Clinical Aspects and Prognosis of Brugada Syndrome in Children

Abstract

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 Ͻ16 years of age (mean, 8Ϯ4 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 (nϭ1), syncope of unexplained origin (nϭ10), symptomatic supraventricular tachycardia (nϭ1), suspicious ECG (nϭ1), and family screening for Brugada syndrome (nϭ17). 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 37Ϯ23 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.

Impact of clinical and genetic findings on the management of young patients with Brugada syndrome

“…However, few data are available on the natural history in children 7,8,11 and the management of this special population has been inconsistent 12 . This study represents the largest series of pediatric BrS patients described as yet.…”

“…Data on 106 patients below age 19 were collected from 16 European tertiary centers; 29 of them had been described originally in 2007 7 . Inclusion required a type 1 Brugada ECG pattern either spontaneously or after challenge with a sodium channel blocker (ajmaline or flecainide).…”

“…The largest pediatric BrS population studied so far included 30 European children and suggested that fever, symptoms and spontaneous type 1 ECG pattern were associated with LTA 7 . Drug-induced BrS in children has been reported 8 .…”

Brugada syndrome: clinical and genetic findings

“…This suggests that febrile states may increase the risk of ventricular arrhythmias, a particularly important trigger factor among the pediatric population. 37 Thus, fast temperature control is crucial in BrS patients. Similarly, drug-induced BrS have been reported, 38 such as cocaine overdose.…”

“…So far, the largest study of pediatric BrS patients included 30 cases from 13 European institutions. 37 Therefore, despite progress in understanding BrS, little is known about this disease in the pediatric population.…”