Wacker-Gussmann Annette, Eckstein Gretchen K, Strasburger Janette F
Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich, 80636 Munich, Germany.
Division of Cardiology, Departments of Pediatrics and Biomedical Engineering, Children's Wisconsin, Herma Heart Institute, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
J Clin Med. 2023 May 10;12(10):3379. doi: 10.3390/jcm12103379.
The number of women of childbearing age who have been diagnosed in childhood with ion channelopathy and effectively treated using beta blockers, cardiac sympathectomy, and life-saving cardiac pacemakers/defibrillators is increasing. Since many of these diseases are inherited as autosomal dominant, offspring have about a 50% risk of having the disease, though many will be only mildly impacted during fetal life. However, highly complex delivery room preparation is increasingly needed in pregnancies with inherited arrhythmia syndromes (IASs). However, specific Doppler techniques show meanwhile a better understanding of fetal electrophysiology. The advent of fetal magnetocardiography (FMCG) now allows the detection of fetal Torsades de Pointes (TdP) ventricular tachycardia and other LQT-associated arrhythmias (QTc prolongation, functional second AV block, T-wave alternans, sinus bradycardia, late-coupled ventricular ectopy and monomorphic VT) in susceptible fetuses during the second and third trimester. These types of arrhythmias can be due to either de novo or familial Long QT Syndrome (LQTS), Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT), or other IAS. It is imperative that the multiple specialists involved in the antenatal, peripartum, and neonatal care of these women and their fetuses/infants have the optimal knowledge, training and equipment in order to care for these highly specialized pregnancies and deliveries. In this review, we outline the steps to recognize symptomatic LQTS in either the mother, fetus or both, along with suggestions for evaluation and management of the pregnancy, delivery, or post-partum period impacted by LQTS.
童年期被诊断患有离子通道病并使用β受体阻滞剂、心脏交感神经切除术和挽救生命的心脏起搏器/除颤器进行有效治疗的育龄妇女数量正在增加。由于其中许多疾病是常染色体显性遗传，后代患该病的风险约为50%，不过许多人在胎儿期只会受到轻微影响。然而，对于患有遗传性心律失常综合征（IAS）的孕妇，越来越需要进行高度复杂的产房准备。与此同时，特定的多普勒技术显示出对胎儿电生理学有了更好的理解。胎儿心电图描记术（FMCG）的出现，现在能够在妊娠中期和晚期检测易感胎儿的尖端扭转型室性心动过速（TdP）和其他与长QT综合征相关的心律失常（QTc延长、功能性二度房室传导阻滞、T波交替、窦性心动过缓、晚发室性早搏和单形性室性心动过速）。这些类型的心律失常可能是由于新发或家族性长QT综合征（LQTS）、儿茶酚胺能多形性室性心动过速（CPVT）或其他IAS。至关重要的是，参与这些妇女及其胎儿/婴儿产前、围产期和新生儿护理的多名专家要具备最佳的知识、培训和设备，以便照料这些高度专业化的妊娠和分娩。在这篇综述中，我们概述了识别母亲、胎儿或两者中症状性LQTS的步骤，以及对受LQTS影响的妊娠、分娩或产后时期进行评估和管理的建议。
