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Follow-up of children with Brugada Syndrome: experience from a tertiary paediatric referral centre
Session:
Comunicações Orais (Sessão 28) - Cardiopatias Congénitas, Doença Vascular Pulmonar e Embolia Pulmonar 3 - Vários Tópicos
Speaker:
SARA ISABEL LOPES FERNANDES
Congress:
CPC 2022
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
20. Congenital Heart Disease and Pediatric Cardiology
Subtheme:
20.7 Pediatric Cardiology
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Sara Lopes Fernandes; Carolina Saleiro; Andreia Palma; Diogo Faim; João Dias; Izidro Borges; Isabel Santos; Helena Andrade; António Pires
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Introduction: </strong>Brugada syndrome (BrS) is an autosomal dominant channelopathy, which typically presents in young adults. It can also be diagnosed in children, but data in this age group is scarce. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Aim: </strong>To describe the clinical features, management and long-term follow-up of children with BrS history followed-up in a tertiary paediatric referral centre.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Methods: </strong>Single centre retrospective study of consecutive patients with history of BrS, defined as having a BrS positive phenotype (BrS (+)), or a negative phenotype-positive genotype (BrS (-)). They were all followed up in a paediatric heart rhythm clinic. Clinical and demographical data were collected and analysed according to the phenotype. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Results: </strong>30 patients were included, with a median age at diagnosis of 7 years (IQR 1-13) and a mean follow-up time of 7 ± 3 years. Sixteen patients were BrS(+), predominantly male (n=13, 81%). 88% (n=14) performed a genetic test, which was positive in 57% (n=8); the most frequent mutation was SCN5A (n=5). Family history of BrS was present in 56% (n=9) and almost one third had family history of sudden cardiac death (SCD). Most of the patients had a type 1 Brugada ECG pattern (n=14) and 2 patients presented a fever and drug induced pattern, respectively. Fourteen patients were BrS(-), mostly female (n=11, 79%) with a loss-of-function mutation in the SCN5A gene (n=10). They all had family members with BrS, mainly from the paternal side, and 43% (n=6) mentioned SCD history. Although most of the patients were asymptomatic, the prevalence of rhythm or conduction disturbances was not infrequent, particularly in BrS(+) patients (n=12, 75%). Also, in this group and during follow-up, 3 patients had documented supraventricular tachyarrhythmias, and 2 patients had syncope episodes, one of which required an implantable cardioverter-defibrillator. No events were reported in the BrS (-) patients. Nine patients (n=9/30, 30%) were hospitalized, 3 due to an arrhythmic event (all in the BrS(+) group). Overall, no sudden cardiac death event was reported during follow-up. </span></span></p> <p><strong><span style="font-size:11.0pt"><span style="font-family:"Calibri",sans-serif">Conclusion: </span></span></strong><span style="font-size:11.0pt"><span style="font-family:"Calibri",sans-serif">In our study, a</span></span><span style="font-size:11.0pt"><span style="font-family:"Calibri",sans-serif">lthough the majority of the patients were asymptomatic, the occurrence of arrhythmic events was not negligible, especially in the BrS(+) patients. Despite the significant family history, patients with BrS(-) had no events reported during follow-up. Nevertheless, the management of these patients is not clear cut, and a personalized therapeutic strategy with close follow-up is essential.</span></span></p>
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