遗传性长QT间期综合征61个家系成员的心电图分析  

Spectrum of ST-T-wave changes in hereditary long-QT syndrome in Chinese

作　　者：刘文玲[1] 胡大一[1] 李志明[2] 李翠兰[1] 李运田[1] 秦绪光[3] 李蕾[1] 

机构地区：[1]北京大学人民医院心内科,100044 [2]首都医科大学附属同仁医院心内科 [3]北京酒仙桥医院心内科

出　　处：《中华内科杂志》2004年第5期352-355,共4页Chinese Journal of Internal Medicine

基　　金：国家自然科学基金资助项目 (3 0 170 3 81)

摘　　要：目的　了解中国遗传性长QT间期综合征 (LQTS)心电图形态改变。方法　应用 12导联心电图对 6 1个遗传性LQTS家系共 32 2例成员观察了心电图ST T形态改变 ,并对其中 78例有症状患者加以分型。应用测序方法进行基因分型。结果　心电图类似LQT1型 32例 ,类似LQT2型 4 1例 ,类似LQT3型 2例。不典型的有 3例。患者不同时间T波形态发生变异者 4 8例。有症状患者QTc(0 5 4 7± 0 0 8)s,无症状患者QTc(0 5 2 6± 0 0 6 )s,显著长于正常成员。同一患者不同时间QTc的差值 (ΔQTc) :患者 (0 0 4 8± 0 0 5 7)s,正常成员 (0 0 2 3± 0 0 17)s,两组间差异有显著性 (P <0 0 0 1)。基因筛查证实LQT112例 ,LQT2 11例。结论　本组遗传性LQTS患者心电图表现不完全与欧美患者相同 ;T波形态变化较大 ,表现在同一类型间、同一家系中和同一患者的不同时间ECGST T均可出现较大的差异 ;同一患者不同时间QTc亦有较大变化。Objective To evaluate the spectrum of ST-T-wave patterns in Chinese patients with hereditary long-QT syndrome. Methods ECGs of 61 families were studied to determine ST-T-wave patterns. Genotypes were identified by sequencing. Results 32 cases showed similarity to LQT1,41 to LQT2 and 2 to LQT3 in ECG ,and 3 cases could not be classified. QTc of the patients with symptom was (0.547±0.08)sec and that of the patients without symptoms was (0.526±0.06) sec,both were much longer than that of normal members of the families. T wave patterns were different in 48 cases at different time.QTc difference in a same person between two times or ΔQTc were as follows:(0.048±0.057) sec in patients and (0.023±0.017) sec in normal members( P <0.001).12 cases of LQT1 and 11 cases of LQT2 were identified by genotype sequencing. Conclusions There are some differences in ST -T waves between these Chinese hereditary long-QT syndrome patients and European and American patterns . These patients showed greater variability in ST-T wave pattern including the changes in the same type,in the same pedigree and in the same patients at different time.

关 键 词：遗传性长QT间期综合征 心电图 LQTS 心电描记术 常染色体遗传性心脏病 

分 类 号：R541.7[医药卫生—心血管疾病]