Long QT Syndrome in Children

QT延長症候群是一種會產生昏厥，抽筋，甚至死亡的疾病。從1990年6月到1996年6月，共有11個小朋友（男/女=7:4；年齡：1天至13歲，平均：5.4歲）；均來自不同的家庭，被發現有QT延長症候群。這些兒童的矯正QT時間(QTc)是0.46-0.59秒（平均：0.53秒）。所有病童都有正常的聽力，但其中有一例是馬凡氏症合併二尖瓣脫垂。這些兒童臨床表現的症狀為：瘁死（2例），抽筋（6例）及昏厥（3例）。他們在心電圖上的異常發現包括：Torsade點（7例），實性緩脈（4例），T波異常（4例），單一相心室頻脈（2例）及先天性房室間傳導完全阻斷（1例）。所有病童都接受β型阻斷劑，而其中一例並裝置了心臟節律器。在追蹤0.5-6年的期間內；六個病童沒有再發生症狀，而兩例有再發的昏厥，而有一病童死於心室頻脈。

關鍵字

Torsade點；心室頻脈；瘁死；房室間傳導阻斷

並列摘要

Long QT syndrome is a disease that can cause syncope, seizures and sudden death. From June 1990 to June 1996, 11 children (male/female: 7/4; ages: 1 day-13 years with a median of 5.4 years) from different families were found to have long QT syndrome. Their corrected QT intervals (QTc) were 0.46-0.59 sec (median. 0.53 sec). All patients had normal hearing. One patient had Marfan syndrome with mitral valve prolapse. The presentation symptoms were: sudden death (2), seizures (6) and syncope (3). Their electrocardiogram abnormalities included: torsades de pointes (7), sinus bradycardia (4), T wave abnormalites (4), monomorphic ventricular tachycardia (2) and congenital complete atrioventricular block (1). All patients were treated with β-blockers and one had pacemaker implantation. In a follow-up period of 0.5-6 years, 6 were symptom free, 1 died of ventricular tachycardia and 2 had recurrent syncope.

並列關鍵字

torsades de pointes ； long QT syndrome ； ventricular tachycardia ； sudden death ； atrioventricular block

國際替代計量

Long QT Syndrome in Children

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