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(Circulation. 2007;115:2042-2048.)
© 2007 American Heart Association, Inc.

Pediatric Cardiology

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; 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.

Correspondence to Vincent Probst, MD, PhD, Service de Cardiologie du CHU de Nantes, CHU de Nantes, Hôpital Nord, Bd Jacques Monod, 44093 Nantes Cedex, France. E-mail vincent.probst{at}chu-nantes.fr

Received September 20, 2006; accepted December 22, 2006.

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

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