HLA-B27阴性强直性脊柱炎一家系的心脏损害特征

目的观察 HLA-B27阴性家族性强直性脊柱炎(AS)心脏损害的特征。方法所有家系成员均采集完整病史、临床检查、常规12导联心电图、彩色多普勒超声心动图检查。并与年龄匹配的正常人对比分析。结果 33例家系成员中发生心房颤动的6例,占总心律失常人数的46%,心动过缓2例,高度房室传导阻滞1例,病态窦房结综合征1例,完全性右束支传导阻滞2例,室内差异性传导1例(排除 AS),所有传导系统异常的心律失常占 AS患者总心律失常人数的36%。因心脏原因死亡3例,其中1例死于全自动双腔起搏器(DDD)术后1年。AS 患者中 NYHA 心功能分级Ⅰ级8例。级2例,Ⅲ级1例,左心房扩大5例(45%),二尖瓣返流6例,三尖瓣返流4例。结论家族性 AS 心脏损害发生心律失常的种类有相似的趋势,主要以心房颤动为主,累及传导系统的心律失常次之,心脏损害以左心房扩大,舒张功能降低为主,可伴有二尖瓣和/或三尖瓣的返流。

The characteristics of cardiac damages in a negative HLA-B27 ankylosing spondylitis family

Objective To observe the characteristics of cardiac damages in a negative HLA-B27 ankylosing spondylitis (AS)family.Methods Case history,clinical examination,routine 12 leads electrocardiogram and color Doppler ultrasound were performed in all family members and compared with health adults matched with age.Results atrial fibrillation occurred in 6 family members,which accounted for 46 percent of whole arrhythmia members(13 cases).Bradycardia,high degree A-V block,sick sinus syndrome,right bundle branch block and conduction variability inside ventricles occurred in 2,1,2 and 1 patients respectively.Arrhythmias with abnormal conduction accounted for 36 percent of whole arrhythmia members.Three Cardiac deaths were observed,1 died 1 year after the DDD pacemaker implant.With the cardiac function,according to the NYHA criteria,8 cases were at the NYHA Ⅰ stage,2 cases at NYHA stage,1 case at NYHA Ⅲ stage.5 cases had left atrial enlargement which accounted for 45 percent of whole AS patients.Mitral regurgitation and tricuspid regurgitation occurred in 6 and 4 patients respectively.Conclusions Arrhythmic types had homoplastic tendency in patients with cordis lesion of familial AS.Atrial fibrillation was primary,arrhythmias related with abnormal conducting system was secondary to it.Left atrial enlargement and the lower diastolic function were primary with mitral and/or tricuspid regurgitation in patients with cordis lesion.