Bettini R, Furlanello F, Vergara G, Durante G, Bertoldi A, Visonà L, Dal Forno P, Camin G, Burelli C, Nicolosi G L
Divisione di Cardiologia e Centro Aritmologico, Ospedale di Trento.
G Ital Cardiol. 1989 Jul;19(7):567-79.
Arrhythmogenic right ventricular disease is a syndrome which involves a wide spectrum of anatomo-clinical features. It is characterised by different levels of right ventricle anomaly, and by life-threatening "right" hyperkinetic ventricular arrhythmias. Fifty consecutive pts were retrospectively examined at the Arrhythmological Centre in Trento between 1977 and 1988. The results of rigorous arrhythmological, echocardiographic and angiographic criteria showed that all pts were affected by arrhythmogenic right ventricular disease.
39/50 (78%) males, 11/50 (22%) females; age 30.6 years (11-78) at the time of the first study.
clinical history in 50/50 pts, electrocardiogram in 50/50 pts, Holter monitoring in 50/50 pts, ergometric test in 49/50 pts, non-invasive analysis using signal-averaging QRS in 17/50 pts, 2D echocardiogram in 50/50 pts, angiography in 38/50 pts, electrophysiological endocavitary study in 35/50 pts.
familial 2/50 (4%); 1/50 (2%) was in class II NYHA; first arrhythmia at 24.6 years (8-60); most severe arrhythmia at 27.7 years (9-74). Forty-three out of fifty patients (86%) were symptomatic for arrhythmias: 28/50 (56%) as a result of stress; 20/50 (40%) had life-threatening symptoms; 6/50 (12%) had aborted sudden death. Arrhythmogenic right ventricular disease was "localized" in 42/50 (84%) and "diffused" in 8/50 (16%) and was associated with anomalies of the left ventricle in 30/50 (60%). Electrocardiogram showed: right bundle branch block in 10/50 (20%), negative T wave on the right precordial leads in 19/50 (38%), delayed ventricular potentials in 4/17 (23.5%). Using the electrocardiogram, Holter monitoring and electrophysiological endocavitary study the following were documented: a) clinical ventricular tachycardia in 40/50 (80%): non-sustained ventricular tachycardia in 10/50 (20%), sustained ventricular tachycardia in 30/50 (60%); b) electrically induced ventricular tachycardia in 26/35 (74.2%): non-sustained ventricular tachycardia in 8/35 (22.8%), sustained ventricular tachycardia in 18/35 (51.4%) (clinical sustained ventricular tachycardia in 18/18); c) multiform ventricular tachycardia in 12/50 (24%) (diffused arrhythmogenic right ventricular disease in 3/12 and associated anomalies of the left ventricle in 11/12); d) pleomorphic sustained ventricular tachycardia in 9/30 (30%) (diffused arrhythmogenic right ventricular disease in 2/9, and associated anomalies of the left ventricle in 8/9). Forty-two out of fifty patients (84%) underwent antiarrhythmic treatment. When the study was carried out 6.6 years (1 month-22 years) had passed since the first symptom; follow-up was 2.1 years (1 month-11 years) while the interval between the first symptom and the last check-up was 8.4 years (1-30 years); 2/50 dropped out and 2/50 died suddenly.(ABSTRACT TRUNCATED AT 400 WORDS)
致心律失常性右心室疾病是一种涉及广泛解剖学 - 临床特征的综合征。其特征为不同程度的右心室异常以及危及生命的“右心室”高动力性室性心律失常。1977年至1988年期间，在特伦托心律失常中心对50例连续患者进行了回顾性研究。严格的心律失常学、超声心动图和血管造影标准结果显示，所有患者均患有致心律失常性右心室疾病。
男性39/50（78%），女性11/50（22%）；首次研究时年龄为30.6岁（11 - 78岁）。
50/50例患者均有临床病史，50/50例患者进行了心电图检查，50/50例患者进行了动态心电图监测，49/50例患者进行了运动试验，17/50例患者进行了信号平均QRS无创分析，50/50例患者进行了二维超声心动图检查，38/50例患者进行了血管造影，35/50例患者进行了心腔内电生理研究。
家族性2/50（4%）；1/50（2%）为纽约心脏病协会（NYHA）Ⅱ级；首次心律失常发生于24.6岁（8 - 60岁）；最严重心律失常发生于27.7岁（9 - 74岁）。50例患者中有43例（86%）因心律失常出现症状：28/50（56%）因应激出现症状；20/50（40%）有危及生命的症状；6/50（12%）有过猝死未遂。致心律失常性右心室疾病“局限型”42/50（84%），“弥漫型”8/50（16%），30/50（60%）与左心室异常相关。心电图显示：右束支传导阻滞10/50（20%），右胸前导联T波倒置19/50（38%），心室晚电位4/17（23.5%）。通过心电图、动态心电图监测和心腔内电生理研究记录到：a）临床室性心动过速40/50（80%）：非持续性室性心动过速10/50（20%），持续性室性心动过速30/50（60%）；b）电诱发室性心动过速26/35（74.2%）：非持续性室性心动过速8/35（22.8%），持续性室性心动过速18/35（51.4%）（临床持续性室性心动过速18/18）；c）多形性室性心动过速12/50（24%）（弥漫性致心律失常性右心室疾病3/12，合并左心室异常11/12）；d）多形性持续性室性心动过速9/30（30%）（弥漫性致心律失常性右心室疾病2/9，合并左心室异常8/9）。50例患者中有42例（84%）接受了抗心律失常治疗。自首次出现症状起至进行该研究时已过去6.6年（1个月 - 22年）；随访时间为2.1年（1个月 - 11年），首次症状至最后一次检查的间隔时间为8.4年（1 - 30年）；2/50退出研究，2/50猝死。（摘要截断于400字）
