[Long QT syndrome].
[Long QT syndrome].
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DOI：
10.1024/1661-8157/a000198
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发表时间：
2010-07-07
期刊：
影响因子：
--
通讯作者：
Skinner, J R
中科院分区：
文献类型：
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作者：
Abrams, D J;Perkin, M A;Skinner, J R
一名19岁女学生向全科医生咨询了最近两次晕厥，均发生在打曲棍球时。她的队友报告说，她突然晕倒，几乎没有任何征兆，在30秒内迅速恢复，没有任何混乱。她在其他方面都很好，尽管当时她正在服用红霉素治疗感染的腿部擦伤。作为晕厥常规检查的一部分，她的全科医生给她做了12导联心电图，结果显示校正后QT间期延长510毫秒。先天性长QT间期综合征是可避免的心源性猝死的潜在原因。受影响的个体可能有室性心律失常，导致心悸，晕厥，如果持续，心脏骤停。1该综合征以常染色体显性遗传方式遗传，具有可变的疾病表达：严重影响的患者可能在胎儿或新生儿期死亡，但其他人终生无症状。在细胞水平上，细胞膜内钠和钾离子通道的遗传编码异常延长心脏复极，表现为心电图QT间期的延长。QT间期延长可能继发于某些药物、代谢紊乱、脑损伤、心肌疾病和体温过低，这些因素也可能在先前无症状的个体中揭示先天性综合征。
A 19 year old female student consulted her general practitioner about two recent episodes of syncope, both of which occurred while playing hockey. Her team mates reported that she collapsed suddenly with little warning, recovering rapidly within 30 seconds without confusion. She was otherwise well, although she was taking erythromycin for an infected leg abrasion at the time of the events. As part of the routine evaluation for syncope, her general practitioner performed a 12 lead electrocardiogram, which showed a prolonged corrected QT interval of 510 ms.Congenital long QT syndrome is a potential cause of avoidable sudden cardiac death. Affected individuals may have ventricular arrhythmias, leading to palpitations, syncope, and, if sustained, cardiac arrest. 1 The syndrome is inherited in an autosomal dominant fashion, with variable disease expression: those severely affected may die in fetal or neonatal life, but others remain asymptomatic throughout their life. At a cellular level, genetically encoded abnormalities in sodium and potassium ion channels within the cell membrane lengthen cardiac repolarisation, which manifests as a prolongation of the QT interval in the electrocardiogram. QT prolongation may be acquired secondary to certain medications, metabolic disturbance, cerebral injury, myocardial disease, and hypothermia—factors that may also unmask the congenital syndrome in a previously asymptomatic individual.