Copyright © 2004 by the European Society of Cardiology.
Clinical research
Low incidence of cardiac events with ß-blocking therapy in children with long QT syndrome
a Cardiologie Pédiatrique, Hôpital Necker-Enfants-Malades, 149 Rue de Sevres, 75015 Paris Cedex, Paris, France
b Cardiologie, Hôpital Lariboisière, Paris, France
c INSERM U582, Institut de Myologie, Hôpital Pitié-Salpêtrière, Paris, France
d Cardiologie Pédiatrique, Hôpital Robert Debré, Paris, France
e Biochimie, Hôpital Pitié-Salpêtrière Paris
f Cardiologie Pédiatrique, Château des Côtes, Les Loges en Josas, France
Received November 4, 2003; revised May 19, 2004; accepted June 10, 2004 * Corresponding author. Tel.: +33-142-73-84-86 (E-mail: elisabeth.villain{at}nck.ap-hop-paris.fr).
Aims To evaluate the effect of beta-blockers in children with long QT syndrome (LQTS) we reviewed the outcome of 122 patients (pts).
Methods LQTS was diagnosed in 24 neonates and in 98 pts aged 0.5–15 years. Diagnosis was made because of syncope in 51 pts, bradycardia in 10 neonates and family history in 61 pts. The longest QTc ranged from 400 to 700 ms. Thirteen pts had 2:1 atrioventricular block and/or ventricular arrhythmias. Screening for mutations was performed in 118 pts. All children were treated with beta-blockers, annually checked by exercise testing and/or 24 h ECG monitoring.
Results Four pts died. Survivors were followed-up for 1–18 years (7.5±5.3 years). Five neonates and 3 older pts received a prophylactic pacemaker (1 death) so that only 111/122 pts survived and were followed-up with beta-blockers alone. None of them died and five experienced a non-fatal cardiac event. There was no cardiac event among pts who were diagnosed because of familial history and among symptomatic KCNQ1 pts who were effectively treated with beta-blockers.
Conclusion The outcome of children with LQTS under effective beta-blockers is favourable. Persisting arrhythmia or symptoms despite beta-blockers should aim at identifying other genotypes than KCNQ1.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  L. Crotti, C. Spazzolini, P. J. Schwartz, W. Shimizu, I. Denjoy, E. Schulze-Bahr, E. V. Zaklyazminskaya, H. Swan, M. J. Ackerman, A. J. Moss, et al. The Common Long-QT Syndrome Mutation KCNQ1/A341V Causes Unusually Severe Clinical Manifestations in Patients With Different Ethnic Backgrounds: Toward a Mutation-Specific Risk Stratification Circulation, November 20, 2007; 116(21): 2366 - 2375. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Balaji Long QT Syndrome in Children: Not One Disease Anymore J. Am. Coll. Cardiol., October 2, 2007; 50(14): 1341 - 1342. [Full Text] [PDF]
|
|
|
|
|
|
E. A. Stephenson and C. I. Berul Electrophysiological Interventions for Inherited Arrhythmia Syndromes Circulation, August 28, 2007; 116(9): 1062 - 1080. [Full Text] [PDF]
|
|
|
|
 |
|
 F. Suto, W. Zhu, A. Chan, and G. J. Gross IKr and IKs remodeling differentially affects QT interval prolongation and dynamic adaptation to heart rate acceleration in bradycardic rabbits Am J Physiol Heart Circ Physiol, April 1, 2007; 292(4): H1782 - H1788. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Bell and K. McLeod Not so funny turns Arch. Dis. Child. Ed. Pract., February 1, 2007; 92(1): ep7 - ep13. [Full Text] [PDF]
|
|
|
|
|
|
M. Viitasalo, L. Oikarinen, H. Swan, H. Vaananen, J. Jarvenpaa, H. Hietanen, J. Karjalainen, and L. Toivonen Effects of Beta-Blocker Therapy on Ventricular Repolarization Documented by 24-h Electrocardiography in Patients With Type 1 Long-QT Syndrome J. Am. Coll. Cardiol., August 15, 2006; 48(4): 747 - 753. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. J. Schwartz, C. Spazzolini, L. Crotti, J. Bathen, J. P. Amlie, K. Timothy, M. Shkolnikova, C. I. Berul, M. Bitner-Glindzicz, L. Toivonen, et al. The Jervell and Lange-Nielsen Syndrome: Natural History, Molecular Basis, and Clinical Outcome Circulation, February 14, 2006; 113(6): 783 - 790. [Abstract] [Full Text] [PDF]
|
|