儿童第三度房室传导阻滞

V. Vrdoljak, M. Šapina, Suzana Bitanga, M. Katavić
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摘要

房室传导阻滞是指由于传导系统的解剖或功能损伤,导致从心房到心室的脉冲传导延迟或中断。传导扰动可以是短暂的,也可以是永久的。在第三度房室传导阻滞,也被称为完全心脏传导阻滞,心房和心室活动完全分离。当房室传导阻滞自发发生在胎儿或幼儿时,被认为是“先天性的”。在儿童中,永久性获得性完全房室传导阻滞最常见的原因是先天性心脏病的手术。总的来说,60%到90%的先天性CHB病例是由经胎盘暴露于与系统性红斑狼疮或干燥综合征相关的母体自身抗体引起的胎儿传导组织损伤1-3。多达40%的先天性CHB病例直到儿童晚期才出现(平均年龄5 - 6岁)。这些患者很少(5%)有自身免疫性病因。猝死风险的增加与深度心动过缓或室性心律失常的发生有关。常规心电图足以诊断这种疾病。一名15岁女孩在" Sestre Milosrdnice "大学医院中心儿科急诊门诊接受了反复发作的晕厥前症检查。体格检查显示除了心动过缓外没有其他主要的变化。除了每分钟约44次的脉搏外,她的生命体征都在参考范围内。心电图显示房室分离,符合三度房室传导阻滞。超声心动图显示心脏除窦性心动过缓外结构正常。这名女孩接受了永久性心外膜起搏器植入,之后没有任何症状。
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Third Degree Atrioventricular Block in Children
Atrioventricular (AV) block is defined as a delay or interruption in the transmission of an impulse from the atria to the ventricles due to an anatomical or functional impairment in the conduction system. The conduction disturbance can be transient or permanent. In third degree AV block, also referred to as complete heart block, there is complete dissociation of the atrial and ventricular activity. Atrioventricular block is considered to be “congenital” when it occurs spontaneously in a fetus or young child. In children, the most common cause of permanent acquired complete AV block is surgery for congenital heart disease. Injury to fetal conduction tissues caused by transplacental exposure to maternal autoantibodies related to systemic lupus erythematosus or Sjogren’s syndrome is responsible for 60 to 90 percent of cases of congenital CHB overall1-3. As many as 40 percent of cases of congenital CHB do not present until later in childhood (mean age five to six years). Only rarely do these patients (5 percent) have proven autoimmune etiology. The increased risk of sudden death is associated with the onset of deep bradycardia or ventricular arrhythmia. A routine electrocardiogram is sufficient to diagnose the disease. A 15-year-old girl has been examined at the emergency pediatric outpatient clinic of the University Hospital Centre “Sestre Milosrdnice” for recurrent episodes of presyncope. Physical examination revealed no major deviations other than bradycardia. Her vital signs were within the reference range, with the exception of a pulse of about 44 beats per minute. The electrocardiogram showed atrioventricular dissociation consistent with third degree atrioventricular block. The echocardiogram showed a structurally normal heart except for sinus bradycardia. The girl underwent permanent epicardial pacemaker implantation after which there were no symptoms.
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