致心律失常性右心室心肌病的流行病学研究和家系随访  

Epidemiology of arrhythmogenic rig ht ventricular cardiomyopathy and follow -up in two Chinese families

作　　者：杨春梅[1] 黄峻[1] 单其俊[1] 许迪[1] 陆凤翔[1] 曹克将[1] 

机构地区：[1]南京医科大学第一附属医院心血管科,210029

出　　处：《中华心律失常学杂志》2002年第1期48-53,共6页Chinese Journal of Cardiac Arrhythmias

摘　　要：目的　通过收集致心律失常性右心室心肌病 (arrhythmogenicrightventricularcardiomyopathy ,ARVC)的散发人群资料和随访ARVC家系 ,提供ARVC的流行病学资料并发现病情变化 ,寻找疾病规律。方法　统计就诊 33例ARVC患者的流行病学资料。分别随访 2个ARVC家系 3 5年和 4 5年。询问随访前后每一家系成员病史 ,做心电图、二维超声心动图和心室晚电位 ,必要时进行 2 4h动态心电图检查 ,了解病情变化。根据欧洲心脏病学会诊断标准作出诊断。结果　 33例ARVC患者中男性 18例 ,女性 15例 ,平均诊断年龄 (4 2 8± 14 9)岁 ,2 0～ 6 0岁者占 88%。随访的第 1个家系共有 2例患者 ,均猝死。另 1个家系随访结束后共发现 11例患者。随访中患者症状无明显变化 ,但 2名家系成员新确诊患病 ;1例患者心室晚电位变为阳性 ;1例患者心电图右胸前导联T波倒置导联数增加 ,3例T波倒置由右胸前导联向左胸前导联扩展 ,3例T波异常累及右胸前导联和左胸前导联 ;1例患者Epsilon波增宽 ,1例患者新出现Epsilon波 ;4例患者V1 导联QRS时限增加。扩展调查此家系又发现 2例患者和高度怀疑的 1例患者 ,先证者外祖父母为近亲结婚。结论　ARVC男女发病率相似 ,好发于青壮年 ;是一进展性疾病 ,虽是右心室疾病 ,可能多有侵犯左心室 。Objective The aim of this study is to provide epidemiology data,explore occurrence and development of arrhythmogenic right ven tricular cardiomyopathy (ARVC) in two Chi nese families Methods Stati stic epidemiology data were obtained in 33 patients with ARVC Two ARVC families were followed up by 3 5 years and 4 5 ye a rs respectively All family members were ob tained medical history and undertaken ca rdiac examination including electrocardi ography (ECG),2 dimensional ech ocardiogra phy (2 DE),and signal averaged electrocar diography (SAECG) during follow up. Holter monitoring were performed in some membe rs when necessary Results ① E pidemiology data There were 18 males and 15 females in 33 ARVC patients,the aver age age was(42 8±14 9) years ol d (±s) Eigh ty eight percent p atien ts were between 20～60 years old ②Follow up in the first family There were totall y 2 patients in this family and both the 2 patients were suffered from sudden de ath ③Follow up in the second family Ther e were 11 patients at the end of the fol low up in this family Two previously con sidered'normal'members became ARVC patie nts There were no significant changes in symptoms SAECG turned to positive in 1 patient The leads of right precordial in verted T waves increased in 1 patient;Th e leads of inverted T waves extended fro m right precordial to left precordial in 3 patients;T waves were abnormal in rig ht precordial and left precordial leads in another 3 patients QRS duration ( QRSd) of V 1 increased in 4 members One patien t had new emerged Epsilon wave in V 1 le ad and Epsilon wave became wide in anoth er 1 patient Two new patients and highly suspected 1 member were found in extend ing investigation,and proband's maternal grandfather and grandmother were confir med to be consanguineous marriage Conclusion ARVC mainly onsets in t he young or adults without sex differenc es There were sudden death and new found patients as well as progressive changes during follow

关 键 词：致心律失常右心室心肌病 家系 随访 猝死 流行病学 ARVC 

分 类 号：R541.7[医药卫生—心血管疾病]