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Characterisation and long-term follow-up of children with Brugada syndrome: experience from a tertiary paediatric referral centre

Published online by Cambridge University Press:  13 December 2022

Sara I.L. Fernandes Open the ORCID record for Sara I.L. Fernandes [Opens in a new window] ,
Maria H. A. Carvalho ,
Isabel C.T. Santos ,
Andreia F.M. Palma Open the ORCID record for Andreia F.M. Palma [Opens in a new window] ,
Diogo R.O. Faim Open the ORCID record for Diogo R.O. Faim [Opens in a new window] ,
João M.O. Dias ,
Izidro R. Borges ,
Hélia A.F. Martins  and
António M.G.S. Pires
Sara I.L. Fernandes*
Affiliation:
Department of Cardiology, Leiria Hospital Centre, Leiria, Portugal
Maria H. A. Carvalho
Affiliation:
Department of Paediatric Cardiology, Coimbra Hospital and University Centre, Coimbra, Portugal
Isabel C.T. Santos
Affiliation:
Department of Paediatric Cardiology, Coimbra Hospital and University Centre, Coimbra, Portugal
Andreia F.M. Palma
Affiliation:
Department of Paediatric Cardiology, Coimbra Hospital and University Centre, Coimbra, Portugal
Diogo R.O. Faim
Affiliation:
Department of Paediatric Cardiology, Coimbra Hospital and University Centre, Coimbra, Portugal
João M.O. Dias
Affiliation:
Department of Paediatric Cardiology, Coimbra Hospital and University Centre, Coimbra, Portugal
Izidro R. Borges
Affiliation:
Department of Paediatric Cardiology, Coimbra Hospital and University Centre, Coimbra, Portugal
Hélia A.F. Martins
Affiliation:
Department of Cardiology, Leiria Hospital Centre, Leiria, Portugal
António M.G.S. Pires
Affiliation:
Department of Paediatric Cardiology, Coimbra Hospital and University Centre, Coimbra, Portugal
*
Author for correspondence: Sara Lopes Fernandes, Department of Cardiology - Leiria Hospital Centre E.P.E. - Hospital de Santo André, Rua das Olhalvas, 2410-197 Leiria, Portugal. Tel: +351 962402208; Fax: +351 244 817 065. E-mail: sarafernandes24892@gmail.com
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Abstract

Aims:

Brugada syndrome is an inherited condition, which typically presents in young adults. It can also be diagnosed in children, but data in this group remain scarce. This study aims to describe the clinical features, management, and follow-up of children with personal or family history of Brugada syndrome.

Methods:

Retrospective study of consecutive patients with Brugada history followed up in a tertiary paediatric referral centre between 2009 and 2021. Patients were assessed according to the phenotype: positive (with variable genotype) or negative (with positive genotype).

Results:

Thirty patients were included (mean age at diagnosis 7 ± 6 years, 53% male). Within the positive phenotype (n = 16), 81% were male, and 88% had spontaneous type 1 ECG pattern. A genetic test was performed in 88% and was positive in 57%. Fourteen patients had a negative phenotype–positive genotype, 79% female, all diagnosed during family screening; 43% mentioned family history of sudden cardiac death. Although most of the patients were asymptomatic, the prevalence of rhythm/conduction disturbances was not negligible, particularly if a positive phenotype. No clinically significant events were reported in the negative phenotype patients. Three patients were hospitalised due to an arrhythmic cause, all in patients with a positive phenotype.

Conclusion:

In our study, the documentation of rhythm and conduction disturbances was not infrequent, especially in patients with a positive phenotype. Despite the significant family history, phenotype negative patients had no relevant events during follow-up. Nevertheless, the management of these patients is not clear cut, and a personalised therapeutic strategy with close follow-up is essential.

Keywords

Brugada syndromeBrugada patternchildrenarrhythmiasyncope
Type
Original Article
Information
Cardiology in the Young , Volume 33 , Issue 10 , October 2023 , pp. 2028 - 2033
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Copyright
© The Author(s), 2022. Published by Cambridge University Press

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