Martini B, Nava A, Thiene G, Buja G, Canciani B, Miraglia G, Scognamiglio R, Daliento L, Dalla Volta S
Department of Cardiology, University of Padua, Italy.
Int J Cardiol. 1990 May;27(2):211-21. doi: 10.1016/0167-5273(90)90162-x.
We studied in detail 17 patients presenting with monomorphic repetitive ventricular rhythms having left bundle branch block morphology and right axis deviation. All had an apparently normal heart at physical examination. At chest radiography, three patients had mild cardiomegaly, and at electrocardiography, five patients had inverted T waves beyond V2. Five patients had syncope or near syncope. In seven patients the tachycardia occurred on effort. One patient died suddenly. The patients were extensively investigated, using cross-sectional echocardiography, complete haemodynamic and angiographic studies, electrophysiology and histology, to search for any structural basis of the arrhythmias. Tachycardia was sustained in 8 patients, nonsustained in 3, and consistent with accelerated idioventricular rhythm and repetitive paroxysmal ventricular tachycardia in 5 and 1 patients, respectively. Despite the differences in clinical and arrhythmologic features, similar abnormalities of right ventricular structure and/or wall motion were detected in all patients, consistent with localized forms of right ventricular cardiomyopathy. Different antiarrhythmic drugs were successfully used in twelve patients (the four patients with accelerated idioventricular rhythm were not treated). The patient who died suddenly had previously had a sustained ventricular tachycardia and was being treated by beta-blockade. Postmortem study revealed massive fibro-adipose substitution of the right ventricular free wall and pulmonary infundibulum.
我们详细研究了17例呈现单形性重复性室性节律、伴有左束支传导阻滞形态及电轴右偏的患者。所有患者体格检查时心脏外观均正常。胸部X线检查中，3例有轻度心脏扩大；心电图检查时，5例在V2导联之后出现T波倒置。5例患者有晕厥或接近晕厥症状。7例患者的心动过速在运动时发作。1例患者突然死亡。对这些患者进行了广泛检查，采用了横断面超声心动图、全面的血流动力学和血管造影研究、电生理学及组织学检查，以寻找心律失常的任何结构基础。心动过速持续存在的有8例，非持续性的有3例，分别有5例和1例符合加速性室性自主心律及重复性阵发性室性心动过速。尽管临床和心律失常特征存在差异，但在所有患者中均检测到右心室结构和/或壁运动的类似异常，符合右心室心肌病的局限性形式。12例患者成功使用了不同的抗心律失常药物（4例加速性室性自主心律患者未接受治疗）。突然死亡的患者此前有持续性室性心动过速，正在接受β受体阻滞剂治疗。尸检研究显示右心室游离壁和肺动脉漏斗部有大量纤维脂肪替代。
