中国人心律失常性右心室发育不良或心肌病的临床特点

Clinical characteristics in Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy

目的总结分析中国致心律失常性右心室发育不良或心肌病（ARVD／C）患者的临床特征。方法分析中国ARVD／C注册研究组收集的中国24个省市1994～2005年间发现的96例ARVD／C患者的临床资料，包括人口统计学、病史、家族史、临床表现、心电图（12导联、24小时动态心电图）和超声心动图以及治疗情况，部分患者接受右心室造影、心脏磁共振成像（MRI）和心内膜心肌活组织检查。结果96例患者的平均年龄（37±15）岁．男性75例（78．1％）；其中37例（38．5％）患者有晕厥病史，70例（72．9％）患者仅有心悸症状。14例患者（14．6%）因劳力诱发了猝死（SD），发生SD的年龄（34±11）岁，其中78．6％（11／14）为男性患者；55例患者（57．3％）心电图记录到起源于右心室（RV）的室性心动过速（VT）。除1例患者外。其余95例（99．0％）超声心动图结果异常，主要为RV扩大；30例（31．25％）患者的心电图中可见epsilon波，47例（49．0％）患者V（1～3）导联T波倒置；有症状的室性心律失常患者接受胺碘酮（n=26）、美西律（n=21）、0阻滞剂（n=14）、维拉帕米（n=4）或采用其他抗心律失常治疗。31．52％的患者（29／96）应用抗心律失常药物治疗无效，19例患者接受射频消融治疗，但其中有11例患者出现VT复发。4例患者植入植入式心脏自动起搏除颤器（ICD）。结论根据国际工作组的诊断指标．在中国只有严重的ARVD病例才能被诊断；30％以上的患者应用抗心律失常药物的疗效很差，57．89％的患者应用射频消融治疗无效，需要植入ICD，但ICD植入率在中国仅占4．17％。

Objective To summarize the clinical presentations in Chinese. To summarize the clinical presentations of arrhythmogenic right ventricular dysplasia/Cardiomyopathy（ARVD/C） in Chinese, Methods ARVD/ C cases were collected by the ARVD Registry of China. The clinical evaluations were assessed by family history of SD, electrocardiagram （ 12-lead, Hoher, and signal-averaging）, echocardiography, magnetic responance imaging and endomyocardial biopsy were applied as additional diagnostic options. Results There were 96 Chinese ARVD/C cases. mean age （37 ~ 15） years old, 75 male, from 1994-2005. Among them, 38.5% （37 cases） had syncope episodes; 72.9% （70 cases） had only palpitations; SD（mean age 34±11 years old, 11 male） occurred in 14.6%（14/96） of patients during physical stress. Ventricular tachycardia（VT） with right ventricular （RV） origin was documented in 57.3% （55/79）. Abnormal echocardiography was found in 95 patients （99.0% ）, mainly RV dilation, Epsilon waves were evident in 31.25%（30 cases） patients, Inverted T waves in leads V1-3 were seen in 49.0% patients（47 cases）. For symptomatic patients, ventricular arrhythmias were treated by amiodarone （n -26）, mexiletine （n ：= 21 ）. β blocker（ n =14） and verapamil（ n = 4） or other antiarrhythmia therapies. Antiarrhythmic drugs were ineffective in 31, 52%（29/96） patients; 19 cases of them were therefore treated by ablations. Nevertheless, VT recurred in 11 patiets. Four patients were implanted cardial defibrillation（ICDs）. Conclusion Based on the Task Force Criteria only the severe ARVD cases were diagnosed in China. More than 30% of patients were refractory to antiarrhythmic drugs, and RF ablation was ineffective in 57.89 %, therefore ICD therapy is necessary in these patients, but only ,1.3594 of patients received ICDs.