High prevalence of concealed Brugada syndrome in patients with atrioventricular nodal reentrant tachycardia

Hasdemir, Can; Payzın, Serdar; Kocabaş, Umut; Şahin, Hatice; Yildirim, Nihal; Alp, Alpay; Aydın, Mehmet; Pfeiffer, Ryan; Burashnikov, Elena; Wu, Yong; Antzelevitch, Charles

doi:10.1016/j.hrthm.2015.03.015

Cited by 83 publications

(89 citation statements)

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“…27 Finally, genetic testing was performed in only 17 patients: 10 with concealed Brugada syndrome and 7 with previous diagnosis of Brugada syndrome who also had AVNRT. 26 Although, in general, the yield of mutation identification in a Brugada syndrome cohort is at most 30%, 29 in this cohort it was unexpectedly high (70%) 26 and the pathogenicity of the variants reported here could not be demonstrated. Furthermore, the fact that conduction intervals (PR, HV, and QRS) were normal in the present Brugada syndrome cohort, where half the patients (13 of 26) harbored a "mutation" mainly affecting sodium channel function (in 10 of 13), argues against a major role of genes impacting the sodium current because patients with SCN5A-associated Brugada syndrome characteristically have prolonged conduction intervals.…”

Section: Q6mentioning

confidence: 61%

“…24 Accordingly, sodium channel defects may be one of the contributory causes of Brugada syndrome but not necessarily the main one. 25 Because of the incertitude surrounding the specificity of the ajmaline test, one should look at the study by Hasdemir et al, 26 published in this issue of HeartRhythm, on the high prevalence of "concealed Brugada syndrome" in patients with atrioventricular (AV) nodal reentry tachycardia (AVNRT) with due caution.…”

Section: Q6mentioning

confidence: 99%

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Everybody has Brugada syndrome until proven otherwise?

et al. 2015

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Section: Q6mentioning

confidence: 61%

Section: Q6mentioning

confidence: 99%

Everybody has Brugada syndrome until proven otherwise?

et al. 2015

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“…8 Recent studies have highlighted concern regarding the possibility of a high false-positive rate with provocative drug challenge, particularly in the case of ajmaline use, although the lack of a gold standard renders this concern challenging to assess in an objective manner. 37,38 Driven by concern for over-diagnosis, experts have proposed the Shanghai Score System that, beyond the ECG analysis, also accounts for clinical and family history and genetic testing results (Figure 1b). 39 The distinctive feature of this scoring system, relative to the recent HRS/EHRA/APHRS expert consensus statement, is the requirement for additional features to be considered beyond the ECG in order to conclude a probable/definite BrS diagnosis in cases in which a type 1 pattern is exclusively observed during fever or a provocative drug challenge.…”

Section: Diagnosismentioning

confidence: 99%

Brugada Syndrome: Evolving Insights and Emerging Treatment Strategies

Duffett

Roberts

2017

JICRM

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ABSTRACT. Brugada syndrome (BrS) is a rare inherited arrhythmia disorder associated with sudden cardiac death secondary to malignant ventricular arrhythmias. Since its first mention approximately 25 years ago, major strides have been made towards unraveling the condition's genetic and mechanistic underpinnings. Despite considerable progress, however, gaps in the understanding of BrS continue to persist, and clinical management of affected individuals remains challenging. Identification of an underlying genetic culprit continues to be elusive in the majority of patients, while discord regarding the condition's underlying pathophysiology also persists, with strong lines of evidence present for both the ''depolarization'' and ''repolarization'' hypotheses. Exciting new therapeutic options hold significant promise, including substrate-based catheter ablation and the subcutaneous implantable cardioverter-defibrillator, although the decision of when to intervene in the cases of asymptomatic patients remains unclear. Provided that the risk of events in BrS is not truly stochastic, distinct sub-phenotypes of the condition, possessing variable levels of arrhythmic risk, may exist, and their identification may lead to the improved care of BrS patients and their families.

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“…Some recent reports show a higher proportion. 4,22 The presence of an identifiable mutation has not been clearly linked to a worse prognosis; this being particularly true amongst asymptomatic patients. 10 Nevertheless, some studies suggest a possible relationship.…”

Section: Geneticsmentioning

confidence: 99%

Brugada Syndrome: Defining the Risk in Asymptomatic Patients

Sieira¹,

Brugada²

2016

Arrhythmia &Amp; Electrophysiology Review

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Since the first description of the Brugada syndrome (BS) in 1992, scientific progress in the understanding of this disease has been enormous; at the same time more and more individuals with the disease have been diagnosed. The profile of patients with BS has changed with more asymptomatic individuals and less expressive clinical features. Asymptomatic BS individuals are at lower arrhythmic risk than those presenting with syncope or sudden cardiac death (SCD). The event incidence rate is around 0.5 % per year; this figure is relevant due to the fact that individuals have a long life expectancy and are otherwise healthy. As a result of the risk of SCD, risk stratification is of utmost importance. As the implantation of a cardioverter defibrillator is the main treatment for those patients at higher risk, benefits and long-term potential risks have to be adequately considered. Some risk factors, such as spontaneous type 1 electrocardiogram (ECG) pattern, are widely accepted, whilst for others contradictory data are present. Furthermore, novel risk factors are now available that might help in the management of BS. The presence of a spontaneous type 1 ECG pattern, history of sinus node dysfunction and inducible ventricular arrhythmias during programmed electrical stimulation of the heart allow us to risk stratify these patients.

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High prevalence of concealed Brugada syndrome in patients with atrioventricular nodal reentrant tachycardia

Cited by 83 publications

References 32 publications

Everybody has Brugada syndrome until proven otherwise?

Everybody has Brugada syndrome until proven otherwise?

Brugada Syndrome: Evolving Insights and Emerging Treatment Strategies

Brugada Syndrome: Defining the Risk in Asymptomatic Patients

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