摘要
目的 调查与研究先天性长 QT综合征 ( long QT syndrome,L QTS)的遗传特点及家系图谱分析 ,L QTS的心电图分型、诊断以及治疗。方法 应用惠普 12导联心电图机对先证者及其家庭成员分别记录 6导联及 12导联常规体表心电图 ,对先证者行 2 4h动态心电图检查 ,分别测量 、V2 导联的QT、QTc以及 QTd。先证者及家族中有症状的患者给予普萘洛尔 2～ 3mg/ kg,每日 3次治疗。结果 187个家庭成员中有 31例为 L QTS患者 ,男性 11例 ,女性 2 0例。其中 L QT1 18例 ,L QT2 12例 ,L QT3 1例。QT间期为 ( 0 .5 7± 0 .0 8) s,QTc为 ( 0 .5 8± 0 .0 8) s,QTd为 ( 0 .16± 0 .0 3) s。除 1例应用阿托品治疗有效 ,6例猝死外 ,其余 2 4例 ( 77.4% )患者应用普萘洛尔治疗有效。无症状的 QTc延长者 5 6例 ,男性 2 3例 ,女性 33例 ,L QT1 2 2例 ,L QT2 34例。 QT间期为 ( 0 .47± 0 .0 9) s,QTc为 ( 0 .5 2± 0 .0 7) s,QTd为( 0 .0 8± 0 .0 3) s,有症状组与无症状组相比有统计学意义。家系图谱分析符合常染色体显性遗传规律。 结论 12导联体表心电图是 L QTS常用的最简单的检查方法 ,心电图对 L QTS的临床分型基本准确可靠。普萘洛尔是预防 L
Objective The inheritary and clinical characteristical electrocardiogram (ECG) types,diagnosis and treatment of long QT syndrome (LQTS) were investigated by pedigree analysis of 20 families with this disease. Methods Six or 12 lead body surface ECG of all the probands and family members were recorded and 24 hour Holter monitoring was recorded for the probands in addition.The QT、QTc of lead Ⅱ or V 2 and the QTd of all the members were measured. Results Among 187 family members there were 31 symptomatic patients with LQTS,including 11 males and 20 females.According to the clinical characteristics 18 patients belong to LQT 1,12 patients to LQT 2 and 1 patient to LQT 3.The QT intervals were (0 57±0 08) seconds,QTc (0 58±0 08) seconds and QTd (0 16±0 03) seconds respectively.There were 56 family members with asymptomatic QT prolongation,including 23 males and 33 females,including LQT 1 22,LQT 2 34.The QT intervals were (0 47±0 09) seconds,QTc (0 52±0 07) seconds, and QTd (0 08±0 03) second respectively.There were significant difference in QT, QTc and QTd between the patients with LQTS and asymptomatic members.Six patients died of sudden cardiac death,one patient was treated with atropine effectively,and the remaining twenty four of thirty one patients (77 4%) with LQTS were effectively treated with propranolol.Pedigree analysis was compatible with law of antosomal dominant inheritance. Conclusion Twelve lead surface ECG is an useful and simple tool to diagnose LQTS,and is a reliable method to distinguish types of LQTS.Propranolol is an effective drug to prevent sudden cardiac death in most patients with LQTS.
出处
《中华心律失常学杂志》
2001年第1期15-17,共3页
Chinese Journal of Cardiac Arrhythmias
关键词
长QT综合征
体表心电图
系谱分析
普萘洛尔
Long QT syndrome
Surface electrocardiogram
Pedigree analysis
Propranolol
