ECG T-WAVE PATTERNS IN GENETICALLY DISTINCT FORMS OF THE HEREDITARY LONG QT SYNDROME
ECG T-WAVE PATTERNS IN GENETICALLY DISTINCT FORMS OF THE HEREDITARY LONG QT SYNDROME
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DOI：
10.1161/01.cir.92.10.2929
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发表时间：
1995-11-15
期刊：
影响因子：
37.8
通讯作者：
TIMOTHY, KW
中科院分区：
文献类型：
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作者：
MOSS, AJ;ZAREBA, W;TIMOTHY, KW
长QT综合征是一种遗传性疾病，具有心室复极延长、室性快速心律失常和猝死的倾向。最近的连锁研究表明，这种疾病在3号、7号和11号染色体上有三个独立的基因座，在其中两条染色体上发现了长QT综合征的特异性突变基因。我们研究了心电图T波模式方法和结果五个定量心电图复极参数，即四个Bazett校正时间间期，（QT（起始-c）、QT（峰值-c）、QT（c）和T-持续时间-c，单位为毫秒）和T波的绝对高度（T-振幅，毫伏），测量了6个长QT综合征家族的153名成员与染色体3（n=47），7（n=30），11（n=76）上的标记。基因型数据被用来定义每个家庭成员是否受长QT综合征的影响。所有6个家族的受累成员的QT间期（QT（onset-c）或QT（c））均长于未受累的家族成员（P
Background The long QT syndrome is an inherited disorder with prolonged ventricular repolarization and a propensity to ventricular tachyarrhythmias and sudden arrhythmic death. Recent linkage studies have demonstrated three separate loci for this disorder on chromosomes 3, 7, and 11, and specific mutated genes for long QT syndrome have been identified on two of these chromosomes. We investigated ECG T-wave patterns (phenotypes) in members of families linked to three genetically distinct forms of the long QT syndrome.Methods and Results Five quantitative ECG repolarization parameters, ie, four Bazett-corrected time intervals (QT(onset-c), QT(peak-c), QT(c), and T-duration-c, in milliseconds) and the absolute height of the T wave (T-amplitude, in millivolts), were measured in 153 members of six families with long QT syndrome linked to markers on chromosomes 3 (n=47), 7 (n=30), and 11 (n=76). Genotypic data were used to define each family member as being affected or unaffected with long QT syndrome. Affected members of all six families had longer QT intervals (QT(onset-c), or QT(c)) than unaffected family members (P