Tuncer C, Cokkeser Y, Komsuoglu B, Ozdemir R, Güven A, Pekdemir H, Sezgin A T, Ilhan A
Department of Cardiology, University of Kocaeli, Medical School, Kocaeli, Turkey.
Pediatr Cardiol. 2000 Mar-Apr;21(2):135-40. doi: 10.1007/s002469910021.
The long QT syndrome is a congenital disease with frequent familial transmission, characterized primarily by prolongation of the QT interval and by the occurrence of life-threatening arrhythmias. The syndrome may be familial, with or without congenital deafness, or it may be idiopathic. We attempted to assess ventricular repolarization and to identify patients with the Jervell and Lange-Nielsen syndrome among 132 deaf-mute school children. Five deaf-mute subjects had Jervell and Lange-Nielsen syndrome. The deaf-mute subjects were divided into two subgroups according to the length of their QT intervals: group 1 included 5 cases with the long QT interval (>440 msec), and group 2 included 127 subjects with the normal QT interval (< or =440 msec). Group 3 was composed of 96 control subjects. The mean QT, QTc, JT, and JTc intervals (418+/-70, 500+/-38, 302+/- 65, and 389+/-36 msec, respectively) in group 1 were significantly longer than those of group 2 (344+/-23, 408+/-22, 249+/-34, and 291+/-28 msec, respectively) and group 3 (325+/-11, 383+/-26, 228 +/-36, and 269+/-46 msec, respectively). The dispersion (d) values (QT-d, QTc-d, JT-d, and JTc-d; 63+/-10, 73+/-8, 60+/-8, and 62+/-11 msec, respectively) of group 1 were significantly longer than those of group 2 (49+/-16, 43+/-11, 48+/-21, and 45+/-18 msec, respectively) and group 3 (33+/-13, 33+/-14, 28+/-16, and 27+/-14 msec, respectively) at similar mean RR intervals. Also, the mean QT, QTc, JT, and JTc intervals and the dispersion values (QT-d, QTc-d, JT-d, and JTc-d) in group 2 were significantly longer than those of group 3 at similar mean RR intervals. Consequently, in this study, we determined that the deaf-mute children who did not meet the criteria for Jervell and Lange-Nielsen syndrome still had evidence of subtle derepolarization abnormalities evidenced by intermediate prolongation of QTc, JTc, and the corresponding measures of dispersion, and we believe an electrocardiogram examination of deaf-mute subjects will reveal this potentially life-threatening syndrome.
长QT综合征是一种具有频繁家族遗传倾向的先天性疾病，主要特征为QT间期延长及危及生命的心律失常的发生。该综合征可能是家族性的，伴有或不伴有先天性耳聋，也可能是特发性的。我们试图在132名聋哑学童中评估心室复极化情况，并识别患有杰韦尔和朗格 - 尼尔森综合征的患者。5名聋哑受试者患有杰韦尔和朗格 - 尼尔森综合征。根据QT间期长度，将聋哑受试者分为两个亚组：第1组包括5例QT间期延长（>440毫秒）的病例，第2组包括127例QT间期正常（≤440毫秒）的受试者。第3组由96名对照受试者组成。第1组的平均QT、QTc、JT和JTc间期（分别为418±70、500±38、302±65和389±36毫秒）显著长于第2组（分别为344±23、408±22、249±34和291±28毫秒）和第3组（分别为325±11、383±26、228±36和269±46毫秒）。在相似的平均RR间期时，第1组的离散度（d）值（QT - d、QTc - d、JT - d和JTc - d；分别为63±10、7
