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)     

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。     

右心室心肌病室性心动过速诊治的新进展

致心律失常右心室心肌病室性心动过速是青壮年猝死的常见疾病,传统的治疗方法是在药物基础上的植入埋藏式自动复律仪治疗;由于我国的经济欠发达状况,普通患者难以负担植入埋藏式自动复律仪,以及导管消融新设备的开发和消融技术的进步,导管消融技术已经是部分患者的一线治疗。现就致心律失常右心室心肌病诊治新进展进行综述。     

Arrhythmogenic Right Ventricular Cardiomyopathy 2012: Diagnostic Challenges and Treatment

ARVC 2012 . The most common presentation of arrhythmogenic right ventricular cardiomyopathy (ARVC) is palpitations or ventricular tachycardia (VT) of left bundle branch morphology in a young or middle‐aged individual. The 12‐lead electrocardiogram may be normal or have T‐wave inversion beyond V₁ in an otherwise healthy person who is suspected of having ARVC. The most frequent imaging abnormalities are an enlarged right ventricle, decrease in right ventricular (RV) function, and localized wall motion abnormalities. Risk factors for implantable cardioverter defibrillator include a history of aborted sudden death, syncope, young age, decreased left ventricular function, and marked decrease in RV function. Recent results of treatment with epicardial ablation are encouraging. (J Cardiovasc Electrophysiol, Vol. 23 pp. 1149‐1153, October 2012)     

Radiofrequency Catheter Ablation of Ventricular Tachycardia in Right Ventricular Cardiomyopathy:

RF Catheter Ablation of VT. Radiofrequency (RF) catheter ablation of ventricular tachycardia (VT) in patients with a right ventricular (RV) cardiomyopathy has only rarely been successful. This report demonstrates reentrant VT in the setting of RV cardiomyopathy in which the tricuspid valve annulus acted as one of the harriers of an isthmus of slow conduction, identified by the presence of entrainment with concealed fusion. The RF pulse was further targeted by analysis of the relationship between the postpacing interval with the tachycardia cycle length, and of the local activation time with the stimulation time. Long-term clinical follow-up has documented no recurrent VT.     

致心律失常性右室心肌病的诊断和治疗

致心律失常性右室心肌病(ARVC)是一种相对少见的原发性心肌疾病,主要临床表现为起源于右室的室性心律失常。由于疾病的异质性导致推荐的诊断标准有较大的局限性。现对该病的诊断和治疗做一简要综述。     

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。     

Epicardial Macro-Reentrant Ventricular Tachycardia Exhibiting an Endocardial Centrifugal Activation Pattern in a Case with Arrhythmogenic Right Ventricular Cardiomyopathy

A 55-year-old man with arrhythmogenic right ventricular cardiomyopathy underwent catheter ablation of ventricular tachycardia (VT) with left bundle branch block and left superior axis QRS morphology with an early precordial transition. Endocardial mapping during the VT revealed a focal activation pattern from a small region of low voltage in the left ventricular (LV) septum. Despite earliest endocardial activation in the LV septum, epicardial mapping demonstrated a macro-reentrant circuit with successful catheter ablation at an inferior peritricuspid annular site. Activation from the reentrant circuit propagated through the scar area in the epicardial right ventricle to the remote endocardial LV breakout site.     

致心律失常性右心室心肌病的诊治与反思

随着近年来对致心律失常性右心室心肌病认识的提高,临床报道逐年增多,但仍有许多问题需要探讨和研究。现就其诊断及治疗方面的问题进行综述。     

致心律失常性右室发育不良的电生理特性

致心律失常性右室发育不良在临床上主要表现为心律失常、猝死和心力衰竭。心电图上主要表现出(1)复极异常;(2)除极/传导异常;(3)室性心律失常。室性心动过速(室速)时舒张期的碎裂电位和心室病变部位的低电压区可以被看作是其诊断标准。Carto系统标测能发现病变区的准确位置、病变严重程度及病变范围。目前致心律失常性右室发育不良的临床治疗主要有:针对室速发生所采取的射频消融法,针对心力衰竭所采用的药物治疗及心脏移植手术,针对心脏猝死所采取的埋藏式心脏复律除颤器植入等。     

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.