CATECHOLAMINERGIC POLYMORPHIC VENTRICULAR-TACHYCARDIA IN CHILDREN - A 7-YEAR FOLLOW-UP OF 21 PATIENTS
CATECHOLAMINERGIC POLYMORPHIC VENTRICULAR-TACHYCARDIA IN CHILDREN - A 7-YEAR FOLLOW-UP OF 21 PATIENTS
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DOI：
10.1161/01.cir.91.5.1512
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发表时间：
1995-03-01
期刊：
影响因子：
37.8
通讯作者：
COUMEL, P
中科院分区：
文献类型：
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作者：
LEENHARDT, A;LUCET, V;COUMEL, P
背景原发性室性快速性心律失常在儿童中很少见。其中，儿茶酚胺能多形性室性心动过速的自发性结局较差。它的诊断往往是延迟后的第一个症状，这是不可接受的，因为治疗与适当的β-受体阻滞剂防止猝死。方法和结果我们观察了21名儿童（平均+/-SD年龄，9.9+/-4岁）的诊断时，谁没有结构性心脏病和正常的QT间期的常规心电图。他们因与室性多形性快速性心律失常相关的应激或情绪诱发的晕厥而转诊。心律失常，包括孤立的多态性室性期外收缩，其次是易变性为室颤的双向和多态性心动过速的齐射阵发，可重复诱导任何形式的增加肾上腺素能刺激。30%的患者有晕厥或猝死的家族史。接受适当的β受体阻滞剂治疗后，患者的症状和多形性快速性心律失常消失。在平均随访7年期间，三晕厥事件和两个突然死亡发生，可能是由于治疗interrupt.Conclusions肾上腺素依赖性，潜在致命的快速性心律失常的实体，没有结构性心脏病值得个性化。它可能形成先天性长QT综合征的一种变体，其中缺乏ECG标记物;这种原发性室性心律失常必须在患有应激或情绪诱导的晕厥的儿科患者中寻找，因为只有β受体阻滞剂治疗可以预防猝死，因此必须在患者的一生中给予。
Background Primary ventricular tachyarrhythmias are rarely seen in children. Among them, catecholaminergic polymorphic ventricular tachycardia has a poor spontaneous outcome. Its diagnosis is often delayed after the first symptoms, which is unacceptable because treatment with the appropriate beta-blocker prevents sudden death.Methods and Results We observed 21 children (mean+/-SD age, 9.9+/-4 years) at the time of the diagnosis who had no structural heart disease and a normal QT interval on routine EGG. They were referred for stress- or emotion-induced syncope related to ventricular polymorphic tachyarrhythmias. The arrhythmia, consisting of isolated polymorphic ventricular extrasystoles followed by salvoes of bidirectional and polymorphic tachycardia susceptible to degeneration into ventricular fibrillation, was reproducibly induced by any form of increasing adrenergic stimulation. There was a familial history of syncope or sudden death in 30% of our patients. On receiving therapy with the appropriate beta-blocker, the patients' symptoms and polymorphic tachyarrhythmias disappeared. During a mean follow-up period of 7 years, three syncopal events and two sudden deaths occurred, probably due to treatment interruption.Conclusions The entity of adrenergic-dependent, potentially lethal tachyarrhythmia with no structural heart disease deserves to be individualized. It may form a variant of the congenital long QT syndrome in which the ECG marker is lacking; this primary ventricular arrhythmia must be looked for in a pediatric patient with stress- or emotion-induced syncope because only beta-blocking therapy can prevent sudden death and therefore must be given for the patient's lifetime.