The prevalence and diagnostic/prognostic utility of sinus arrhythmia in the evaluation of congenital long QT syndrome.
The prevalence and diagnostic/prognostic utility of sinus arrhythmia in the evaluation of congenital long QT syndrome.
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DOI：
10.1016/j.hrthm.2010.07.030
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发表时间：
2010-12
期刊：
影响因子：
5.5
通讯作者：
Ackerman, Michael J.
中科院分区：
文献类型：
--
作者：
Johnson, Jonathan N.;Ackerman, Michael J.
先天性长QT综合征（LQTS）每2,500人中就有1人发病，可导致晕厥和猝死。窦性心律失常（SA）是RR间期的非病理性基线呼吸变化。本研究旨在确定SA的频率及其在LQTS患者中的临床意义。我们对2000年7月至2008年3月在我们的LQTS诊所评估的所有患者（N = 571）进行了机构审查委员会批准的回顾性审查，诊断为LQTS（N = 281）或因其他原因正常而被排除（N = 290）。在对诊断设盲的情况下，检查每位患者的第一个可用心电图以定量RR间期变异性。总体而言，281例LQTS患者中的151例（54%）（159名女性患者，平均年龄21.8 ± 16.5岁，平均QTc 466 ± 43 ms）患有SA，平均RR变异性为13% ± 8%，而290名患者中有201名（69%）被视为正常（178例女性患者，平均年龄21.7 ± 16岁，平均QTc 424 ± 30 ms），显示SA，RR变异性为16% ± 10%（P < .0001）。当排除同时接受β受体阻滞剂治疗的患者时，这些差异仍然显著（P <0.001）。SA在LQT 3中最少（23%），而LQT 1（61%，P <0.005）和LQT 2（51%，P = 0.055）。尖端扭转型室性心动过速或心脏骤停中止的患者RR变异性较低（10% ± 7%，P <0.03）。与其他方面正常的患者相比，LQTS患者的SA频率和RR变异幅度较低。当排除接受β受体阻滞剂治疗的患者时，RR间期变异性的这种衰减仍然存在。尽管由于队列重叠，窦性心律失常的存在/不存在几乎没有诊断价值，但RR间期变异可忽略的LQTS患者可能处于更高的风险中。
Congenital long QT syndrome (LQTS) affects 1 in 2,500 people and can cause syncope and sudden death. Sinus arrhythmia (SA) is nonpathologic baseline respiratory variation of the RR interval. This study sought to determine the frequency of SA and its clinical significance among patients with LQTS. We performed an institutional review board–approved retrospective review of all patients (N = 571) evaluated in our LQTS clinic from 7/2000 to 3/2008 diagnosed with LQTS (N = 281) or dismissed as otherwise normal (N = 290). Blinded to diagnosis, the first available electrocardiogram for each patient was examined to quantitate RR interval variability. Overall, 151 of 281 patients (54%) with LQTS (159 female patients, average age 21.8 ± 16.5 years, average QTc 466 ± 43 ms) had SA with an average RR variability of 13% ± 8% compared with 201 of 290 (69%) patients dismissed as normal (178 female patients, average age 21.7 ± 16 years, average QTc 424 ± 30 ms) who demonstrated SA with RR variability of 16% ± 10% (P < .0001). These differences remained significant when patients on concurrent beta-blocker therapy were excluded (P < .001). SA was least common in LQT3 (23%) compared with LQT1 (61%, P < .005) and LQT2 (51%, P = .055). Patients presenting with torsades de pointes or aborted cardiac arrest had lower RR variability (10% ± 7%, P < .03). SA frequency and magnitude of RR variability was lower among patients with LQTS compared with those patients dismissed as otherwise normal. This attenuation in RR interval variability remained when patients on beta-blocker therapy were excluded. Although the presence/absence of sinus arrhythmia is of little diagnostic value due to cohort overlap, LQTS patients with negligible RR interval variation may be at higher risk.
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DOI：
10.1073/pnas.0710527105
发表时间：
2007-12-26
影响因子：
11.1
作者：
Chen, Lei;Marquardt, Michelle L.;Kass, Robert S.
通讯作者：
Kass, Robert S.
影响因子：
24
作者：
Buxton, Alfred E.;Calkins, Hugh;Redberg, Rita F.
通讯作者：
Redberg, Rita F.
影响因子：
3.3
作者：
BERNARDI, L;KELLER, F;PINSKY, MR
通讯作者：
PINSKY, MR
影响因子：
4.8
作者：
HAYANO, J;YAMADA, A;TAKATA, K
通讯作者：
TAKATA, K
影响因子：
37.8
作者：
Singh, JP;Larson, MG;Levy, D
通讯作者：
Levy, D