of the right ventricular wall. Ventricular tachyarrhythmias can be seen in the early stages of the disease, which is one of the most important causes of sudden death in young healthy individuals. Radiofrequency (RF) catheter ablation is an option for the treatment of medically refractory ventricular arrhythmias and it has shown to successfully abolish recurrent ventricular tachycardias (VT) as well as reduce the frequency in defibrillator therapies. However, variable acute and long‐term success rates have been reported. The current mapping and ablation techniques include activation and entrainment mapping during tolerated VT and substrate ablation using 3‐dimensional electroanatomic mapping systems. This article aims at providing a comprehensive review of RF catheter ablation of ventricular arrhythmias in the context of ARVD. (J Cardiovasc Electrophysiol, Vol. 21, pp. 473‐14, April 2010)     

The Use of Fontaine Leads in the Diagnosis of Arrhythmogenic Right Ventricular Dysplasia

We report a case of a 68‐year‐old man admitted to the emergency department with syncope preceded by rapid palpitations. His admission ECG demonstrated a sustained ventricular tachycardia (VT) originating from the right ventricular outflow tract (RVOT). This report highlights the importance of distinguishing ventricular tachycardia caused by arrhythmogenic right ventricular dysplasia (ARVD) from the more benign idiopathic RVOT‐VT. Furthermore, we demonstrate the utility of the Fontaine leads placement in increasing the sensitivity for uncovering epsilon waves, a highly specific electrocardiographic feature that increases diagnostic accuracy in patients with ARVD.     

Usefulness of Biplanar Transesophageal Echocardiography in Arrhythmogenic Right Ventricular Dysplasia

This study evaluated the clinical usefulness of biplanar transesophageal echocardiography (TEE) in suspected arrhythmogenic right ventricular dysplasia (ARVD). Seven symptomatic subjects (3 male and 4 female: ages 18–64 years, mean 45) with clinical features of ARVD (typical ventricular arrhythmias) underwent comprehensive noninvasive assessment including transthoracic echocardiography (TTE), nuclear magnetic resonance (NMR), and TEE. Other systemic and cardiac diseases were reasonably excluded. TEE identified a significant right ventricular regional alteration in one subject with negative TTE and NMR, and nonsignificant abnormalities in two subjects with negative or no TTE and positive NMR. TEE confirmed the pathological findings detected by TTE in the four remaining patients and visualized several more abnormalities that approximately corresponded to NMR results. On the basis of these initial results, biplanar TEE appears to be comparable to NMR in the assessment of ARVD and a promising technique in identifying ARVD morphological alterations.     

Non-invasive Recognition of Arrhythmogenic Right Ventricular Dysplasia

ABSTRACT Arrhythmogenic right ventricular dysplasia causes ventricular arrhythmias and sometimes heart failure. The condition is easily overlooked, but once suspected, it may be diagnosed non-invasively. This is illustrated by the case reported. The clinical features of this syndrome are discussed, with special emphasis on the non-invasive findings.     

Arrhythmogenic Right Ventricular Dysplasia: Clinical Results with Implantable Cardioverter Defibrillators

Arrhythmogenic right ventricular dysplasia is a clinical entitycharacterized by fatty infiltration of the right ventricle and left bundlemorphology ventricular tachycardia occurring in young patients. The mostcommon cause of death is tachyarrhythmic. Pharmacological andnonpharmacological therapies, including implantable cardioverterdefibrillators, have been used to treat the arrhythmias. However, rightventricular endocardial leads in this population may be associated with anincreased risk of perforation and suboptimal sensing and defibrillationefficacy due to the diseased right ventricle. We report on 12 patients witharrhythmogenic right ventricular dysplasia who were treated with implantablecardioverter defibrillators. The mean age was 31± 9 years (range15-48). Patients presented with presyncope (5), syncope (4), or cardiacarrest (3). All patients had electrocardiographic abnormalitiescharacteristic of the condition.Follow-up averaged 22 ± 13months (range 1-45). There was one sudden death at 1 month of follow-up. Ofthe 12 patients, 8 have had appropriate therapy delivered by the implantabledefibrillator. Six patients are currently on sotalol to reduce the frequencyof implantable defibrillator discharges. In conclusion, implantablecardioverter defibrillators with nonthoracotomy leads are feasible and safein patients with arrhythmogenic right ventricular dysplasia. The frequencyof appropriate therapy is high, supporting the use of implantablecardioverter defibrillators in this population.During programmedelectrical stimulation nine patients had sustained ventricular tachycardia,while three patients had no inducible arrhythmia. Transvenous leads wereplaced in nine patients. In these patients pacing thresholds weresignificantly higher, R-wave amplitudes were significantly lower, anddefibrillation thresholds were not significantly different than in a cohortof patients without right ventricular dysplasia. There were no acute orchronic complications of right ventricular lead placement.