Abstract
Introduction: Brugada syndrome (BrS) is a cardiac ion channelopathy with a higher prevalence in Asia compared to the Western populations. The present study compared the differences in clinical and electrocardiographic (ECG) presentation between paediatric/young (≤25 years old) and adult (>25 years) BrS patients.Method: This was a territory-wide retrospective cohort study of consecutive BrS patients presenting to public hospitals in Hong Kong. The primary outcome was spontaneous ventricular tachycardia/ventricular fibrillation (VT/VF).Results: The cohort consists of 550 consecutive patients (median age of initial presentation = 51 ± 23 years; female = 7.3%; follow-up period = 83 ± 80 months), divided into adult (n = 505, mean age of initial presentation = 52 ± 19 years; female = 6.7%; mean follow-up period = 83 ± 80 months) and paediatric/young subgroups (n = 45, mean age of initial presentation = 21 ± 5 years, female = 13.3%, mean follow-up period = 73 ± 83 months). The mean annual VT/VF incidence rate were 17 and 25 cases per 1,000 patient-year, respectively. Multivariate analysis showed that initial presentation of type 1 pattern (HR = 1.80, 95% CI = [1.02, 3.15], p = 0.041), initial asymptomatic presentation (HR = 0.26, 95% CI = [0.07, 0.94], p = 0.040) and increased P-wave axis (HR = 0.98, 95% CI = [0.96, 1.00], p = 0.036) were significant predictors of VT/VF for the adult subgroup. Only initial presentation of VT/VF was predictive (HR = 29.30, 95% CI = [1.75, 492.00], p = 0.019) in the paediatric/young subgroup.Conclusion: Clinical and ECG presentation of BrS vary between the paediatric/young and adult population in BrS. Risk stratification and management strategies for younger patients should take into consideration and adopt an individualised approach.
Highlights
Brugada syndrome (BrS) is a cardiac ion channelopathy with a higher prevalence in Asia compared to the Western populations
sudden cardiac death (SCD) in young people is more commonly caused by hypertrophic cardiomyopathy in the United States and arrhythmogenic right ventricular cardiomyopathy in parts of Europe, cardiac ion channelopathies often underly juvenile cases of SCD without pre-existing comorbidities, which can cause great distress toward patients’ families and the general public [7, 8]
There was a significantly greater proportion of paediatric/young patients presenting with fever at the onset of Brugada ECG pattern (BrP) (p = 0.018), or with a family history of BrS (p = 0.009)
Summary
Brugada syndrome (BrS) is a cardiac ion channelopathy with a higher prevalence in Asia compared to the Western populations. SCD in young people is more commonly caused by hypertrophic cardiomyopathy in the United States and arrhythmogenic right ventricular cardiomyopathy in parts of Europe, cardiac ion channelopathies often underly juvenile cases of SCD without pre-existing comorbidities, which can cause great distress toward patients’ families and the general public [7, 8]. Of these conditions, BrS is the most prevalent ion channelopathy found in Asia [9,10,11,12]. The present study aims to demonstrate the difference in clinical and electrocardiographic (ECG) presentation between paediatric/young and adult BrS patients
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