Cherian Anne George, Lankala Pramitha, Krupa Jesu, Roshan John
Department of Community Health, Christian Medical College and Hospital, Vellore, India.
Department of Community Health, Christian Medical College and Hospital, Vellore, India.
Indian Pacing Electrophysiol J. 2021 May-Jun;21(3):186-190. doi: 10.1016/j.ipej.2021.03.001. Epub 2021 Mar 24.
Congenital long QT syndrome [LQTS] is a channelopathy characterized by QT prolongation and polymorphic VT. LQTS however need not be a purely electrical disease. Defects in ion channels may cause myocardial architectural disruption leading to ventricular non compaction [VNC]. It is defined as the presence of prominent ventricular trabeculations and deep intertrabecular recesses within the endomyocardium. We describe the in-utero management of a foetus who was later found to have LQTS with VNC. The detection of ventricular tachycardia and complete heart block in utero should arouse the suspicion of LQTS. It would be wise to avoid QT prolonging antiarrhythmics in this subset of patients.
先天性长QT综合征[LQTS]是一种以QT间期延长和多形性室性心动过速为特征的离子通道病。然而，LQTS不一定是纯粹的电学疾病。离子通道缺陷可能导致心肌结构破坏，进而导致心室肌致密化不全[VNC]。它被定义为心内膜内存在突出的心室小梁和深陷的小梁间隐窝。我们描述了一名胎儿的宫内管理情况，该胎儿后来被发现患有LQTS合并VNC。宫内检测到室性心动过速和完全性心脏传导阻滞应引起对LQTS的怀疑。对这部分患者避免使用可延长QT间期的抗心律失常药物是明智的。
