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3型长QT综合征无症状儿童运动期间的QT动态变化

QT Dynamics During Exercise in Asymptomatic Children with Long QT Syndrome Type 3.

作者信息

Takahashi Kazuhiro, Nabeshima Taisuke, Nakayashiro Mami, Ganaha Hitoshi

机构信息

Department of Pediatric Cardiology, Okinawa Children's Medical Center, 118-1 Arakawa, Haebaru-chou, Okinawa, 901-1193, Japan.

出版信息

Pediatr Cardiol. 2016 Jun;37(5):860-7. doi: 10.1007/s00246-016-1360-4. Epub 2016 Feb 26.

Abstract

Sympathetic provocative testing is commonly used to detect the abnormal QT dynamics in long QT syndrome (LQTS) patients, particularly LQTS type 1 and type 2. However, little is known about LQTS type 3 (LQT3). We investigated QT dynamics during exercise testing in LQTS patients, particularly LQT3. This study included 37 subjects, comprising 16 genotyped LQTS patients and 21 unrelated healthy subjects without QT prolongation. LQTS patients were divided into LQT3 and non-LQT3 groups. During exercise tests using a modified Bruce protocol, 12-lead electrocardiogram monitoring was performed using a novel multifunctional electrocardiograph. QT intervals were automatically measured. The QT/heart rate (HR) relationship was visualized by plotting the beat-to-beat confluence of the recorded data. A linear regression analysis was performed to determine the QT/HR slope and intercept. Estimated QT intervals at HR 60 bpm (QT60) were calculated by the regression line formula. QT/HR slopes were steeper for each LQTS group than for the control group (P < 0.001). QT60 values demonstrated a moderate correlation with QT intervals at rest (P < 0.0001) for both groups. The corrected QT intervals (QTc) at 4 min of recovery after exercise were significantly longer in the non-LQT3 group than in the control group but were not different between the LQT3 and the control groups. Abnormal QT dynamics during exercise testing were observed in both LQT3 patients and other LQTS subtypes. This method may be useful for directing genetic testing in subjects with borderline prolonged QT intervals.

摘要

交感神经激发试验常用于检测长QT综合征(LQTS)患者,尤其是1型和2型LQTS患者的异常QT动态变化。然而,对于3型LQTS(LQT3)知之甚少。我们研究了LQTS患者,特别是LQT3患者运动试验期间的QT动态变化。本研究纳入了37名受试者,包括16名基因分型的LQTS患者和21名无QT间期延长的无关健康受试者。LQTS患者分为LQT3组和非LQT3组。在使用改良布鲁斯方案进行运动试验期间,使用新型多功能心电图仪进行12导联心电图监测。QT间期自动测量。通过绘制记录数据的逐搏融合情况来直观显示QT/心率(HR)关系。进行线性回归分析以确定QT/HR斜率和截距。通过回归线公式计算心率为60次/分钟时的估计QT间期(QT60)。各LQTS组的QT/HR斜率均比对照组更陡(P < 0.001)。两组的QT60值与静息时的QT间期均呈中度相关(P < 0.0001)。运动后恢复4分钟时,非LQT3组的校正QT间期(QTc)显著长于对照组,但LQT3组与对照组之间无差异。LQT3患者和其他LQTS亚型在运动试验期间均观察到异常QT动态变化。该方法可能有助于指导QT间期临界延长受试者的基因检测。

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