Nakayama Hiroki, Aiba Takeshi, Miyazaki Yuichiro, Oshima Yoshitake, Ueda Nobuhiko, Wakamiya Akinori, Oka Satoshi, Nakamura Toshihiro, Nakajima Kenzaburo, Kamakura Tsukasa, Wada Mitsuru, Inoue Yuko, Ishibashi Kohei, Miyamoto Koji, Nagase Satoshi, Kusano Kengo
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan.
Department of Clinical Laboratory Medicine and Genetics, National Cerebral and Cardiovascular Center, Suita, Japan.
J Cardiol Cases. 2024 Feb 2;29(5):214-217. doi: 10.1016/j.jccase.2024.01.006. eCollection 2024 May.
An implantable loop recorder (ILR) is now widely used for differential diagnosis of unexplained syncope or recurrent syncope with unknown causes. In the inherited arrhythmia syndromes, ILR may be useful for management of the therapeutic strategies; however, there is no obvious evidence to uncover arrhythmic syncope by ILR in long-QT syndrome (LQTS) patients. Here we experienced a 19-year-old female patient with LQTS type 1 who had recurrent syncope even after beta-blocker therapy but no arrhythmias were documented, and some episodes might be due to non-cardiogenic causes. Implantable cardioverter defibrillator (ICD) therapy was also recommended; however, she could not accept ICD but was implanted with ILR for further continuous monitoring. Two years later, she suffered syncope during a brief run, and ILR recorded an electrocardiogram at that moment. Thus a marked QT interval prolongation as well as T-wave alternance resulting in development of torsades de pointes could be detected. Although ILR is just a diagnostic tool but does not prevent sudden cardiac death, most arrhythmic events in LQTS are transient and sometimes hard to be diagnosed as arrhythmic syncope. ILR may provide direct supportive evidence to select the optimal therapeutic strategy in cases where syncope is difficult to diagnose.
Long-QT syndrome (LQTS) patients often suffer recurrent syncope even after beta-blocker therapy, but torsades de pointes (TdP) is not always detected by standard 12‑lead electrocardiogram or Holter monitoring, and some syncope might be non-cardiogenic. In this case, implantable loop recorder (ILR) documented the evidence of QT interval prolongation and beat-by-beat T-wave alternance subsequent TdP. Thus, ILR may provide useful evidence for the optimal treatment strategy in LQTS cases where syncope is difficult to diagnose.
植入式循环记录仪(ILR)目前广泛用于不明原因晕厥或病因不明的复发性晕厥的鉴别诊断。在遗传性心律失常综合征中,ILR可能有助于治疗策略的管理;然而,尚无明显证据表明ILR能在长QT综合征(LQTS)患者中发现心律失常性晕厥。我们在此报告一例19岁1型LQTS女性患者,即使在接受β受体阻滞剂治疗后仍有复发性晕厥,但未记录到心律失常,部分发作可能是非心源性原因所致。也曾建议植入式心脏复律除颤器(ICD)治疗;然而,她无法接受ICD,而是植入了ILR以进行进一步的持续监测。两年后,她在一次短跑时发生晕厥,ILR当时记录到一份心电图。由此检测到显著的QT间期延长以及T波交替,进而导致尖端扭转型室速。尽管ILR只是一种诊断工具,不能预防心源性猝死,但LQTS中的大多数心律失常事件是短暂的,有时难以诊断为心律失常性晕厥。在晕厥难以诊断的病例中,ILR可能为选择最佳治疗策略提供直接的支持证据。
长QT综合征(LQTS)患者即使在接受β受体阻滞剂治疗后仍常发生复发性晕厥,但标准12导联心电图或动态心电图监测并不总能检测到尖端扭转型室速(TdP),且部分晕厥可能是非心源性的。在本病例中,植入式循环记录仪(ILR)记录到QT间期延长以及随后TdP的逐搏T波交替的证据。因此,在晕厥难以诊断的LQTS病例中,ILR可能为最佳治疗策略提供有用的证据。