Bar Dayan Y, Amital H, Eldar M
Medical Dept. B, Chaim Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University.
Harefuah. 1995 Dec 1;129(11):464-7, 535.
Arrhythmogenic right ventricular dysplasia is a cardiomyopathy with autopsy evidence of fibrous and fatty infiltration of the right ventricular. The disease, which shows familial clustering, causes electrical instability that may place affected subjects at risk of sudden death. It is characterized by ECG changes and right ventricular dysfunction. Death can occur due to ventricular arrhythmia. We describe a 46-year-old female Cypriot who presented with recurrent syncope and palpitation due to ventricular tachycardia with the LBBB pattern; T waves were inverted in V1-V3. Echocardiogram and cardiac catheterization revealed severe right ventricular dysfunction. Her sister had died several years before of a similar syndrome, including ventricular tachycardia. Family members should be screened for right ventricular dysplasia. Those currently asymptomatic should be given prophylactic therapy to prevent sudden death. Treatment is either medical: sotalol, beta-blockers or verapamil; or surgical: ablation of the arrhythmogenic focus in the right ventricle, total disconnection of the right ventricular free wall; or implantation of a cardioverter defibrillator.
致心律失常性右室心肌病是一种心肌病，尸检证据显示右心室存在纤维和脂肪浸润。该疾病具有家族聚集性，会导致电不稳定，使受影响的个体面临猝死风险。其特征为心电图改变和右心室功能障碍。死亡可能因室性心律失常而发生。我们描述了一名46岁的塞浦路斯女性，因左束支传导阻滞型室性心动过速出现反复晕厥和心悸；V1-V3导联T波倒置。超声心动图和心导管检查显示严重的右心室功能障碍。她的姐姐几年前死于类似综合征，包括室性心动过速。家庭成员应接受右室心肌病筛查。目前无症状者应给予预防性治疗以预防猝死。治疗方法包括药物治疗：索他洛尔、β受体阻滞剂或维拉帕米；或手术治疗：右心室致心律失常灶消融、右心室游离壁完全离断；或植入心脏复律除颤器。
