Impact of clinical and genetic findings on the management of young patients with Brugada syndrome

Andorin, Antoine; Behr, Elijah R.; Denjoy, Isabelle; Crotti, Lia; Dagradi, Federica; Jesel, Laurence; Sacher, Frédéric; Petit, Bertrand; Mabo, Philippe; Maltret, Alice; Wong, Leonie C.H.; Degand, Bruno; Bertaux, Géraldine; Maury, Philippe; Dulac, Yves; Delasalle, Béatrice; Gourraud, Jean‐Baptiste; Babuty, Dominique; Blom, Nico A.; Schwartz, Peter J.; Wilde, Arthur A.M.; Probst, Vincent

doi:10.1016/j.hrthm.2016.02.013

Cited by 91 publications

(64 citation statements)

References 28 publications

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“…In young patients, the presence of the spontaneous Brugada type 1 ECG pattern and symptoms at diagnosis are predictors of life‐threatening arrhythmias (LTA). The management of BrS should be age‐specific, and prevention of SCD may involve genetic testing and aggressive use of antipyretics and quinidine, with risk‐specific consideration for ICD implantation (Andorin et al, ). Provocative drug testing during childhood had a low yield for predicting future AEs and was associated with serious complications in some patients.…”

Section: Discussionmentioning

confidence: 99%

Repetitive syncopal episodes in a child with documented ventricular tachycardia, early repolarization pattern in leads I an aVL, Brugada syndrome, and fever

Rodríguez-Reyes¹,

Pérez‐Riera

López³

et al. 2019

Noninvasive Electrocardiol

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We present a small child with febrile peaks and syncopal episodes secundary to ventricular tachycardia, in whom it was eventually possible to demostrate the Brugada Syndrome with a special presentation in the ECG; early repolarization pattern in lead I and a aVL and Brugada pattern during fever in V1‐V2. This is, to our knowledge, tha first case with this special ECG presentation in a small child.

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Section: Discussionmentioning

confidence: 99%

Repetitive syncopal episodes in a child with documented ventricular tachycardia, early repolarization pattern in leads I an aVL, Brugada syndrome, and fever

Rodríguez-Reyes¹,

Pérez‐Riera

López³

et al. 2019

Noninvasive Electrocardiol

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“…The dominant susceptibility gene is SCN5A, which encodes the alpha subunit of a myocardial sodium channel [10]. The overall prevalence of SCN5A mutations is 20-25%, but SCN5A variants are even more frequent among children with BrS [11]. The phenotypic effect is disequilibrium between the natrium and potassium currents in the epicardial layers of the right ventricle [3,8].…”

Section: Discussionmentioning

confidence: 99%

Optimal management of Brugada syndrome

Stătescu¹,

Vasilcu²,

Chiorescu³

et al. 2017

Arch Clin Cases

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Brugada syndrome can induce malignant ventricular arrhythmias in the absence of structural heart disease. The hallmark of the condition is the ''coved'' (type I) or ''saddle-back'' (types II and III) ST segment elevation in the right precordial leads. The definite diagnosis requires the documentation of the type I morphology. The electrocardiographic pattern is often dynamic, but it can be unmasked by sodium channel blockers such as flecainide. We report the case of a 33-year old male, with family history significant for sudden cardiac death, who underwent successful cardio-pulmonary resuscitation for ventricular fibrillation-associated cardiac arrest. The 12 lead electrocardiogram performed after the resuscitation maneuvers showed intermittent type 1 Brugada pattern. General physical examination and routine laboratory evaluation were unremarkable. A repeated electrocardiogram revealed sinus tachycardia with right bundle branch block. We performed a flecainide challenge test which reproduced the initial coved-type ST segment elevation in V1 and V2. We decided to implant a ventricular single chamber cardioverter-defibrillator with one therapeutical window for ventricular fibrillation (at 300 ms), high energy shocks without antitachycardia pacing. The device was successful in preventing another episode of ventricular fibrillation just 2 days after the procedure. Implantable cardioverter-defibrillators are the most effective secondary prophylaxis therapeutic options for individuals with Brugada Syndrome, but they subject the patient to complications related to device implantation and inappropriate shocks.

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“…Paediatric BS patients who present symptoms have an especially bad prognosis. 4 Conversely, asymptomatic patients appear to have good outcomes, even more so when they do not show the type 1 pattern spontaneously; however, they are not risk free. 15 Therefore, individual evaluation is needed and those patients at higher risk should undergo an ICD implantation.…”

Section: Agementioning

confidence: 99%

“…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%

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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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Impact of clinical and genetic findings on the management of young patients with Brugada syndrome

Abstract: Backgroung: Brugada Syndrome (BrS) is an arrhythmogenic disease associated with sudden

Cited by 91 publications

References 28 publications

Repetitive syncopal episodes in a child with documented ventricular tachycardia, early repolarization pattern in leads I an aVL, Brugada syndrome, and fever

Repetitive syncopal episodes in a child with documented ventricular tachycardia, early repolarization pattern in leads I an aVL, Brugada syndrome, and fever

Optimal management of Brugada syndrome

Brugada Syndrome: Defining the Risk in Asymptomatic Patients

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