Krause Ulrich
Division of Pediatric Cardiology and Intensive Care Medicine, Department of Pediatric and Adolescent Medicine, University Medical Center, Georg-August-University Göttingen, Robert-Koch-Str. 40, 37099, Göttingen, Germany.
Herzschrittmacherther Elektrophysiol. 2025 Aug 5. doi: 10.1007/s00399-025-01098-2.
Cardiac channelopathies are a group of hereditary diseases that expose affected children and adolescents to an increased risk of syncope and sudden cardiac death (SCD) due to malignant ventricular tachyarrhythmias. Although cardiac channelopathies are rare, with an estimated prevalence of 1:2000-1:10,000, early recognition in order to start treatment and prevent SCD is warranted. The following article provides an overview of current recommendations and facts on the diagnosis and treatment of cardiac channelopathies in children and adolescents. The most commonly encountered cardiac channelopathies during childhood and adolescence include long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), Brugada syndrome (BrS) and short QT syndrome (SQTS). While subjects with LQTS and CPVT commonly respond well to β‑blocker medication, and flecainide is an additional option in patients with CPVT, no such highly effective drug therapy exists for the treatment of patients with BrS or SQTS. Left cardiac sympathetic denervation is an additional treatment option in subjects with LQTS or CPVT. Implantable cardioverter-defibrillator implantation is indicated in patients with channelopathies and life-threatening ventricular tachyarrhythmias despite adequate antiarrhythmic medication.
心脏离子通道病是一组遗传性疾病，会使患病的儿童和青少年因恶性室性心律失常而面临晕厥和心源性猝死（SCD）风险增加。尽管心脏离子通道病较为罕见，估计患病率为1:2000至1:10000，但仍有必要尽早识别以便开始治疗并预防心源性猝死。以下文章概述了儿童和青少年心脏离子通道病诊断和治疗的当前建议及相关事实。儿童期和青少年期最常见的心脏离子通道病包括长QT综合征（LQTS）、儿茶酚胺能多形性室性心动过速（CPVT）、Brugada综合征（BrS）和短QT综合征（SQTS）。虽然LQTS和CPVT患者通常对β受体阻滞剂药物反应良好，氟卡尼是CPVT患者的另一种选择，但对于BrS或SQTS患者的治疗，尚无如此高效的药物疗法。左侧心脏交感神经去神经支配是LQTS或CPVT患者的另一种治疗选择。对于患有离子通道病且尽管使用了足够的抗心律失常药物仍有危及生命的室性心律失常的患者，建议植入植入式心脏复律除颤器。
