Low Prevalence of Risk Markers in Cases of Sudden Death Due to Brugada Syndrome

Raju, Hariharan; Papadakis, Michael; Govindan, Malini; Bastiaenen, Rachel; Chandra, Navin; O’Sullivan, Aifric; Baines, Georgina; Sharma, Sanjay; Behr, Elijah R.

doi:10.1016/j.jacc.2010.11.067

Cited by 67 publications

(51 citation statements)

References 25 publications

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“…This is, however, consistent with our population and previous experience, 12,13 although there is no overlap between previously reported groups and this current study. The higher prevalence of BrS may partly be explained by provocation testing with a class I antiarrhythmic being a routine part of the cardiological evaluation performed on adult relatives during familial screening for SADS in our center.…”

Section: Limitationssupporting

confidence: 94%

Cardiac Evaluation of Pediatric Relatives in Sudden Arrhythmic Death Syndrome

Wong

Rosés‐Noguer

Till

et al. 2014

Circ: Arrhythmia and Electrophysiology

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Background-Sudden arrhythmic death syndrome defines a sudden unexpected and unexplained death despite comprehensive pathological and toxicological investigation. Previous studies have focused on evaluation of adult relatives. There is, however, a lack of data in children, leading to highly variable management. We sought to determine the clinical utility of cardiac evaluation in pediatric relatives of sudden arrhythmic death syndrome probands. Methods and Results-Retrospective review was undertaken of pediatric patients with a family history of sudden arrhythmic death syndrome assessed from 2010 to 2013 in 2 centers. Clinical history, cardiac, and genetic investigations were assessed, including diagnoses made after evaluation of adult relatives. A total of 112 pediatric relatives from 61 families were evaluated (median age at presentation, 8 years; range, 0.5-16 years). A probable diagnosis was made in 18 (29.5%) families: Brugada syndrome, 13/18 (72%); long QT syndrome, 3/18 (17%); and catecholaminergic polymorphic ventricular tachycardia, 2/18 (11%). Genetic testing identified mutations in 20% of Brugada syndrome (2/10) and 50% of long QT syndrome (1/2) and catecholaminergic polymorphic ventricular tachycardia families (1/2) who were tested. Pediatric evaluation diagnosed 6/112 relatives (5.4%), increasing to 7% (6/85) if only first-degree relatives were assessed. The only useful diagnostic tests were the 12-lead and exercise electrocardiograms and ajmaline provocation test. The median duration of follow-up was 2.1 years (range, 0.2-8.2 years) with no cardiac events. Conclusions-The yield of evaluating pediatric relatives is significant and higher when focused on first-degree relatives and on conditions usually expressed in childhood. We propose a management pathway for these children. (Circ Arrhythm Electrophysiol. 2014;7:800-806.)

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Section: Limitationssupporting

confidence: 94%

Cardiac Evaluation of Pediatric Relatives in Sudden Arrhythmic Death Syndrome

Wong

Rosés‐Noguer

Till

et al. 2014

Circ: Arrhythmia and Electrophysiology

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“…In keeping with these inconsistencies, two recent papers [8, 9] highlighted the inadequacy of traditional markers of risk stratification to detect Brugada patients at higher risk for SCD, hence calling for evaluation of new risk stratifiers.…”

Section: Introductionmentioning

confidence: 99%

Cardiac autonomic control in Brugada syndrome patients during sleep: The effects of sleep disordered breathing

Tobaldini

Brugada

Benito

et al. 2013

International Journal of Cardiology

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Aims Brugada syndrome is characterized by typical ECG features, ventricular arrhythmias and sudden cardiac death (SCD), more frequent during nighttime. Autonomic cardiovascular control has been implicated in triggering the ventricular arrhythmias. Sleep-disordered breathing (SDB) elicits marked autonomic changes during sleep and it is associated with an increased risk of nighttime SCD. Brugada patients may have a higher likelihood of SDB compared to controls. However, no data are available on cardiac autonomic control in Brugada patients, particularly with regard to the comorbidity of SDB. Methods We evaluated autonomic cardiovascular control in Brugada patients with SDB (BRU-SDB, n=9), without SDB (BRU, n= 9), in controls (CON, n=8) and in non-Brugada patients with SDB (n=6), during wakefulness and sleep (N2, N3 and REM). Linear spectral and entropy-derived measures of heart rate variability (HRV) were performed during apnea-free stable breathing epochs. Results Total HRV was attenuated in BRU-SDB compared to CON and BRU. During N2 and REM, in BRU-SDB patients sympathetic modulation decreased compared to BRU and CON, while during REM, they showed an increased parasympathetic modulation, compared to the other two groups. BRU-SDB and SDB were similar in terms of spectral components. Entropy-derived indices showed preserved dynamic changes in Brugada patients compared to controls through the different sleep stages. Conclusion Brugada syndrome per se does not appear associated with an altered autonomic cardiovascular control during wakefulness and sleep. The comorbidity with SDB may contribute to disrupted autonomic cardiovascular regulation during sleep, possibly predisposing to the increased likelihood of sleep-related ventricular tachyarrhythmias and SCD.

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“…4 In a recent report, most SCDs occurred in previously asymptomatic subjects. 5 Given that current management of patients with BS is limited to the implantation of a cardioverter defibrillator, which is related to a high rate of complications in young patients, 6 risk stratification of asymptomatic subjects clearly requires improvement. This meta-analysis evaluated current risk stratification tools including the presence of spontaneous diagnostic ECG pattern or inducible ventricular arrhythmias at programmed ventricular stimulation (PVS) as predictors of future arrhythmic events in asymptomatic subjects with BS.…”

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confidence: 99%