Published Online
on April 2, 2007

A more recent version of this article appeared on April 17, 2007

This Article Full Text (PDF) All Versions of this Article: 115/15/2042    most recent CIRCULATIONAHA.106.664219v1 Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Probst, V. Articles by Wilde, A. A.M. Search for Related Content PubMed PubMed Citation Articles by Probst, V. Articles by Wilde, A. A.M. Pubmed/NCBI databases Compound via MeSH Substance via MeSH Hazardous Substances DB QUINIDINE QUINIDINE SULFATE Related Collections Electrophysiology Clinical genetics Ion channels/membrane transport Electrocardiology Pediatric and congenital heart disease, including cardiovascular surgery Arrhythmias, clinical electrophysiology, drugs Genetics of cardiovascular disease Related Article

Submitted on September 20, 2006
Accepted on December 22, 2006

Clinical Aspects and Prognosis of Brugada Syndrome in Children

Vincent Probst MD, PhD^*, Isabelle Denjoy MD, Paola G. Meregalli MD, Jean-Christophe Amirault MD, Fréderic Sacher MD, Jacques Mansourati MD, Dominique Babuty MD, Elisabeth Villain MD, Jacques Victor MD, Jean-Jacques Schott PhD, Jean-Marc Lupoglazoff MD, PhD, Philippe Mabo MD, Christian Veltmann MD, Laurence Jesel MD, Philippe Chevalier MD, Sally-Ann B. Clur MD, Michel Haissaguerre MD, Christian Wolpert MD, Hervé Le Marec MD, PhD, and Arthur A.M. Wilde MD, PhD

From L’Institut du Thorax, CHU de Nantes (V.P., J.A., H.L.M.), INSERM U533 (V.P., J.S., H.L.M.), and CIC de Nantes (V.P., J.A., H.L.M.), Nantes, France; Lariboisière Hospital and Centre Cardiologique Infantile (I.D.), Château des Côtes, Les Loges en Josas, France; Department of Cardiology (P.G.M., A.A.M.W.), Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands; Service de Cardiologie (F.S., M.H.), Hôpital Cardiologique du Haut Leveque, Bordeaux, France; Service de Cardiologie (J.M.), Centre Hospitalo-Universitaire de Brest, Brest, France; Service de Cardiologie B (D.B.), Hôpital Trousseau, Tours, France; Cardiologie Pédiatrique (E.V.), Hôpital Necker-Enfants Malades, Paris, France; Service de Cardiologie (J.V.), Centre Hospitalo-Universitaire d’Angers, Angers, France; Paediatric Cardiology (J.L.), Robert Debré Hospital, Paris, France; Departement de Cardiologie (P.M.), Hôpital Pontchaillou, Rennes, France; University Hospital Mannheim (C.V., C.W.), University of Heidelberg, Germany; Service de Cardiologie (L.J.), CHU de Strasbourg, Strasbourg, France; Service de Cardiologie (P.C.), CHU de Lyon, Lyon, France; and Department of Paediatric Cardiology (S.B.C.), Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.

^* To whom correspondence should be addressed. E-mail: vincent.probst{at}chu-nantes.fr.

Background--Brugada syndrome is an arrhythmogenic disease characterized by an ECG pattern of ST-segment elevation in the right precordial leads and augmented risk of sudden cardiac death. Little is known about the clinical presentation and prognosis of this disease in children.

Methods and Results--Thirty children affected by Brugada syndrome who were <16 years of age (mean, 8±4 years) were included. All patients displayed a type I ECG pattern before or after drug provocation challenge. Diagnosis of Brugada syndrome was made under the following circumstances: aborted sudden death (n=1), syncope of unexplained origin (n=10), symptomatic supraventricular tachycardia (n=1), suspicious ECG (n=1), and family screening for Brugada syndrome (n=17). Syncope was precipitated by fever in 5 cases. Ten of 11 symptomatic patients displayed a spontaneous type I ECG. An implantable cardioverter-defibrillator was implanted in 5 children; 4 children were treated with hydroquinidine; and 1 child received a pacemaker because of symptomatic sick sinus syndrome. During a mean follow-up of 37±23 months, 1 child experienced sudden cardiac death, and 2 children received an appropriate implantable cardioverter-defibrillator shock; all of them were symptomatic and had manifested a type I ECG spontaneously. One child had a cardioverter-defibrillator infection that required explantation of the defibrillator.

Conclusions--In the largest population of children affected by Brugada syndrome described to date, fever represented the most important precipitating factor for arrhythmic events, and as in the adult population, the risk of arrhythmic events was higher in previously symptomatic patients and in those displaying a spontaneous type I ECG.

Key words: arrhythmia • Brugada syndrome • death, sudden • genetics • ion channels • pediatrics • tachyarrhythmias

Related Article:

Issue Highlights
Circulation 2007 115: 1969. [Extract] [Full Text]

This article has been cited by other articles:

				R M Campbell, S Berger, and J Drezner Sudden cardiac arrest in children and young athletes: the importance of a detailed personal and family history in the pre-participation evaluation Br. J. Sports Med., May 1, 2009; 43(5): 336 - 341. [Abstract] [Full Text] [PDF]

				S. G. Priori, C. Napolitano, S. E. Humphries, and J. Skipworth CHAPTER 9 Genetics of Cardiovascular Diseases ESC Textbook of Cardiovascular Medicine, January 1, 2009; 2(1): med-9780199566990-chapter - med-9780199566990-chapter. [Abstract] [Full Text] [PDF]

				B. Benito, A. Sarkozy, L. Mont, S. Henkens, A. Berruezo, D. Tamborero, D. Arzamendi, P. Berne, R. Brugada, P. Brugada, et al. Gender Differences in Clinical Manifestations of Brugada Syndrome J. Am. Coll. Cardiol., November 4, 2008; 52(19): 1567 - 1573. [Abstract] [Full Text] [PDF]

				Brugada Syndrome in Children Journal Watch Cardiology, May 16, 2007; 2007(516): 4 - 4. [Full Text]

				S. Viskin Brugada Syndrome in Children: Don't Ask, Don't Tell? Circulation, April 17, 2007; 115(15): 1970 - 1972. [Full Text] [PDF]