Section of Cardiology, Department of Molecular Medicine, University of Pavia, Pavia, Italy.
Heart Rhythm. 2012 Jul;9(7):1104-12. doi: 10.1016/j.hrthm.2012.02.014. Epub 2012 Feb 13.
Although QT prolongation following myocardial infarction (MI) is generally moderate, cases with marked QT prolongation leading to life-threatening torsades de pointes (TdP) have been described.
To investigate the genetic substrate of this phenomenon.
We studied 13 patients who developed TdP in the subacute phase of MI (2-11 days) and a group of 133 ethnically matched controls with uncomplicated MI. Long QT syndrome genes and the KCNH2-K897T polymorphism were screened by using denaturing high-performance liquid chromatography plus direct sequencing and a specific TaqMan assay, respectively.
Two of the 13 patients (15%) who presented with QT prolongation and TdP were found to carry long QT syndrome mutations (KCNH2-R744X and SCN5A-E446K). Nine of the remaining 11 patients (82%) carried the KCNH2-K897T polymorphism, which was present in 35% of the controls (P = .0035). Thus, patients with an acute MI carrying the KCNH2-K897T polymorphism had an 8-fold greater risk of experiencing TdP compared with controls (95% confidence interval = 2-40).
Our data suggest that the common K897T polymorphism is associated with an increased risk of TdP developing in the subacute phase of MI. Our findings support the concept that the electrical remodeling associated with this healing phase of MI may unmask a genetic substrate predisposing to a time-limited development of life-threatening arrhythmias. They also provide the first line of evidence in support of the hypothesis that a common polymorphism, previously described as a modifier of the severity of LQTS, may increase the risk of life-threatening arrhythmias in a much more prevalent cardiac disease such as myocardial infarction.
尽管心肌梗死后(MI)的 QT 延长通常是中度的,但已有明显 QT 延长导致危及生命的尖端扭转型室性心动过速(TdP)的病例报道。
研究这种现象的遗传基础。
我们研究了 13 例在 MI 亚急性期(2-11 天)发生 TdP 的患者和一组 133 例无并发症 MI 的种族匹配对照者。采用变性高效液相色谱法结合直接测序和特定 TaqMan 测定法分别筛选长 QT 综合征基因和 KCNH2-K897T 多态性。
在 13 例出现 QT 延长和 TdP 的患者中,有 2 例(15%)发现携带长 QT 综合征突变(KCNH2-R744X 和 SCN5A-E446K)。其余 11 例患者中的 9 例(82%)携带 KCNH2-K897T 多态性,而对照组中该多态性的发生率为 35%(P=0.0035)。因此,携带 KCNH2-K897T 多态性的急性 MI 患者发生 TdP 的风险比对照组高 8 倍(95%置信区间=2-40)。
我们的数据提示,常见的 K897T 多态性与 MI 亚急性期 TdP 的发生风险增加相关。我们的研究结果支持这样一种概念,即与 MI 愈合期相关的电重构可能会揭示一种遗传基础,使心律失常的发生具有一定的时间限制,危及生命。这些结果还首次为假设提供了证据,即先前被描述为长 QT 综合征严重程度修饰因子的常见多态性,可能会增加比心肌梗死更为普遍的心脏疾病(如心肌梗死)中危及生命的心律失常的风险。