Nava A, Canciani B, Daliento L, Miraglia G, Buja G, Fasoli G, Martini B, Scognamiglio R, Thiene G
Department of Cardiology, University of Padua Medical School, Italy.
Int J Cardiol. 1988 Nov;21(2):111-26. doi: 10.1016/0167-5273(88)90212-4.
A family with occurrence of juvenile sudden death and effort polymorphous ventricular tachycardias is reported. Nineteen members aged 9 to 63 years were investigated. Four of them died suddenly in their youth. Postmortem investigation performed in 2 deceased subjects disclosed an apparently normal heart at macroscopy but fibro-fatty substitution of the right ventricular free wall was noted at histologic examination. The 14 living members underwent physical examination, resting electrocardiography, chest X-radiography, Holter monitoring, exercise stress testing, and M-mode and cross-sectional echocardiography. Four patients underwent hemodynamic and electrophysiologic studies. All 14 subjects had normal physical examination as well as normal electrocardiographic and cardiothoracic indices. Localized right ventricular structural and dynamic abnormalities were noted at cross-sectional echocardiographic and angiographic investigation of 9 of the patients. The right ventricular volumes in these subjects were normal or slightly increased. In 7 of them, polymorphous ventricular tachycardias were induced by exercise stress testing. The arrhythmias which were responsive to beta-blockade, do not seem to depend on reentry. Enhanced automaticity appeared to be the more likely mechanism of their production. These data demonstrate that right ventricular cardiomyopathy may occur in an occult form with life-threatening electrical instability.
报道了一个出现青少年猝死和运动诱发多形性室性心动过速的家族。对19名年龄在9至63岁的家族成员进行了调查。其中4人在年轻时突然死亡。对2名死者进行的尸检显示，大体检查心脏外观正常，但组织学检查发现右心室游离壁有纤维脂肪替代。14名在世成员接受了体格检查、静息心电图、胸部X线摄影、动态心电图监测、运动负荷试验以及M型和切面超声心动图检查。4名患者接受了血流动力学和电生理研究。所有14名受试者的体格检查以及心电图和心胸指数均正常。在对9名患者进行的切面超声心动图和血管造影检查中发现了局限性右心室结构和动态异常。这些受试者的右心室容积正常或略有增加。其中7人在运动负荷试验中诱发了多形性室性心动过速。对β受体阻滞剂有反应的心律失常似乎不依赖于折返。增强的自律性似乎是其产生的更可能机制。这些数据表明，右心室心肌病可能以隐匿形式出现，并伴有危及生命的电不稳定。
