Short QT Syndrome Manifesting with Neonatal Atrial Fibrillation and Bradycardia
Short QT Syndrome Manifesting with Neonatal Atrial Fibrillation and Bradycardia
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DOI：
10.1159/000360758
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发表时间：
2014-01-01
期刊：
影响因子：
1.9
通讯作者：
Gebauer, Roman
中科院分区：
文献类型：
--
作者：
Villafane, Juan;Fischbach, Peter;Gebauer, Roman
目的：心房颤动（AF）在儿童时期是罕见的，通常与其他心血管疾病有关。在孤立性房颤中，心室反应率通常很快。我们试图描述一个子集的儿童谁目前与早发性房颤和心室反应率慢谁被发现有短QT综合征（SQTS）。方法：使用MEDLINE/PubMed检索，确定患有AF、结构正常心脏和心动过缓的儿童。收集每个儿童的人口统计学资料、临床表现、心电图（ECG）结果、电生理检查、遗传分析和随访评估进行分析。结果：在文献中确定了4名儿童，并与作者随访的另外2名儿童合并。所有人的QT间期都很短，而那些接受测试的人被发现在KCNQ 1基因中有一个功能获得性突变。结论：我们描述了一个亚类的儿童SQTS谁提出了AF和缓慢的心室反应。药物治疗对维持窦性心律无效。这些儿童的长期结局仍然未知。这种情况可能在子宫内表现为持续性心动过缓，出生后ECG显示QT间期非常短。(C)2014 S. Karger AG，巴塞尔
Objectives: Atrial fibrillation (AF) is rare during childhood and usually associated with other cardiovascular pathology. In lone AF, the ventricular response rate is usually rapid. We sought to describe a subset of children who present with early-onset AF and a slow ventricular response rate who were found to have the short QT syndrome (SQTS). Methods: Using a MEDLINE/PubMed search, children with AF, a structurally normal heart and bradycardia were identified. Demo-graphics, clinical presentation, electrocardiographic ( ECG) findings, electrophysiologic testing, genetic analysis and follow-up assessment were collected on each child for analysis. Results: Four children were identified in the literature and combined with 2 other children followed by the authors. All had a short QT interval and those who were tested were found to have a gain-of-function mutation in the KCNQ1 gene. Conclusions: We describe a subclass of children with SQTS who present with AF and a slow ventricular response. Medical therapy has not been effective in maintaining sinus rhythm. The long-term outcome remains unknown for these children. This condition may present in utero as persistent bradycardia with postnatal ECG showing a very short QT interval. (C) 2014 S. Karger AG, Basel