Hoch D H, Rosenfeld L E
Section of Cardiovascular Medicine, Yale University School of Medicine, New Haven, Connecticut.
Cardiol Clin. 1992 Feb;10(1):151-64.
Ventricular tachycardia arising from the right ventricle usually has a left bundle branch morphology and occurs in a variety of disorders. Uhl's anomaly and right ventricular dysplasia may represent a spectrum of one disorder and are a cause of right heart dilatation, failure, and premature sudden death due to ventricular arrhythmias. Familial forms of the disorder may account for focal clustering in some geographic areas. Management should involve aggressive stratification of arrhythmia risk and may include medical, surgical, or device therapy. In contrast, the syndrome of right ventricular outflow tract tachycardia, including nonischemic exercise-induced and repetitive monomorphic ventricular tachycardia, is a more benign entity. Management often involves beta- and calcium channel blocking drugs or type IC antiarrhythmic drugs. Catheter ablation of the arrhythmia focus in the right ventricular outflow tract has been used in selected patients. In this syndrome the right ventricle is normal, and noninvasive testing as well as electrophysiologic studies can be helpful in distinguishing it from the more malignant right ventricular dysplasia. Ventricular arrhythmias may also be seen after right ventricular incision, as in surgical repair of tetralogy of Fallot and ventricular septal defects. Significant ventricular ectopy associated with an abnormal right ventricle (enlarged or depressed systolic function) is associated with an increased risk for sustained arrhythmia and sudden cardiac death in this group. The optimal indicator(s) of highest risk in these patients remains under investigation but will likely include electrophysiologic testing. Bifascicular block occurs commonly after repair of tetralogy of Fallot, but is usually benign. Isolated right ventricular infarction is rare. Most right ventricular arrhythmias associated with ischemia occur in the setting of iatrogenic catheter manipulation for pacing or hemodynamic monitoring. In conclusion, right ventricular arrhythmias involve an unusual and interesting group of clinical entities and appear to span the spectrum of arrhythmias mechanisms. A macroreentrant activation ring around the ventriculotomy scar may account for the arrhythmias following repair of tetralogy of Fallot. Microreentry at sites of morphologic abnormalities results in the arrhythmias associated with right ventricular dysplasia and ischemia. Triggered activity related to DADs or, less likely, abnormal automaticity, produce repetitive monomorphic ventricular tachycardia and nonischemic exercise-induced ventricular tachycardia, both of which usually originate from the right ventricular outflow tract. Iatrogenic ventricular tachycardia associated with catheter manipulation is especially likely to occur in the presence of right ventricular ischemia and infarction. It is important to recognize these clinical entities because treatment is specific.(ABSTRACT TRUNCATED AT 400 WORDS)
起源于右心室的室性心动过速通常呈左束支形态,可发生于多种疾病。乌尔畸形和右心室发育不良可能代表一种疾病的不同表现形式,是导致右心扩张、衰竭以及因室性心律失常而猝死的原因。该疾病的家族性形式可能导致某些地理区域出现聚集发病情况。治疗应积极对心律失常风险进行分层,可能包括药物、手术或器械治疗。相比之下,右心室流出道心动过速综合征,包括非缺血性运动诱发的和反复单形性室性心动过速,是一种相对良性的疾病。治疗通常涉及使用β受体阻滞剂和钙通道阻滞剂或ⅠC类抗心律失常药物。对于部分患者,可采用导管消融右心室流出道的心律失常病灶。在该综合征中,右心室正常,无创检查以及电生理研究有助于将其与更恶性的右心室发育不良相鉴别。右心室切开术后也可见室性心律失常,如法洛四联症和室间隔缺损的手术修复。与异常右心室(扩大或收缩功能降低)相关的显著室性早搏与该组患者持续性心律失常和心源性猝死风险增加有关。这些患者中最高风险的最佳指标仍在研究中,但可能包括电生理检查。法洛四联症修复术后常出现双分支阻滞,但通常为良性。孤立性右心室梗死罕见。大多数与缺血相关的右心室心律失常发生在因起搏或血流动力学监测进行医源性导管操作的情况下。总之,右心室心律失常涉及一组不寻常且有趣的临床疾病,似乎涵盖了心律失常机制的范围。围绕心室切开术瘢痕的大折返激活环可能是法洛四联症修复术后心律失常的原因。形态学异常部位的微折返导致与右心室发育不良和缺血相关的心律失常。与延迟后除极相关的触发活动或可能性较小的异常自律性产生反复单形性室性心动过速和非缺血性运动诱发的室性心动过速,两者通常起源于右心室流出道。与导管操作相关的医源性室性心动过速在存在右心室缺血和梗死时尤其容易发生。认识这些临床疾病很重要,因为治疗具有针对性。(摘要截选至400字)