Congenital long QT syndrome and 2:1 atrioventricular block: An optimistic outcome in the current era
Congenital long QT syndrome and 2:1 atrioventricular block: An optimistic outcome in the current era
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DOI：
10.1016/j.hrthm.2010.02.035
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发表时间：
2010-06-01
期刊：
影响因子：
5.5
通讯作者：
Shah, Maully J.
中科院分区：
文献类型：
--
作者：
Aziz, Peter F.;Tanel, Ronn E.;Shah, Maully J.
背景以前的研究与长QT综合征（LQTS）和2：1房室传导阻滞（AVB）患者的死亡率大于50%during infantation.Objective本研究的目的是要确定在当前时代的结果，这一高风险的人口从2000年1月至2009年1月，从四个三级保健儿科中心的回顾性研究评估先天性LQTS和2：1 AVB患者。所有出现2：1 AVB和与药物无关的QTc延长的新生儿均纳入研究。使用配对t检验进行统计分析。我们回顾了心电图结果、基因型、药物和器械治疗的病历。所有患者均在出生后24小时内接受诊断性ECG检查。治疗前的平均QTc间期为616 ± 99 ms（范围531-840 ms）。在71 +/- 45个月（范围15-158个月）的随访期内，12例患者中有11例接受了器械（8例永久性起搏器，3例植入式心律转复除颤器）。器械置入的平均年龄为48个月（中位数2个月，范围3天至10.5岁）。所有患者均接受β受体阻滞剂治疗; 3例患者加用美西律，1例患者加用美西律和氟卡尼。3例（25%）患者在接受β受体阻滞剂治疗时发生尖端扭转型室性心动过速，其中1例药物治疗无效。该患者接受了左心交感神经去神经和植入式心律转复除颤器放置。6例（50%）患者（2例SCN 5A突变，4例KCNH 2突变）的基因分型可用。末次随访时，未观察到死亡。随访QTc间期缩短（平均480 ± 20 ms，范围450-507 ms，P <0.002）结论LQTS和2：1 AVB患者的管理面临独特的挑战。尽管历史数据表明预后不良，但我们的研究代表了一组具有相对乐观结局的高风险LQTS患者。这一发现反映了早期诊断和干预，再加上改进的管理策略，在当今时代。
BACKGROUND Previous studies of patients with long QT syndrome (LQTS) and 2:1 atrioventricular block (AVB) have reported a mortality rate greater than 50% during infancy.OBJECTIVE The purpose of this study was to determine the outcome of this high-risk population in the current era.METHODS A retrospective study from four tertiary care pediatric centers assessed patients with congenital LQTS and 2:1 AVB from January 2000 to January 2009. All neonates who presented with 2: 1 AVB and prolonged QTc unrelated to medication were included in the study. Statistical analysis was performed using a paired t-test. Medical records were reviewed for ECG findings, genotype, medications, and device therapy.RESULTS Twelve patients that met the inclusion criteria were identified. All patients underwent diagnostic ECG in the first 24 hours of life. The average QTc interval prior to therapy was 616 +/- 99 ms (range 531-840 ms). Over a follow-up period of 71 +/- 45 months (range 15-158 months), 11 of 12 patients received devices (8 permanent pacemaker, 3 implantable cardioverter-defibrillator). Average age of device placement was 48 months (median 2 months, range 3 days to 10.5 years). All patients were treated with beta-blockers; mexiletine was added in three patients, and mexiletine and flecainide were added in one patient. Three (25%) patients experienced torsades de pointes while receiving beta-blockers, one of which was refractory to medical therapy. This patient underwent left cardiac sympathetic denervation and implantable cardioverter-defibrillator placement. Genotyping was available for 6 (50%) patients (2 SCN5A mutation, 4 KCNH2 mutation). At last follow-up, no mortality was observed. Follow-up QTc intervals had decreased (mean 480 +/- 20 ms, range 450-507 ms, P < .002)CONCLUSION Management of patients with LQTS and 2:1 AVB presents unique challenges. Despite historical data indicating poor prognosis, our study represents a cohort of high-risk LQTS patients with a relatively optimistic outcome. This finding reflects early diagnosis and intervention, coupled with improved management strategies, in the current era.