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目的:通过对22例致心律失常性右室心肌病(ARVC)患者的远期随访观察,探讨ARVC的临床特征和远期预后。方法:对1996～2006年于南京医科大学第一附属医院确诊为ARVC的22例患者进行回顾性分析并随访,随访内容包括治疗经过、症状、有无猝死以及心电图、超声心动图、动态心电图等。结果:22例ARVC患者平均随访时间(6.1±2.9)年(2.2～12年)。共有2例患者猝死,年平均病死率1.5%。心电图随访中新出现Epsilon波3例、肢体导联低电压2例、完全性右束支传导阻滞1例,完全性左束支传导阻滞1例,T波倒置1例,T波倒置导联数增加2例;比较随访前后肢体导联电压的绝对值和有明显降低(P<0.01)。心脏超声示左室结构和功能在随访前后无明显变化(P>0.05)。10例射频消融成功的患者经随访有7例室速复发,均为新形态的室速;2例安装ICD患者,ICD有效工作并存活;10例单纯口服抗心律失常药物治疗患者,6例室速复发,其中3例患者出现新形态的室速,2例猝死。结论:①ARVC患者发病年龄以中青年为主,随访期间心电图肢导电压绝对值和降低,超声心动图较少发现左心结构、功能有明显变化;②以药物或射频消融治疗ARVC复发率均较高,目前ICD使用率较低。
Objective: To investigate the clinical features and long-term prognosis of ARVC by observing the long-term follow-up of 22 patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods: Twenty-two patients with ARVC diagnosed at the First Affiliated Hospital of Nanjing Medical University from 1996 to 2006 were retrospectively analyzed and followed up. The follow-up included treatment, symptom, sudden death, electrocardiogram, echocardiography, . Results: The average follow-up time of 22 ARVC patients was (6.1 ± 2.9) years (2.2 to 12 years). A total of 2 patients died of sudden death, the average annual mortality rate of 1.5%. There were 3 new cases of Epsilon wave, 2 cases of low limb lead voltage, 1 case of complete right bundle branch block, 1 case of complete left bundle branch block, 1 case of T wave inversion, T wave inversion guide The number of conjunctions increased in 2 cases. The absolute value of limb lead voltage was significantly decreased before and after follow-up (P <0.01). Echocardiography showed no change in left ventricular structure and function before and after follow-up (P> 0.05). Seven patients with successful RFA were followed up for ventricular tachycardia. All of them were new forms of ventricular tachycardia. ICD was effective and survived in 2 patients with ICD. Six patients were treated with oral antiarrhythmic drugs alone Fast recurrence, of which 3 patients showed a new form of ventricular tachycardia, 2 cases of sudden death. Conclusion: ①The age of onset of ARVC is mainly young and middle-aged. The absolute value and decrease of electrocardiogram limbal conduction voltage during follow-up, echocardiography has less left ventricular structure, and the function has obvious changes. ② The recurrence rate of ARVC with drugs or radiofrequency ablation High, the current ICD usage is low.