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首页> 外文期刊>PACE: Pacing and clinical electrophysiology >Ventricular repolarization and heart rate responses during cardiovascular autonomic function testing in LQT1 subtype of long QT syndrome.
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Ventricular repolarization and heart rate responses during cardiovascular autonomic function testing in LQT1 subtype of long QT syndrome.

机译：长QT综合征LQT1亚型的心血管自主功能测试中的心室复极化和心率反应。

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BACKGROUND: In the most prevalent LQT1 form of inherited long QT syndrome symptoms often occur during abrupt physical or emotional stress. Sympathetic stimulation aggravates repolarization abnormalities in experimental LQT1 models. We hypothesized that autonomic function tests might reveal the abnormal repolarization in asymptomatic LQT1 patients. METHODS: We measured heart rates (HRs) and QT intervals in nine asymptomatic carriers of a C-terminal KCNQ1 mutation and 8 unaffected healthy subjects using an approach of global QT values derived from 28 simultaneous electrocardiographic leads on beat-to-beat base during Valsalva maneuver, mental stress, sustained handgrip, and light supine exercise. RESULTS: LQT1 patients exhibited impaired shortening of both QTpeak and QTend intervals during autonomic interventions but exaggerated lengthening of the intervals--a QT overshoot--during the recovery phases. The number of tests with a QT overshoot was 2.4 +/- 1.7 in LQT1 patients and 0.8 +/- 0.7 in unaffected subjects (P = 0.02). Valsalva strain prolonged T wave peak to T wave end interval (TPE) in LQT1 but not in unaffected patients. LQT1 patients showed diminished HR acceleration in response to adrenergic challenge whereas HR responses to vagal stimuli were similar in both groups. CONCLUSIONS: Standard cardiovascular autonomic provocations induce a QT interval overshoot during recovery in asymptomatic KCNQ1 mutation carriers. Valsalva maneuver causes an exaggerated fluctuation of QT and TPE intervals partly explaining the occurrence of cardiac events during abrupt bursts of autonomic activity in LQT1 patients.
机译：背景：以最普遍的LQT1形式遗传的长QT综合征通常在突然的身体或情绪压力期间出现症状。交感刺激加剧了实验性LQT1模型中的复极异常。我们假设自主神经功能检查可能会揭示无症状LQT1患者的异常复极化。方法：我们使用全局QT值的方法，测量了来自C端KCNQ1突变的9个无症状携带者和8名未受影响的健康受试者的心率（HRs）和QT间隔，该方法来自于Valsalva期间逐搏的28条同步心电图导联动作，精神压力，持续握力和轻度仰卧运动。结果：LQT1患者在自主干预期间表现出QTpeak和QTend间隔缩短的受损，但是在恢复阶段夸大了间隔的延长-QT过冲。 LQT1患者的QT过冲测试数量为2.4 +/- 1.7，未受影响的受试者为0.8 +/- 0.7（P = 0.02）。 Valsalva菌株延长了LQT1的T波峰到T波结束间隔（TPE），但未影响患者。 LQT1患者对肾上腺素刺激的反应显示HR加速度降低，而两组对迷走神经刺激的HR反应相似。结论：在无症状的KCNQ1突变携带者恢复过程中，标准的心血管自主神经激发导致QT间期过冲。瓦尔萨尔瓦（Valsalva）动作导致QT和TPE间隔的波动过大，部分解释了LQT1患者突然发生自主神经活动期间发生的心脏事件。

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