摘要目的研究致心律失常性右室心肌病的特点。 方法本文对7例致心律失常性右室心肌病(ARVC)及其3个家系中的34个成员进行调查。所有病人和家族成员进行了病史调查、临床检查、心电图、超声心动图和晚电位检查。5例ARVC行电生理检查。 结果所有调查的病人和家族成员超声心动图示左室结构正常。14例有异常临床表现者诊断为ARVC。 其中5例右室扩大伴弥漫性搏动减弱；8例右室心尖区变薄，搏动减弱伴收缩期膨出；l例右室下壁变薄，搏动减弱伴收缩期膨出。25例心电图异常(7例病人，18例成员)。13例晚电位阳性(6例病人)。5例病 人电生理检查诱发出2-3种左束支阻滞室性心动过速。2例病人在电生理检查时诱发室颤。5例病人右 室一个或多个部位起搏阈值明显增高或起搏无效。一家系中2个成员猝死。5例病人记录到自发的左束 支阻滞室性心动过速。一例ARVC同时伴有侏儒症。一例病人有联律间期极短的多形性室速。12例(6 例病人)有左束支阻滞室性早搏。 结论本研究结果提示ARVC是一遗传性疾病，家系调查有助于诊断和发现新病例，右室结构和功能的异 常、心电图复极异常和起源于右室的室性心律失常是其最常见的表现。

abstractsObjective To explore the characteristics of arrhythmogenic rightventricular cardiomyopathy (ARVC). Methods Seven patients with arrhythmogenic right ventricular cardiomyopathy and 34 members of three families were studied. All patients and family members underwent history collection, clinical examination, electrocardiogram (ECG), two-dimensional echocardiography (2-DE) and a signal averaging electrocardiogram. Programmed ventricular stimulation was performed in five patients. Results All patients and family members had normal morphologic characteristics and normal function of the left ventricular by 2-DE. Fourteen persons had abnormal findings indicating ARVC. Five had enlargement of the right ventricular with diffused hypocontractility, eight had thin and systolic bulging in the focal anterior wall with hypokinesia and one had bulging of the inferior wall. Twenty-five persons (seven patients and 18 family members) had abnormal findings in ECG. Positive ventricular late potential was recorded in 13 persons (six patients). Two to three monomorphic ventricular tachycardia (VT) with left bundle branch block (LBBB) configurations were induced in five patients. Ventricular fibrillation was induced in two patients during the electrophysiologic study (EPS). Five patients had very high pacing threshold and/or ineffective pacing in one or many regions of the right ventricle. Two members of one family died suddenly. One member was a dwarf with ARVC. Spontaneous VT with a left bundle branch block (LBBB) configuration was recorded in five patients, polymorphic VT with extremely short coupling interval in one, and premature ventricular complexes with LBBB configuration in 12 (six patients). Conclusion Our familial study strongly suggests that ARVC may be a hereditary disease and it is helpful in the diagnosis and detection of ARVC. The most common manifestations were abnormal structure and function of the right ventricle and abnormal ECG of repolarization and ventricular arrhythmia which originates from the right ventricle.