Long-QT syndrome

被引:284
作者
Roden, Dan M. [1 ,2 ]
机构
[1] Vanderbilt Univ, Sch Med, Oates Inst Expt Therapeut, Dept Med, Nashville, TN 37232 USA
[2] Vanderbilt Univ, Sch Med, Oates Inst Expt Therapeut, Dept Pharmacol, Nashville, TN 37232 USA
关键词
D O I
10.1056/NEJMcp0706513
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
After the sudden death of a 13-year-old girl while she was playing basketball, her family comes to the clinic for medical evaluation (Fig. 1). Her parents' resting electrocardiograms (ECGs) are normal, but her 9-year-old sister's ECG shows an abnormally long QT interval. There is a history of recurrent syncope in female relatives of the maternal grandmother, but there is no family history of other sudden deaths, the sudden infant death syndrome, drowning, or death from a motor vehicle accident. How should these family members be further evaluated and treated? Copyright © 2008 Massachusetts Medical Society.
引用
收藏
页码:169 / 176
页数:8
相关论文
共 52 条
[1]   Prevalence of long-QT syndrome gene variants in sudden infant death syndrome [J].
Arnestad, Marianne ;
Crotti, Lia ;
Rognum, Torleiv O. ;
Insolia, Roberto ;
Pedrazzini, Matteo ;
Ferrandi, Chiara ;
Vege, Ashild ;
Wang, Dao W. ;
Rhodes, Troy E. ;
George, Alfred L., Jr. ;
Schwartz, Peter J. .
CIRCULATION, 2007, 115 (03) :361-367
[2]  
Benhorin J, 2000, CIRCULATION, V101, P1698
[3]   Phenotypic variability and unusual clinical severity of congenital long-QT syndrome in a founder population [J].
Brink, PA ;
Crotti, L ;
Corfield, V ;
Goosen, A ;
Durrheim, G ;
Hedley, P ;
Heradien, M ;
Geldenhuys, G ;
Vanoli, E ;
Bacchini, S ;
Spazzolini, C ;
Lundquist, AL ;
Roden, DM ;
George, AL ;
Schwartz, PJ .
CIRCULATION, 2005, 112 (17) :2602-2610
[4]   Spectrum and frequency of cardiac channel defects in swimming-triggered arrhythmia syndromes [J].
Choi, G ;
Kopplin, LJ ;
Tester, DJ ;
Will, ML ;
Haglund, CM ;
Ackerman, MJ .
CIRCULATION, 2004, 110 (15) :2119-2124
[5]   Role of implantable cardioverter defibrillator therapy in patients with long OT syndrome [J].
Daubert, James P. ;
Zareba, Wojciech ;
Rosero, Spencer Z. ;
Budzikowski, Adam ;
Robinson, Jennifer L. ;
Moss, Arthur J. .
AMERICAN HEART JOURNAL, 2007, 153 (04) :53-58
[6]   Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome [J].
Hobbs, Jenny B. ;
Peterson, Derick R. ;
Moss, Arthur J. ;
McNitt, Scott ;
Zareba, Wojciech ;
Goldenberg, Ilan ;
Qi, Ming ;
Robinson, Jennifer L. ;
Sauer, Andrew J. ;
Ackerman, Michael J. ;
Benhorin, Jesaia ;
Kaufman, Elizabeth S. ;
Locati, Emanuela H. ;
Napolitano, Carlo ;
Priori, Silvia G. ;
Towbin, Jeffrey A. ;
Vincent, G. Michael ;
Zhang, Li .
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION, 2006, 296 (10) :1249-1254
[7]  
Hofman N, 2007, EUR HEART J, V28, P575, DOI 10.1093/eurheartj/ehl355
[8]   Female predominance and transmission distortion in the long-QT syndrome [J].
Imboden, Medea ;
Swan, Heikki ;
Denjoy, Isabelle ;
Marijke Van Langen, Irene ;
Latinen-Forsblom, Paivi Johanna ;
Napolitano, Carlo ;
Fressart, Veronique ;
Breithardt, Guenter ;
Berthet, Myriam ;
Priori, Silvia ;
Hainque, Bernard ;
Wilde, Arthur Arnold Maria ;
Schulze-Bahr, Eric ;
Feingold, Josue ;
Guicheney, Pascale .
NEW ENGLAND JOURNAL OF MEDICINE, 2006, 355 (26) :2744-2751
[9]   Molecular and cellular mechanisms of cardiac arrhythmias [J].
Keating, MT ;
Sanguinetti, MC .
CELL, 2001, 104 (04) :569-580
[10]   Clinical implications for affected parents and siblings of probands with long-QT syndrome [J].
Kimbrough, J ;
Moss, AJ ;
Zareba, W ;
Robinson, JL ;
Hall, J ;
Benhorin, J ;
Locati, EH ;
Medina, A ;
Napolitano, C ;
Priori, S ;
Schwartz, PJ ;
Timothy, K ;
Towbin, JA ;
Vincent, GM ;
Zhang, L .
CIRCULATION, 2001, 104 (05) :557-562