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首页> 外文期刊>Archives of cardiovascular diseases. Supplements >Congenital long QX syndrome, catecholaminergic VT, Brugada syndrome and unexplained sudden cardiac death in paediatric population
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Congenital long QX syndrome, catecholaminergic VT, Brugada syndrome and unexplained sudden cardiac death in paediatric population

机译：小儿先天性长QX综合征，儿茶酚胺能性室速，Brugada综合征和无法解释的心源性猝死

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摘要

Sudden death in childhood is rare, representing only 10 % of paediatric mortality after the age of 1 year. The individual risk is estimated between 1 in 20 000 and 1 in 50 000 per year. In case of a negative autopsy for cardiac morphological anomalies, the most likely cause is a genetically determined malignant primary ventricular arrhythmia. Rhythmic sudden cardiac death can be categorized as a complication of a cardiomyopathy (dilated or hypertrophic) or as a primary channelopathy without any structural heart disease. Primary ventricular arrhythmias include long QT syndrome, Brugada syndrome, short QT syndrome and polymorphic ventricular tachycardia. The diagnosis of such syndromes relies upon specific electrocardiogram anomalies, personal history of family members, eventual echocardiography and drug challenge. Morbid genes have been identified for some of these diseases, thus rendering possible the management of presymptomatic or undiagnosed family members within specialized multidisciplinary teams. In cases of sudden arrhythmic death in children, the parents and siblings must be examined. Rescued sudden death exposes the patient to a high risk of recurrence. In such patients, the automatic implantable defibrillator has dramatically improved survival.
机译：儿童期猝死很少见，仅占1岁以后小儿死亡率的10％。据估计，每年的个人风险在2万分之一到5万分之一之间。如果心脏形态异常的尸检结果为阴性，则最可能的原因是遗传确定的恶性原发性室性心律失常。心律失常性猝死可归类为心肌病（扩张性或肥厚性）的并发症或无任何结构性心脏病的原发性通道病。原发性室性心律失常包括长QT综合征，Brugada综合征，短QT综合征和多形性室性心动过速。此类综合征的诊断取决于特定的心电图异常，家庭成员的个人病史，最终的超声心动图和药物挑战。已经为其中一些疾病鉴定了病态基因，从而使在专门的多学科团队中管理症状前或未经诊断的家庭成员成为可能。如果儿童突然出现心律失常性死亡，则必须检查父母和兄弟姐妹。被抢救的猝死使患者极有可能复发。在这类患者中，自动植入式除颤器可显着提高生存率。

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