Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome

Michowitz, Yoav; Milman, Anat; Andorin, Antoine; Sarquella‐Brugada, Georgia; Corcia, M Cecilia Gonzalez; Gourraud, Jean‐Baptiste; Conte, Giulio; Sacher, Frédéric; Juang, Jimmy J.M.; Kim, Sung Hwan; Leshem, Eran; Mabo, Philippe; Postema, Pieter G.; Hochstadt, Aviram; Wijeyeratne, Yanushi D.; Denjoy, Isabelle; Giustetto, Carla; Mizusawa, Yuka; Huang, Zhengrong; Jespersen, Camilla H B; Maeda, Shingo; Takahashi, Yoshihide; Kamakura, Tsukasa; Aiba, Takeshi; Arbelo, Elena; Mazzanti, Andrea; Allocca, Giuseppe; Brugada, Ramón; Casado-Arroyo, Rubén; Champagne, Jean; Priori, Silvia G.; Veltmann, Christian; Delise, Pietro; Corrado, Domenico; Brugada, Josép; Kusano, Kengo; Hirao, Kazuyuki; Calò, Leonardo; Takagi, Masahiko; Tfelt-Hansen, Jacob; Yan, Gan Xin; Gaïta, Fiorenzo; Leenhardt, Antoine; Behr, Elijah R.; Wilde, Arthur A.M.; Nam, Gi Byoung; Brugada, Pedro; Probst, Vincent; Belhassen, Bernard

doi:10.1016/j.jacc.2019.01.048

Cited by 59 publications

(52 citation statements)

References 26 publications

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“…According to a recent large cohort targeting BrS patients under 20 years old, 3 there were marked differences in the symptoms between the pediatric group (under 12 years old) and adolescent group (13 to 20 years old). In the pediatric group, the mean age of the onset was 3 years and they were characterized by a greater prevalence of females (42%) and a high morbidity from a fever (52%) during arrhythmic events.…”

Section: Discussionmentioning

confidence: 99%

Successful endocardial catheter ablation of a drug-resistant monomorphic ventricular tachycardia in a child with Brugada syndrome

Nabeshima

Sumitomo

Muraji

et al. 2020

HeartRhythm Case Reports

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Section: Discussionmentioning

confidence: 99%

Successful endocardial catheter ablation of a drug-resistant monomorphic ventricular tachycardia in a child with Brugada syndrome

Nabeshima

Sumitomo

Muraji

et al. 2020

HeartRhythm Case Reports

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“…Considering the fact that quinidine effectively prevents VF induction in patients with BrS, it is the ideal medication for managing patients with BrS that exhibit atrial or ventricular arrhythmias and preventing both episodes of AF and VF 68 . Recently, Michowitz et al 69 studied 57 young patients [(age ≤ 12 years; n = 26) and adolescents (age 13‐20 years; n = 31)] with BrS and arrhythmic events. During follow‐up, 68% of pediatric and 64% of adolescents had recurrent arrhythmic events while approximately one‐third of recurrent events occurred on quinidine therapy, 69 Atrial arrhythmias were included among the risk factors for recurrent arrhythmic events in the pediatric group 69 …”

Section: Long‐term Management and Prognosismentioning

confidence: 99%

“…Considering the fact that quinidine effectively prevents VF induction in patients with BrS, it is the ideal medication for managing patients with BrS that exhibit atrial or ventricular arrhythmias and preventing both episodes of AF and VF. 68 Recently, Michowitz et al 69 Amiodarone (a class III AAD) has been demonstrated to be ineffective in prolonging survival in BrS. Sotalol may cause bradycardia, facilitating the induction of VAs, while beta-blockers and calcium channel blockers may increase transmural dispersion of repolarization and ECG ST-segment elevation.…”

mentioning

confidence: 99%

Atrial fibrillation in Brugada syndrome: Current perspectives

Vlachos

Mascia

Martin

et al. 2020

Cardiovasc electrophysiol

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The incidence of atrial fibrillation (AF) in Brugada syndrome (BrS) has been reported at between 9% and 53% by different series, but the true prevalence is unknown. However, AF may be the presenting feature in some patients. The underlying mechanisms for AF may be a combination of multiple factors, genetic or acquired, that may impact upon autonomic function, atrial structure, and conduction velocities or other unknown factors. The presence of AF has been associated with a more malignant course, with a greater incidence of syncope and ventricular arrhythmias, thus acting as marker of more advanced disease. Regarding the management of patients with AF, antiarrhythmic drugs effective in preventing malignant arrhythmias in BrS such as quinidine or invasive treatment with pulmonary vein isolation (PVI) may be useful in AF treatment. In this review, we aim to present the current perspectives regarding the genetics, pathophysiology, management, and prognosis of AF in patients with BrS.

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“…Brugada syndrome is associated with syncope and cardiac arrest resulting from degeneration into VF of episodes of polymorphic ventricular tachycardia (Brugada and Brugada, 1992;Michowitz et al, 2019). Pathogenic changes in SCN5A, which encodes the α-subunit of the cardiac voltage-gated Na channel, were the first described molecular cause of Brugada syndrome and remain the only gene unequivocally associated with Brugada syndrome, although subsequently several other genes with pathogenic changes have been implicated collectively contributing to approximately 2-5% of cases (Brugada et al, 2018).…”

Section: Genetic Ion Channelopathiesmentioning

confidence: 99%

Sudden cardiac death in children and young adults without structural heart disease: a comprehensive review

Tsuda

Fitzgerald

Temple

2020

Rev. Cardiovasc. Med.

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Sudden cardiac death (SCD) is a rare clinical encounter in pediatrics, but its social impact is immense because of its unpredicted and catastrophic nature in previously healthy individuals. Unlike in adults where the primary cause of SCD is related to ischemic heart disease, the etiology is diverse in young SCD victims. Although certain structural heart diseases may be identified during autopsy in some SCD victims, autopsy-negative SCD is more common in pediatrics, which warrants the diagnosis of sudden arrhythmic death syndrome (SADS) based upon the assumption that the usual heart rhythm is abruptly replaced by lethal ventricular arrhythmia. Despite current advances in molecular genetics, the causes of more than half of SADS cases remain unanswered even after postmortem genetic testing. Moreover, the majority of these deaths occur at rest or during sleep even in the young. Recently, sudden unexpected death in epilepsy (SUDEP) has emerged as another etiology of SCD in children and adults, suggesting critical involvement of the central nervous system (CNS) in SCD. Primary cardiac disorders may not be solely responsible for SCD; abnormal CNS function may also contribute to the unexpected lethal event. In this review article, we provide an overview of the complex pathogenesis of SADS and its diverse clinical presentation in the young and postulate that SADS is, in part, induced by unfortunate miscommunication between the heart and CNS via the autonomic nervous system.

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Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome

Cited by 59 publications

References 26 publications

Successful endocardial catheter ablation of a drug-resistant monomorphic ventricular tachycardia in a child with Brugada syndrome

Successful endocardial catheter ablation of a drug-resistant monomorphic ventricular tachycardia in a child with Brugada syndrome

Atrial fibrillation in Brugada syndrome: Current perspectives

Sudden cardiac death in children and young adults without structural heart disease: a comprehensive review

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