Francis Johnson, Fontaine Guy
Department of Cardiology, Medical College, Calicut, Kerala, India.
Indian Pacing Electrophysiol J. 2005 Apr 1;5(2):81-5.
Arrhythmogenic right ventricular dysplasia/cardiomyopathy is a disorder characterized by frequent ventricular tachycardia originating from the right ventricle and fibro-fatty replacement of right ventricular myocardium. Though the disorder was originally described during surgical ablation of refractory ventricular tachycardia, catheter ablation of tachycardia is one of the options for patients not responding to anti arrhythmic agents. Direct current fulguration was used in the initial phase followed by radiofrequency catheter ablation. In the present day scenario, all patients with risk for sudden cardiac death should receive an implantable cardioverter defibrillator. Radiofrequency catheter ablation remarkably reduces the frequency of defibrillator therapies. Direct current fulguration can still be considered in cases when radiofrequency ablation fails, though it requires higher expertise, general anesthesia and carries a higher morbidity. Newer mapping techniques have helped in identification of the site of ablation. In general, the success rate of ablation in arrhythmogenic right ventricular dysplasia is less than in other forms of right ventricular tachycardias like right ventricular outflow tract tachycardia.
致心律失常性右室发育不良/心肌病是一种以起源于右心室的频发室性心动过速和右心室心肌纤维脂肪替代为特征的疾病。尽管该疾病最初是在难治性室性心动过速的手术消融过程中被描述的,但对于对抗心律失常药物无反应的患者,心动过速的导管消融是一种选择。最初阶段使用直流电电灼术,随后是射频导管消融。在当前情况下,所有有心脏性猝死风险的患者都应接受植入式心脏复律除颤器。射频导管消融显著降低了除颤器治疗的频率。当射频消融失败时,尽管直流电电灼术需要更高的专业技能、全身麻醉且发病率较高,但仍可考虑使用。更新的标测技术有助于确定消融部位。一般来说,致心律失常性右室发育不良的消融成功率低于其他形式的右室性心动过速,如右室流出道心动过速。