Clinical aspects and prognosis of Brugada syndrome in children
Clinical aspects and prognosis of Brugada syndrome in children
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DOI：
10.1161/circulationaha.106.664219
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发表时间：
2007-04-17
期刊：
影响因子：
37.8
通讯作者：
Wilde, Arthur A. M.
中科院分区：
文献类型：
--
作者：
Probst, Vincent;Denjoy, Isabelle;Wilde, Arthur A. M.
背景-Brugada 综合征是一种致心律失常疾病，其特征是心电图右心前导联 ST 段抬高，心源性猝死的风险增加。关于这种疾病在儿童中的临床表现和预后知之甚少。方法和结果-纳入了 30 名年龄 < 16 岁（平均 8 +/- 4 岁）的 Brugada 综合征儿童。所有患者在药物激发挑战之前或之后均表现出 I 型心电图模式。 Brugada综合征的诊断是在以下情况下进行的：流产性猝死（n=1）、不明原因晕厥（n=10）、症状性室上性心动过速（n=1）、可疑心电图（n=1）、家庭筛查Brugada综合征（n=17）。发热诱发晕厥5例。 11 名有症状的患者中有 10 名显示自发 I 型心电图。 5 名儿童植入了植入式心脏复律除颤器； 4名儿童接受氢奎尼丁治疗； 1 名儿童因有症状的病态窦房结综合征而接受了起搏器治疗。在平均 37 +/- 23 个月的随访期间，1 名儿童出现心源性猝死，2 名儿童接受了适当的植入式心脏复律除颤器电击；他们全部都有症状，并自发出现 I 型心电图。一名儿童患有心脏复律除颤器感染，需要拔除除颤器。 结论 - 在迄今为止描述的最大的受 Brugada 综合征影响的儿童群体中，发烧是心律失常事件最重要的诱发因素，并且与成年人一样，先前有症状的患者和显示自发 I 型心电图的患者发生心律失常事件的风险较高。
Background-Brugada syndrome is an arrhythmogenic disease characterized by an ECG pattern of ST-segment elevation in the right precordial leads and augmented risk of sudden cardiac death. Little is known about the clinical presentation and prognosis of this disease in children.Methods and Results-Thirty children affected by Brugada syndrome who were < 16 years of age ( mean, 8 +/- 4 years) were included. All patients displayed a type I ECG pattern before or after drug provocation challenge. Diagnosis of Brugada syndrome was made under the following circumstances: aborted sudden death ( n=1), syncope of unexplained origin ( n=10), symptomatic supraventricular tachycardia ( n=1), suspicious ECG ( n=1), and family screening for Brugada syndrome ( n=17). Syncope was precipitated by fever in 5 cases. Ten of 11 symptomatic patients displayed a spontaneous type I ECG. An implantable cardioverter-defibrillator was implanted in 5 children; 4 children were treated with hydroquinidine; and 1 child received a pacemaker because of symptomatic sick sinus syndrome. During a mean follow-up of 37 +/- 23 months, 1 child experienced sudden cardiac death, and 2 children received an appropriate implantable cardioverter-defibrillator shock; all of them were symptomatic and had manifested a type I ECG spontaneously. One child had a cardioverter-defibrillator infection that required explantation of the defibrillator.Conclusions-In the largest population of children affected by Brugada syndrome described to date, fever represented the most important precipitating factor for arrhythmic events, and as in the adult population, the risk of arrhythmic events was higher in previously symptomatic patients and in those displaying a spontaneous type I ECG.