背景:由于其可用性,阿替洛尔是澳大利亚用于长QT综合征儿童的主要β受体阻滞剂。关于其使用的长期随访数据有限。
方法:单三级中心,回顾性,我们进行了一项观察性研究,调查所有经基因证实患有长QT综合征1型(LQT1)和2型(LQT2)的儿童和青少年.对他们的预处理运动测试进行了QTc间隔评估,以进入运动恢复阶段。
结果:从2004年到2014年,共确定了86例患者(LQT1,67和LQT2,19)。大多数(86%)的患者最初被转诊为家庭筛查。阿替洛尔的平均剂量为1.58±0.51mg/kg/天。在4.29年的中位随访期间,只有一个先证者在服用阿替洛尔时出现室性心律失常,没有患者有心脏骤停或心脏骤停中断。关于阿替洛尔的副作用,只有2例患者出现无法耐受的副作用,需要更换药物治疗.运动测试(预处理)的评估表明,LQT1患者在运动恢复期2-3分钟的校正QT(QTc)间隔显着延长。有跨膜突变的LQT1患者的QTc间期比他们的C端突变对应物更长,在运动恢复阶段3分钟达到统计学意义。
结论:阿替洛尔是经基因证实的LQT1和LQT2儿童和青少年的有效治疗方法,具有良好的耐受性。在LQT1患者中,运动恢复期2-3分钟的QTc间隔明显延长,特别是跨膜突变患者。
BACKGROUND: Due to its availability, atenolol is the primary beta-blocker used in Australia for children with long QT syndrome. There is limited data on long-term follow-up of its use.
METHODS: A single-tertiary-center, retrospective, observational study investigating all children and adolescents who had genetically proven long QT syndrome type 1 (LQT1) and type 2 (LQT2) was conducted. Their pretreatment exercise tests were evaluated for QTc intervals into the recovery phase of exercise.
RESULTS: Eighty six patients were identified (LQT1, 67, and LQT2, 19) from 2004 to 2014. The majority (86%) of patients were initially referred for family screening. Atenolol was administered at a mean dose of 1.58 ± 0.51 mg/kg/day. During the median follow-up period of 4.29 years, only one proband developed ventricular arrhythmia whilst taking atenolol, No patient had cardiac arrest or aborted cardiac arrest. With respect to side effects of atenolol, only two patients had intolerable side effects necessitating changes of medication. Evaluation of exercise tests (pretreatment) demonstrated that corrected QT (QTc) intervals at 2-3 min into the recovery phase of exercise were significantly prolonged for LQT1 patients. LQT1 patients with transmembrane mutation had longer QTc intervals than their C-terminus mutation counterparts, reaching statistical significance at 3 min into the recovery phase of exercise.
CONCLUSIONS: Atenolol is an effective treatment for genetically proven LQT1 and LQT2 children and adolescents, with good tolerability. In LQT1 patients, QTc intervals at 2-3 min into the recovery phase of exercise were significantly prolonged, particularly in patients with transmembrane mutations.