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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 ,易致猝死
OBJECTIVE: To collect the epidemiological data of ARVC and find out the change of the disease by looking for the distribution of arrhythmogenic right ventricular cardiomyopathy (ARVC) disseminated population data and ARVC pedigree. Methods Statistics 33 cases of ARVC epidemiological data. Two ARVC pedigrees were followed up for 35 years and 45 years respectively. Asked before and after follow-up each family member’s medical history, do electrocardiogram, two-dimensional echocardiography and ventricular late potential, if necessary, 24 h Holter check to understand the condition changes. According to the diagnostic criteria of the European Society of Cardiology diagnosis. Results Among the 33 patients with ARVC, 18 were male and 15 were female, with a mean age at diagnosis of 428 ± 14 9 years and 88% of those aged 20-60 years. A total of 2 patients in the first pedigree were followed up. Another family after follow-up found a total of 11 patients. There was no significant change in the symptoms of the patients at follow-up, but two newly diagnosed members of the pedigree became ill. One patient had positive ventricular late potentials. One patient had an increased number of inverted T wave leads in the right chest anterior descending lead, From left anterior thoracic lead to the left anterior thoracic extension, 3 cases of T-wave abnormalities involving the right anterior thoracic anterior leads and left anterior thoracic lead; Epsilon wave broadened in 1 patient, Epsilon wave in 1 patient; 4 Patients with lead V1 QRS time limit increased. Expanded survey The family also found two patients and one highly suspected patient, the proband’s grandparents married. Conclusions The incidence of ARVC is similar between men and women and occurs in young and middle-aged adults. It is a progressive disease. Although it may be a right ventricular disease, there may be multiple violations of the left ventricle and sudden death