Rad Elaheh Malakan, Ilali Hamidreza Mirzaei, Majnoon Mohammad-Taghi, Zeinaloo Aliakbar
Children's Medical Center (Pediatric Center of Excellence), Tehran University of Medical Sciences, End of Keshavarz Boulevard, Tehran, Iran.
Ann Pediatr Cardiol. 2022 Jul-Aug;15(4):364-373. doi: 10.4103/apc.apc_169_21. Epub 2023 Jan 6.
Congenital long QT syndrome (CLQTS) is a life-threatening ion channelopathy leading to syncope and sudden death. Early diagnosis during the prenatal period and timely intervention can prevent sudden cardiac death and catastrophic consequences of this genetic ion channelopathy. Fetal magnetocardiography and fetal electrocardiography (ECG) enable the measurement of fetal QT and JT intervals, but their inherently technically challenging and/or resource-intensiveness nature preclude their routine clinical application. On the other hand, the high-temporal resolution of M-mode echocardiography makes it a well-suited and widely available modality for the measurement of cardiac events.
We aimed to investigate the mechanical counterparts of the electrical QT and JT intervals on M-mode echocardiographic images of the tricuspid, mitral and aortic valves, and aortic wall.
We performed a prospective study on consecutive children referred to the outpatient pediatric cardiology clinic at a tertiary children's hospital. We defined M-mode echocardiographic landmark points on tracings of tricuspid annular planar systolic excursion, mitral and aortic valves, and aortic wall with simultaneous electrocardiographic recording. We measured the mean±SD of the absolute time difference and RR-adjusted time difference in cases with non-coincident ECG events and echocardiographic landmarks.
Fifty healthy children were enrolled in the study. In 47 (94%) out of the 50 children, Q was coincident with the starting point of the tricuspid annular plane systolic excursion. In all children, the Q was coincident with the mid-point of the A-C line of the mitral valve. In 38 (76%) cases, there was a bump on the anterior wall of the aortic root immediately before the change in the slope of the aortic wall. This was coincident with the Q wave in 100% of cases. In all cases, the J point coincided with the point of acceleration of velocity on TAPSE. In all children, the J point coincided with the initial maximal opening of the aortic cusps. The end of the T wave occurred coincident with the peak of the tricuspid annular planar systolic excursion in 47 children (94%). In 48 children (96%), the end of the T wave coincided with the aortic cusps' closure point.
Based on our findings, we propose to measure the averaged mechanical QT and JT intervals by using an angled M-mode tracing of the aortic and mitral valve in five consecutive beats in the parasternal long-axis view. This is the first study on mechanical QT and JT intervals in healthy children. The study opens the horizons into the in-utero diagnosis of congenital long QT syndrome by measuring fetal QT and JT intervals using the widely available M-mode echocardiography.
先天性长QT综合征(CLQTS)是一种危及生命的离子通道病,可导致晕厥和猝死。产前早期诊断并及时干预可预防这种遗传性离子通道病导致的心源性猝死和灾难性后果。胎儿心磁图和胎儿心电图(ECG)能够测量胎儿QT和JT间期,但因其在技术上具有内在挑战性和/或资源密集性,妨碍了它们在临床中的常规应用。另一方面,M型超声心动图的高时间分辨率使其成为测量心脏事件的合适且广泛可用的检查方法。
我们旨在研究三尖瓣、二尖瓣和主动脉瓣以及主动脉壁的M型超声心动图图像上QT和JT电间期的机械对应物。
我们对一家三级儿童医院门诊儿科心脏病诊所转诊的连续儿童进行了一项前瞻性研究。我们在三尖瓣环平面收缩期位移、二尖瓣和主动脉瓣以及主动脉壁的描记图上定义M型超声心动图界标点,并同时记录心电图。我们测量了心电图事件与超声心动图界标点不一致的病例中绝对时间差和RR校正时间差的平均值±标准差。
50名健康儿童纳入研究。50名儿童中有47名(94%)Q点与三尖瓣环平面收缩期位移的起始点一致。所有儿童中,Q点与二尖瓣A-C线的中点一致。38例(76%)病例中,主动脉壁斜率改变前主动脉根部前壁有一个凸起。100%的病例中该凸起与Q波一致。所有病例中,J点与三尖瓣环平面收缩期位移的速度加速点一致。所有儿童中,J点与主动脉瓣叶的初始最大开口一致。47名儿童(94%)中T波终点与三尖瓣环平面收缩期位移峰值一致。48名儿童(96%)中T波终点与主动脉瓣叶关闭点一致。
基于我们的研究结果,我们建议在胸骨旁长轴视图中连续五个心动周期使用主动脉瓣和二尖瓣的倾斜M型描记来测量平均机械QT和JT间期。这是第一项关于健康儿童机械QT和JT间期的研究。该研究通过使用广泛可用的M型超声心动图测量胎儿QT和JT间期,为先天性长QT综合征的宫内诊断开辟了新视野。