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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V1~3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)、β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 and analyze the clinical features of patients with arrhythmogenic right ventricular dysplasia or cardiomyopathy (ARVD / C) in China. Methods The clinical data of 96 patients with ARVD / C, including demographics, medical history, family history, clinical manifestations and electrocardiogram (ECG) were collected from 24 Chinese provinces and cities collected from China ARVD / C registered in 1994-2005. , 24-hour ambulatory electrocardiogram) and echocardiography and treatment, some patients underwent right ventricular angiography, cardiac magnetic resonance imaging (MRI) and endomyocardial biopsy. Results The average age of 96 patients (37 ± 15) years was 75 years (78.1%). Among them, 37 (38.5%) had a history of syncope and 70 (72.9%) had only palpitations. Sudden death (SD) was induced in 14 patients (14.6%), and SD (34 ± 11) years old occurred in 78.6% (11/14) of male patients; 55 patients (57.3%) had ECG records of origin Ventricular tachycardia (VT) in the right ventricle (RV). In addition to 1 patient, the remaining 95 patients (99.0%) had abnormal echocardiography, mainly RV enlargement; epsilon waves were found in electrocardiogram in 30 patients (31.25%) and V1 ~ 3 in 47 patients (49.0%) T wave inversion; patients with symptomatic ventricular arrhythmias received amiodarone (n = 26), mexiletine (n = 21), beta blockers (n = 14), verapamil (n = 4) Or use other antiarrhythmic treatment. 31.52% of patients (29/96) were refractory to antiarrhythmic drug therapy and 19 patients underwent radiofrequency catheter ablation. Eleven of the patients had VT recurrence. Four patients were implanted with an implanted cardioverter defibrillator (ICD). Conclusions According to the diagnostic criteria of the International Working Group, only severe cases of ARVD can be diagnosed in China. The effect of antiarrhythmic drugs is poor in over 30% of the patients. 57.89% of the patients are ineffective with radiofrequency ablation and need ICD implantation. However, ICD implantation rate in China only accounted for 4.17%.