Congenital long QT syndrome: Diagnosis and management in pediatric patients

被引：7

作者：

Bar-Cohen Y. ^{[1
]}

Silka M.J. ^{[1
]}

机构：

[1] Department of Pediatrics, Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, CA 90027

来源：

Current Treatment Options in Cardiovascular Medicine | 2006年 / 8卷 / 5期

关键词：

Sudden Cardiac Death; Mexiletine; Cardiac Pace; Cardiac Sympathetic Denervation; Abort Sudden Cardiac Death;

D O I：

10.1007/s11936-006-0043-5

中图分类号：

学科分类号：

摘要：

The long QT syndrome (LQTS) is characterized by electrocardiographic abnormalities and a high incidence of syncope and sudden cardiac death (SCD). The diagnosis is suggested when ventricular repolarization abnormalities result in prolongation of the corrected QT interval. When LQTS is suspected, genetic screening may identify a specific long QT subtype and provide guidance for appropriate therapy. Treatment depends on the relative risk of SCD, which is increased with longer QT durations, prior cardiac events, and a family history of SCD. β Blockers are considered the initial treatment of choice, with implantable cardioverter-defibrillator (ICD) therapy warranted in high-risk patients. In patients with frequent ICD shocks or in those at high risk for SCD where ICD placement cannot be performed, cardiac pacing and/or left cardiac sympathetic denervation may be indicated. Copyright © 2006 by Current Science Inc.

引用

页码：387 / 395

页数：8

共 38 条

[1]

Moss A.J., Long QT syndrome, JAMA, 289, pp. 2041-2044, (2003)

[2]

Shah M., Akar F.G., Tomaselli G.F., Molecular basis of arrhythmias, Circulation, 112, pp. 2517-2529, (2005)

[3]

Roden D.M., Drug-induced prolongation of the QT interval, N Engl J Med, 350, pp. 1013-1022, (2004)

[4]

Prioro S.G., Schwartz P.J., Napolitano C., Et al., Risk stratification in the long-QT syndrome, N Engl J Med, 348, pp. 1866-1874, (2003)

[5]

Goel A.K., Berger S., Pelech A., Dhala A., Implantable cardioverter defibrillator therapy in children with long QT syndrome, Pediatr Cardiol, 25, pp. 370-378, (2004)

[6]

Moss A.J., Robinson J.L., Gessman L., Et al., Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome, Am J Cardiol, 84, pp. 876-879, (1999)

[7]

Zareba W., Moss A.J., Schwartz P.J., Et al., Influence of genotype on the clinical course of the long-QT syndrome, N Engl J Med, 339, pp. 960-965, (1998)

[8]

Splawski I., Shen J., Timothy K.W., Et al., Spectrum of mutations in long-QT syndrome genes KVLQT1, HERG, SCN5A, KCNE1, and KCNE, Circulation, 102, pp. 1178-1185, (2000)

[9]

Schwartz P.J., Moss A.J., Vincent G.M., Crampton R.S., Diagnostic criteria for the long QT syndrome. An update, Circulation, 88, pp. 782-784, (1993)

[10]

Zareba W., Moss A.J., Long QT syndrome in children, J Electrocardiol, 34, SUPPL., pp. 167-171, (2001)

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